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   Table of Contents - Current issue
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July-September 2019
Volume 62 | Issue 3
Page Nos. 365-518

Online since Friday, July 26, 2019

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EDITORIAL  

From Editor's desk Highly accessed article p. 365
Ranjan Agrawal
DOI:10.4103/0377-4929.263463  PMID:31361220
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GUEST EDITORIAL Top

Biomarker for colorectal carcinoma -ERCC1-New kid on the block Highly accessed article p. 366
Nalini Bansal
DOI:10.4103/IJPM.IJPM_80_19  PMID:31361221
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ORIGINAL ARTICLES Top

Revisiting metastatic central nervous system tumors with unknown primary using clinicopathological findings: A single neurosciences institutional study Highly accessed article p. 368
Anshu Gupta, Sujata Chaturvedi, Deepak Jha, Monali Chaturvedi
DOI:10.4103/IJPM.IJPM_592_18  PMID:31361222
Background: Metastatic tumors are the most common central nervous system (CNS) tumors wherein the primary site remains unknown in most of the cases. Aim: The study was carried out to evaluate metastatic CNS tumors with unknown primary by using simplified diagnostic (clinico-histopathologic) approach. Material and Methods: A 2 years study was conducted on 32 cases of CNS metastases having unknown primary tumors in a neurosciences institute. Statistical Analysis: All the results were prepared using software version of SPSS 22. Results: The most common metastatic site found in brain was cerebrum (59.3%) [frontal > frontoparietal > parieto-occipital > temporal] [left cerebrum > right cerebrum], followed by cerebellum (12.5%), spinal cord (9.3%), and leptomeninges (3.12%). Most of the metastatic tumors presented as ill-defined (34%) rather than well-defined (22%) lesions with ring enhancement seen only in 16% of the cases on magnetic resonance imaging (MRI).On histopathology findings with targeted immunohistochemistry, most common histological tumor type identified irrespective of site was adenocarcinoma (68.7%), followed by squamous cell carcinoma (15.6%) and poorly differentiated carcinoma (12.5%). Only one case of lymphoma was reported. Corroborating all the above findings along with clinical history and other relevant investigations, primary sites could be detected in 23 cases (71.8%).The most common primary site deduced was lungs (39.1%), followed by thyroid (17.3%), breast in females (13.0%), gastrointestinal tract (8.6%), and prostate in males (4.3%). Only in nine cases (28.1%) with mainly poorly differentiated histopathological type, primary site remained unknown. Conclusion: Detection of the primary site in metastatic CNS tumors is possible by adopting this simple and effective diagnostic approach at centers/hospitals having cost and other constraints.
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Akt and estrogen receptor expression in nasal polyps p. 375
Selen Bahçeci, Fatma Şimşek, Erdem Eren, Ibrahim Aladaǧ
DOI:10.4103/IJPM.IJPM_168_18  PMID:31361223
Background: Nasal polyps (NP) is a common chronic inflammatory disease of the mucous membranes in the nose and paranasal sinuses. The underlying mechanisms of pathologic conditions to NP formation remains unclear. The aim of this study is to evaluate the expression of Akt and estrogen receptor (ER) in nasal polyps. Material and Methods: We respectively obtained 20 nasal polyp tissue and 15 concha from patients undergoing endoscopic polyp biopsy and turbinate resection. All samples were fixed in 10% formalin for 24 h and embedding in paraffin was using routine protocol for histological prepation. Sections 5 μm thick were cut and stained H&E. Tissue samples were stained with anti-ER and anti-Akt primary antibody, ER and Akt were evaluated immunohistochemically. There is a relationship between the estrogen receptor and PI3K/Akt signaling pathway in malignansy. In this study, it showed the effect of estrogen on the activation of Akt signaling pathway in nasal polyps. Mann– Whitney-U test was applied to evaluate the statistical differences between nasal polyp and control group, (P < 0.05) was accepted as significant. Results: In H&E stained sections we observed a lot of inflamatory cells and eosinophils in the mucosa, submucosal connective tissue and around the glandular epithelium in nasal polyps. The mucosa and submucosal connective tissue was seen normally in control group in H&E stained. We determined that ER and Akt were intensely expressed in nasal polyps. Expression is localized especially in epithelial and glandular epithelium cells and submucosal connective tissue. In contrast, expression of ER and Akt were mildly expressed in turbinate resection samples. Conclusion: The expression of ER and Akt might be important factors in nasal polyp pathogenesis and may shed new light on clinical approaches in nasal polyp treatment.
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A cytohistological correlation in salivary gland swelling with special reference to the proposed Milan system Highly accessed article p. 379
Soumendra Mishra, Suchandra Ray, Moumita Sengupta, Arunabha Sengupta
DOI:10.4103/IJPM.IJPM_662_17  PMID:31361224
Context and Background: Cytological grading of salivary gland lesion, which is a simple, cost-effective, and reproducible method, can be used as a tool for the selection of treatment modality. The proposed Milan classification establishes one guideline for reporting of salivary gland cytology and thus helps in individualized treatment and follow-up. Aims and Objectives: (1) The aims and objectives of this study were to establish the validity and reliability of the Milan classification of cytological grading in salivary gland swelling and (2) to calculate the malignancy risk. Materials and Methods: This prospective study was designed in clinically diagnosed salivary gland swelling at the Department of Pathology of a tertiary care referral hospital. Fine-needle aspiration (FNA) was done, and stained smears were examined under light microscope and cytological findings were noted according to the Milan classification. Tissue for the histopathological study was obtained in 119 cases. The previous cytological findings were compared to subsequent histopathology report. Results: Among 119 FNAs, 2.5% were nondiagnostic and 55.4% were nonneoplastic. While no samples were placed in the atypia of undetermined significance category, benign tumors accounted for 25.2%. About 1.7% was grouped in the salivary gland neoplasm of uncertain malignant potential, 2.5% of cases were categorized as suspicious of malignancy, and 12.6% of cases comprised as malignant tumors. Overall, malignancy risk was observed to be the highest (93.3%) in Category 6 and lowest (3.0%) in nonneoplastic category. Conclusions: The six-tier diagnostic categories of the Milan classification scheme help in segregating patients with salivary gland lesions into the management categories of follow-up, conservative surgery, and radical surgery with/without chemotherapy.
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Clinicopathological correlation of cancer stem cell markers Oct-4 and CD133 expression as prognostic factor in malignant lesions of gallbladder: An immunohistochemical study p. 384
Naseem Fatima, Anand Narain Srivastava, Jaya Nigam, Nishi Tandon, Rumana Ahmad, Vijay Kumar
DOI:10.4103/IJPM.IJPM_134_19  PMID:31361225
Background: Gallbladder cancer (GBC) is the most frequent biliary tract cancer, with high morbidity and poor prognosis, and shows early metastasis and invasiveness. No reliable biomarkers are available for detection of GBC progression. Aim: To investigate the immunohistochemical expression of Oct-4 and CD133 in malignant and nonneoplastic lesions of gallbladder and to analyze the clinical significance of the expressions related to clinicopathological parameters. Settings and Design: This is a prospective case control study, conducted in medical college background. Materials and Methods: A total of 103 cases of gallbladder were grouped into malignant lesions (n = 48) and nonneoplastic lesions (simple epithelial hyperplasia; n = 35 and chronic cholecystitis; n = 20). All tissue samples were evaluated for expression of Oct-4 and CD133 using immunohistochemistry in an effort to elucidate the correlation between their expressions with clinicopathological parameters. Statistical Analysis: The final score was calculated by multiplying the intensity to the percentage of positive cells. The scores ≥2 were considered as positive. Results: Significant positive correlation of higher expression levels of Oct-4 and CD133 were observed in malignant as compared to nonneoplastic lesions of gallbladder (P < 0.0001). High expression of Oct-4 and CD133 were significantly associated with tumor grading (Oct-4, P = 0.04; CD133, P = 0.02), staging (Oct-4, P = 0.03; CD133, P = 0.02), and liver metastasis (Oct-4, P = 0.01; CD133, P = 0.007). Significantly reduced survival was observed with high expression of Oct-4 (P = 0.002). No significant correction was observed between CD 133 and survival. Conclusion: This study revealed that high expression level of Oct-4 may provide a new insight for the prognosis of the disease in terms of clinical staging and grade.
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Development of an efficient in vivo cell-based assay system for monitoring hepatitis C virus genotype 4a NS3/4A protease activity p. 391
Mohamed M Naguib, Mohamed R Mohamed, Mohamed A M. Ali, Amr M Karim
DOI:10.4103/IJPM.IJPM_774_18  PMID:31361226
Background: Hepatitis C virus (HCV) represents a serious worldwide healthcare problem. No protective vaccines against HCV have been developed yet due to the fact that HCV is rapidly mutable, allowing the virus to escape from the neutralizing antibodies. Understanding of HCV was initially hampered by the inability to achieve viral replication in cell culture. Given its essential roles in viral polyprotein processing and immune evasion, HCV NS3/4A protease is a prime target for antiviral chemotherapy. We aimed to establish in vivo cell-based assay system for monitoring the activity of NS3/4A protease from HCV genotype 4a, the predominant genotype in Egypt, and the Middle East. Furthermore, the developed system was used to evaluate the inhibitory potency of a series of computer-designed chemically-synthesized compounds against NS3/4A protease from HCV genotype 4a. Materials and Methods: Native as well as mutant cleavage sites to NS3/4A protease were cloned in frame into β-galactosidase gene of TA cloning vector. The target specificity of HCV NS3/4A was evaluated by coexpression of β-galactosidase containing the protease cleavage site with NS3/4A protease construct in bacterial cells. The activity of β-galactosidase was colorimetrically estimated in the cell lysate using orthonitro phenyl β-D-galactopyanoside (ONPG) as a substrate. Results and Conclusions: We successfully developed an efficient cell-based system based on the blue/white selection of bacterial cells that are able to express functional/nonfunctional β-galactosidase enzyme.
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Duodenal mucosal immune cells in treatment-naive adult patients with celiac disease having different histological grades and controls p. 399
Gaurav P S. Gahlot, Prasenjit Das, Vandana Baloda, Alka Singh, Sreenivas Vishnubhatla, Siddhartha Datta Gupta, Govind K Makharia
DOI:10.4103/IJPM.IJPM_703_18  PMID:31361227
Background: It is hypothesized that the duodenal mucosal damage in patients with celiac disease (CeD) is caused by the mucosa-infiltrating lymphoid cells. This study aimed to analyze the immune effective and regulatory T (Treg) cells in duodenal biopsies from treatment-naive adult patients with CeD having different histological grades and controls. Patients and Methods: Dual-color immunohistochemical staining was done in a total of 234 duodenal biopsies, including 132 controls and 102 adult patients with CeD using CD20, CD3:CD4, CD3:CD8, CD4:FoxP3, CD8:FoxP3, and TCRαβ:TCRγδ antibodies. The density of these lymphoid cells in lamina propria and mucosal epithelium was compared between controls and CeD, with different modified Marsh grades. Results: Densities of CD4+ T cells in lamina propria and CD8+γδ intraepithelial lymphocytes (IELs) were significantly more in biopsies from patients with CeD, than in controls. An increasing linear pattern of IELs, CD3+ T cells, and CD20+ B cells was observed with increasing grades of villous abnormalities. Although CD8+ FoxP3+ Treg cells were significantly more in biopsies from patients with CeD, there was no significant difference in CD4+ FoxP3+ Treg cell infiltrate between both the groups. Conclusion: Our finding in this observational study generates interest to study the local intestinal mucosal immunity in CeD in detail. A study to prove the failure of CD4+ FoxP3+ Treg cell recruitment in CeD and its direct functional impact may yield valuable information regarding loss of mucosal tolerance.
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Role of ERCC1 expression in colorectal adenoma-carcinoma sequence and relation to other mismatch repair proteins expression, clinicopathological features and prognosis in mucinous and non-mucinous colorectal carcinoma p. 405
Abd AlRahman Mohammad Foda, Andrea Palicelli, Abdelhadi Shebl, Renzo Boldorini, Khaled Elnaghi, Amira K ElHawary
DOI:10.4103/IJPM.IJPM_684_18  PMID:31361228
Background: There are several DNA repair pathways that protect cellular DNA from injury, such as nucleotide excision repair (NER) and mismatch repair (MMR). The protein product of the excision repair cross-complementation group 1 (ERCC1) gene plays a pivotal role in NER. The exact relationship between MMR proteins and ERCC1 is not well known in colorectal carcinoma (CRC). Aim of the Study: To investigate expression of ERCC1 and MMR proteins in colorectal mucinous carcinoma (MA) and non-mucinous carcinoma (NMA) using tissue microarray technique. Material and Methods: We studied tumor tissue specimens from 150 patients with colorectal mucinous (MA) and non-mucinous adenocarcinoma (NMA). Tissue microarrays were constructed using modified mechanical pencil tips technique and immunohistochemistry for ERCC1, MLH1, MSH2, MSH6, and PMS2. Results: NMA showed a significantly more frequent aberrant cytoplasmic expression than MA while MA showed a more frequent intact nuclear expression than NMA. There were no significant differences between the NMA and MA groups in the expression of MMR proteins. In NMA cases, ERCC1 expression was significantly related to MMR status while was not significantly related in MA cases. ERCC1 expression was not significantly related to overall and disease-free survival in both NMA and MA groups. Conclusion: this study is the first to investigate the relation between MMR status and ERCC1 expression in colorectal MA and NMA. ERCC1 expression was significantly related to MMR status only in NMA cases. Hence, the current study emphasizes that further research about the relation between various DNA repair pathways is needed.
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Assessment of appendix carcinoid tumors: A retrospective study p. 413
Servet Kocaöz, Gülay Turan
DOI:10.4103/IJPM.IJPM_390_18  PMID:31361229
Aim: This study aimed to analyze the frequency of carcinoid tumor, the applied treatments to cure it, and the survival periods of the patients thereafter in a city located in the west of Turkey, Balıkesir. Materials and Methods: The data for this study were gathered by the careful retrospective analysis of 6369 files of patients who underwent an appendectomy operation during the time span of January 2011 and December 2017 in Balıkesir Atatürk City Hospital. Results: The results revealed that among the patients who underwent appendectomy, 17 carcinoid tumor cases, 16 of which with combine classic carcinoid and 1 with goblet cell carcinoid, were recorded. It was discovered that about two times more carcinoid tumors were found in females compared to males. It was also revealed that in the 14 (82.4%) of the patients, the diameter of the tumor was <1 cm. Since the diameter of the tumor is <2 cm in classic carcinoid cases, these patients were only observed. Moreover, within the 5-year follow-up period, no recurrence or progress of carcinoid syndrome was detected. Furthermore, this study found out that the 5-year-survival rate of all the carcinoid tumor patients involved in the study was 100%. Conclusions: It is highly important that the histopathological diagnosis of the patients after appendectomy be carefully followed as the appendicitis carcinoid tumors which are encountered more commonly in women than in men are asymptomatic and can only be incidentally detected.
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Role of CD71 in acute leukemia– An immunophenotypic marker for erythroid lineage or proliferation? p. 418
Seema Acharya, Pooja Sharma Kala
DOI:10.4103/IJPM.IJPM_604_18  PMID:31361230
Background: CD71 or Transferrin receptor is expressed on the surface of erythroid lineage cells. CD71 expression has been found to be significantly increased in rapidly proliferating cells. Methods: This cross-sectional study included 37 bone marrow samples of acute leukemia cases diagnosed between October 2016 to April 2018. The samples were analysed on BD FACS Canto II. We evaluated the expression of CD71 on leukemic blasts and compared median fluorescent intensities (MFI) of blasts in different types of acute leukemias. Results: The 37 cases comprised of 21 Acute Myeloid Leukemia (AML), 13 B-Acute Lymphoblastic Leukemia (B-ALL), 2 T- Acute Lymphoblastic Leukemia (T-ALL) and 1 mixed phenotypic acute leukemia (MPAL), T/Myeloid. CD 71 expression was noted in 70.3% (n= 26/37) of acute leukemia cases. CD71 expression was most commonly observed in AML (n= 15/21;71.4%), followed by B-ALL (n= 9/13;69.2%) and T-ALL (n= 1/2;50%). Single case of MPAL revealed blasts positive for CD71. MFI of leukemic blasts of single CD71 positive T-ALL was found to be highest, followed by AML, MPAL (T/Myeloid) and least in B ALL. Of the AML cases, the blasts of AML-M6, acute promyelocytic leukemia and AML-M1 showed higher CD71 expression in terms of MFI. Conclusions: Surface CD71 expression is not only found in erythroid lineage cells, but also in proliferating cells. CD71 MFI is highest in T lymphoblasts followed by leukemic erythroblasts, myeloblasts and least in B lymphoblasts.
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A novel approach to decalcification in histopathology laboratory: An adaptation from the Hammersmith protocol Highly accessed article p. 423
Prita Pradhan, Nachiappa Ganesh Rajesh, Bhawana Ashok Badhe, Kalaivizhi Ilanchezian, Dharmalingam Manimehalai, Arikkotil Jyothish
DOI:10.4103/IJPM.IJPM_853_18  PMID:31361231
Aim: Utility of modified Hammersmith protocol in the deacalcification and/or softening of tissues and samples in a histopathology laboratory were studied. The object of the study was to prepare a novel method for softening/decalcifying tissue for histopathology. Materials and Methods: All the hard tissues received in the histopathology section were received in 10% neutral buffered formalin and then placed in freshly prepared combination of 10 mL of concentrated formaldehyde and 5 mL of 10% formic acid in 85 mL distilled water was used for decalcification. The tissue was checked for evidence of adequate decalcification/softening every 6 hours. Those which were decalcified/softened were sent for routine tissue processing and staining, while those which were not, were again placed in formalin. The process was repeated until the tissue was ready for further processing. The routine sections of these slides were reviewed for morphology and stain quality along with special stains and immunohistochemistry performed. The time taken for decalcification, the variables most likely to affect decalcification, the morphology and staining characteristics were documented. Statistical analysis was done to determine the effect of softening/decalcification process on each variable. Results: A total of 201 blocks in 119 specimens from humans including 61 males and 58 females were studied. Time taken was found to have a significant correlation only with the nature of the tissue (bone vs nonbone) and not with any other parameter viz. age, gender, specimen size, type of bone, and nature of pathology. Conclusion: This novel and modified method has circumvented the common problems of overdecalcification, preserved morphology, and produced consistent results without interfering with special stains and immunohistochemistry.
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BRIEF COMMUNICATIONS Top

Thoracopagus: Autoptic and radiologic study of 2 cases p. 430
Bappa Mandal, Nelofar Islam, Suchandra Mukherjee, Uttara Chatterjee
DOI:10.4103/IJPM.IJPM_716_18  PMID:31361232
Conjoined twins are a very rare complication of monozygotic twinning, most common being thoracopagus. Here we report about two cases of thoracopagus male twins illustrating the autopsy details of one case and the prenatal magnetic resonance imaging (MRI) details of another case. While the first case was misdiagnosed as separate twins on antenatal USG, only to be later confirmed as thoracopagus twins after birth, the antenatal MRI done in the second case helped in accurate detection of thoracopagus twins. Bilateral peripheral cortical cysts with dysplasia was noted in one of the twins of the first case, which has not been reported earlier in conjoint thoracopagii. Early prenatal diagnosis of conjoined twins is essential for better counselling of parents regarding post natal surgical management or termination of pregnancy. Importance of prenatal MRI for accurate detection of these cases is thereby highlighted.
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ROS-1 re-arrangements and c-MET amplifications in adenocarcinoma lung: A tertiary care center study from North India p. 433
Saumya Shukla, Rahul K Pandey, Surya Kant, Rajiv Garg, Nuzhat Husain
DOI:10.4103/IJPM.IJPM_754_18  PMID:31361233
Background: C-ros oncogene 1, receptor tyrosine kinase (ROS 1) proto-oncogene 1, receptor tyrosine kinase (ROS-1) fusions are potent oncogenic drivers and these re-arrangements promote signal transduction programs leading to uninhibited cell survival and proliferation identified in 1–2% of cases of nonsmall-cell lung cancer. Mesenchymal epithelial transition factor (MET) receptor tyrosine kinase and its ligand are predominantly involved in epithelial mesenchymal transition and tissue regeneration. The MET amplification and overexpression is oncogenic in 3–7% cases. The objectives of this study were to identify the frequency of ROS-1 and c-MET protein expression in adenocarcinoma lung and to correlate it with the clinicopathological parameters and to analyze the histomorphology of cases that harbor the characteristic mutations (c-MET and ROS-1). Materials and Methods: Study group comprised a prospective cases series of 90 cases of adenocarcinoma lung. ROS-1 protein expression was determined by immunohistochemistry using the D4D6 rabbit monoclonal antibody (Cell Signaling, Danvers, MA) and c-MET protein expressed was analyzed using the SP-44 clone (Ventana Medical Systems). Results: c-MET protein expression was identified in 33.33% cases (n = 30/90) with statistically significant thyroid transcription factor-1 (TTF-1) positivity. ROS-1 protein expression was detected in 3.33% cases (n-3/90), in biopsies from the respiratory tree with TTF-1 expression. Conclusion: This is the first study from the Indian subcontinent to identify the frequency of ROS-1 re-arrangements and MET amplification in the Indian population. The availability of targeted therapy that has a significant impact on survival makes it essential to detect these less frequent mutations.
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Kimura disease with nephrotic syndrome in a child- A rare association p. 437
Anusree Majumder, Debraj Sen
DOI:10.4103/IJPM.IJPM_517_18  PMID:31361234
Lymphadenopathy along with various systemic manifestations is commonly encountered in pediatric patients, tuberculosis being the commonest etiology. Occasional patients may present a diagnostic conundrum. Here, the authors report an unusual manifestation of Kimura disease (KD) presenting as nephrotic syndrome associated with mesenteric lymphadenitis in an 11-year-old male child. KD is a chronic inflammatory disorder of unknown etiology. It typically affects young adult males in the age range of 27-40 years and usually presents as painless itchy nodular masses in the head and neck region. The involvement of mesenteric lymph nodes along with a very young age of presentation makes it a rare case, posing a diagnostic challenge for the unsuspecting physician.
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Light chain myeloma: A brief report from India p. 441
Neha Singh, Narendra Agrawal, Radhika Sekhri, Anurag Mehta, Dushyant Kumar, Gayatri Vishwakarma, Rayaz Ahmed, Dinesh Bhurani
DOI:10.4103/IJPM.IJPM_385_18  PMID:31361235
Light chain myeloma (LCM) has a reported worldwide incidence of approximately 15%–20% among all multiple myeloma (MM) patients. Few western studies have shown strong correlation of LCM with anemia, higher International Staging System scores, proclivity to renal failure, elevated lactate dehydrogenase levels, raised serum-free light chain ratio, higher frequency of extramedullary plasmacytomas, and poorer overall survival, attributable probably to lack of differentiation and skeletal destruction. The primary aim of this retrospective observational study was to define the clinical and hematological characteristics as well as prognostic outcome of Indian LCM patients in comparison with the IgG and IgA subtypes. Patients were defined according to the International Myeloma Working Group diagnostic criteria 2016 and staged as per the International Staging System. Out of 104 patients of newly diagnosed MM in which results of serum immunofixation (IFE) were available, 65 were of IgG isotype (62.5%), 15 had IgA (14.4%), and 24 had light chain myelomas (LCMs) (23.1%). It was observed that LCM patients significantly correlated with hypercalcemia and higher serum-free light chain ratios, whereas IgA patients were strongly associated with anemia and lower serum albumin levels. However, no difference was found among the three subgroups in terms of serum lactate dehydrogenase levels, proclivity to renal failure, presence of lytic bone lesions, prognostic scoring, pretransplant chemosensitivity, and progession-free survival (1 year). Thus, it may be concluded that Indian LCM patients have significantly different clinico-hematological profile in comparison with other published studies worldwide. Also, their prognostic outcomes are not worse when compared with patients of other protein isotypes, probably due to standardized treatment regimens applied.
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CASE REPORTS Top

Pigmented variant of pleomorphic xanthoastrocytoma - A rare long-term epilepsy associated neoplasm p. 445
Rajalakshmi Poyuran, Nirupam Moudgil, Arivazhagan Arimappamagan, Rose D Bharath, Anita Mahadevan
DOI:10.4103/IJPM.IJPM_723_18  PMID:31361236
Pleomorphic xanthoastrocytoma (PXA) is an uncommon, long-term epilepsy associated tumor of young adults. Its pigmented variant is exceedingly rare, with only five previously reported cases on record. We report the sixth case of pigmented PXA in a 24-year-old lady presenting with long-standing seizures. The MRI revealed a solid cystic lesion located in the right medial temporal lobe. Histopathologically, the superficially located tumor showed typical features of PXA with melanin-laden astrocytic component and was negative for V600E-mutant BRAF. The histogenesis is discussed.
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Xanthomatous hypophysitis: A rare case report with review of literature p. 448
Hema Kini, Ranjitha Rao, Muralidhar Pai
DOI:10.4103/IJPM.IJPM_319_18  PMID:31361237
Hypophysitis is classified into primary and secondary. Xanthomatous hypophysitis is one of the rare types of primary hypophysitis. A 55-year-old female presented with headache, vomiting, and blurring of vision. She also had endocrine dysfunction in the form of low serum T3, T4, and low cortisol levels. MRI scan showed a sellar expansile lesion suggestive of pituitary macroadenoma. Microscopy showed pituitary tissue replaced by inflammatory infiltrate made up of foamy histiocytes arranged in sheets along with lymphoplasmacytic infiltrate. Interspersed areas of fibrosis, hyalinization, few congested and sclerosed blood vessels were seen. Compressed residual pituitary tissue was identified at the periphery. Xanthomatous hypophysitis is a rare entity which can mimic as pituitary adenoma both clinically and radiologically. Accurate diagnosis at an early stage with postsurgical steroid therapy may help to prevent permanent pituitary damage.
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Nonparameningeal alveolar rhabdomyosarcoma, with cytohistological features: A case report p. 451
Neelam Sood, Arun K Haldia
DOI:10.4103/IJPM.IJPM_471_18  PMID:31361238
Rhabdomyosarcoma (RMS) is a tumor arising from primitive mesenchymal cell with tendency for myogenesis. WHO classification categorizes this entity as embryonal, alveolar, spindle cell/sclerosing, and pleomorphic subtypes removing botryoid as a separate entity. The alveolar variant has worse prognosis and the cytological features of this entity are similar to embryonal type with little variations. This case report describes the cytohistological features of alveolar RMSfrom a 9-year-old child with nonparameningeal location.
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Primary triple head and neck tumors: Laryngeal squamous cell carcinomas, Kaposi's sarcoma, and non-Hodgkin's lymphoma p. 454
Metin Yildirim, Seyda Belli, Sule Ozsoy, Umit Taskin
DOI:10.4103/IJPM.IJPM_555_18  PMID:31361239
Simultaneously triple head and neck malignancies are extremely rare. We report a case who had epithelial and mesenchymal malignant tumor with lymphoid malignancy in head and neck area. A patient who is 74 year old male patient presented to the otorhinolaryngology department with severe breathing difficulty due to laryngeal mass. The result of laryngeal biopsy was invasive SCCs, so patient underwent total larygectomy and bilateral level 2,3,4 neck disection operation. Primary 3 different type head and neck tumors were observed with histopathological examination. These were orderly invasive SCCs in larynx, B-cell Non-Hodgkin's lymphoma in tongue root and one lymph node of left neck dissection material, Kaposi's sarcoma in one lymph node of right neck dissection material. Although synchronous head and neck tumors occurs very rare with laryngeal carcinoma, the neck dissection materials should be researched for synchronous tumors.
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Nephropathic cystinosis presenting with uveitis: Report of a “Can't See, Can't Pee” situation p. 457
Smita Mary Matthai, Shibu Jacob, Mandeep S Bindra, Vinoi George David, Santosh Varughese
DOI:10.4103/IJPM.IJPM_623_18  PMID:31361240
Nephropathic cystinosis is a rare autosomal recessive lysosomal disease characterized by accumulation of pathognomonic cystine crystals in renal and other tissues of the body. Cystinosis is caused by mutant cystinosin, the cystine transport protein located in lysosomal membranes, leading to systemic deposits of cystine and resultant end organ damage. Cystinosis is rarer in Asians than Caucasians with only a handful of cases reported from India to date. Due to its extreme rarity and clinically insidious presentation in contrast to the infantile form, the diagnosis of juvenile nephropathic cystinosis is frequently delayed or overlooked. Moreover, routine processing and sectioning of paraffin embedded tissues dissolves cystine crystals, making it difficult to diagnose this condition on light microscopic examination alone, mandating electron microscopic (EM) analysis of renal biopsies for an accurate diagnosis of this condition. We describe a case of juvenile nephropathic cystinosis presenting with uveitis and photophobia in a 17-year-old Indian male, diagnosed after EM examination of the patient's renal biopsy for evaluation of nephrotic syndrome. While highlighting the diagnostic utility of EM, we describe a few histopathologic clues which can prompt inclusion of EM analysis of renal biopsies in this setting.
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Hibernoma: A missed diagnosis!! p. 461
Sunita D Patil, Abdul R Sheik, Vanmalini Tewari, Deepti Mutreja
DOI:10.4103/IJPM.IJPM_577_18  PMID:31361241
Hibernoma is a rare, benign soft tissue tumor described in <200 case reports/case series. It is slow-growing, painless, and commonly mistaken for lipoma or liposarcoma. Histopathological diagnosis is must for confirmation. Total excision is the treatment of choice. We present a rare case of hibernoma of thigh, which was initially misdiagnosed as atypical lipomatous tumor/well-differentiated liposarcoma on imaging, which turned out to be a hibernoma on histopathological examination.
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HPV related cloacogenic carcinoma of the anal canal with divergent histomorphology p. 464
Radhika Yajaman Gurumurthy, N Siddharth Shankar, CS Mohan Raj, N Sriram
DOI:10.4103/IJPM.IJPM_305_18  PMID:31361242
Cloacogenic carcinoma also known as basaloid squamous cell carcinoma is a rare anorectal tumor presenting with varied histomorphology. In this case report, we describe a case of 58-year-old man presenting with bleeding per rectum and pain. A polypoidal tumor was noted in anal canal which on microscopy was diagnosed to be cloacogenic carcinoma with transitional carcinoma-like, basaloid and mucinous patterns. An unusual finding in the present case was the presence of signet ring cells in the mucinous areas. A thorough knowledge of the wide histomorphological spectrum of the tumor and a limited IHC panel are crucial for the diagnosis. Here, we also present a review of literature and describe in detail the origin and histopathological features of the tumor.
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Extranodal histiocytic sarcoma mimicking colorectal lymphoma: Case report and review of literature p. 467
Subhashis Mitra, Amiya Jhunjhunwala, Paromita Mukherjee
DOI:10.4103/IJPM.IJPM_412_18  PMID:31361243
Histiocytic sarcoma is a rare malignant neoplasm that demonstrates mature histiocytic traits as characterized by immunohistochemistry. We report a case of extranodal histiocytic sarcoma (ENHS) of colon in a 56-year-old man presenting with gastrointestinal symptoms. Radiological findings were indicative of lymphoma or diffuse metastatic disease in colon. Histopathology of colectomy specimen was suggestive of ENHS, and immunohistochemical studies confirmed the uncommon diagnosis. The patient refused further therapy and succumbed to systemic complications of metastatic disease within a month of diagnosis. There have only been seven previous reports in world literature of ENHS involving large intestine.
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A rare case of primary cutaneous diffuse large B-cell lymphoma, leg type in a patient with chronic lymphedema of the leg p. 470
Basavaraj Vijaya, Saravu RamaBhatta Narahari, Mysore Krishna Shruthi, Guruprasad Aggithaaya
DOI:10.4103/IJPM.IJPM_581_18  PMID:31361244
A rare complication of chronic lymphedema is development of cutaneous malignant tumors particularly cutaneous lymphomas. We report a case of primary cutaneous diffuse large B-cell lymphoma, leg type, associated with chronic lymphedema of the lower limbs. A literature review identified 12 additional cases of primary cutaneous lymphomas associated with chronic lymphedema. Chronic lymphatic stasis may cause a local dysfunction of the immune system that predisposes to the induction of neoplasia, cutaneous lymphomas in particular.
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Cytological findings of NK/T-cell lymphoma in pericardial effusion: A case report with a review of the literature p. 473
Yeeun Han, Kyung Ha Kim, In Ho Choi
DOI:10.4103/IJPM.IJPM_540_18  PMID:31361245
Extranodal Natural Killer/T-cell lymphoma (ENKL) is an aggressive NK or cytotoxic T-cell neoplasm. The cytological features of NK/T-cell lymphoma have been rarely described, mainly focusing on the fine needle aspiration cytology from lymph nodes or soft tissue, except for a few cases focused on body fluid. A 46-year-old man visited the hospital due to generalized weakness and weight loss. Three months prior, computed tomographic scan revealed mesenteric panniculitis and reactive lymph nodal enlargement, as well as a mildly thickened left adrenal gland, suggesting an inflammatory condition. About 100 days later, marked enlargement of both adrenal glands with pericardial effusion was noted. The pericardial effusion contained medium-sized atypical lymphocytes, suspicious for malignant lymphoma, and the left adrenal mass was histologically confirmed as ENKL on biopsied specimen. Herein, we describe the cytological features of NK/T-cell lymphoma in body fluid cytology along with a review of the literature.
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Wolfram syndrome: A rare case report p. 477
Anitha Padmanabhan, Aditi Parihar, Urmi S C. Vartak, Nitin M Gadgil
DOI:10.4103/IJPM.IJPM_397_18  PMID:31361246
We present an autopsy case of a 19 year old male admitted for breathlessness and oliguria. He was diabetic since 7 years of age and was on insulin. Patient was on testosterone and anti hypertensives. He was diagnosed of hypocontractile bladder and congenital bilateral megaureter with vesico-ureteric reflux 2 years back. History of hemiparesis 2 years back. CT scan of the brain showed a right fronto- parietal healed infarct. At autopsy, bilateral kidneys showed coarse granularity and scarring. Pelvicalyceal system and both ureters were dilated. A right sided intrabdominal testes was identified. On histology, kidney showed features of diabetic nephropathy and pancreas showed decreased number of islet cells. Correlating the clinical, laboratory and autopsy parameters, our case satisfies the EURO-WABB criteria (1major+2minor) for diagnosis of Wolfram Syndrome, even though genetic confirmation could not be done.[1],[2]
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IMAGES Top

Inverted mucoepidermoid papilloma of conjunctiva: A rare histological entity p. 481
Vikram Singh, Amber Parwaiz, Bishan Das Radotra, Manpreet Singh
DOI:10.4103/IJPM.IJPM_276_18  PMID:31361247
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A rare case of nocardial pachymeningitis and osteomyelitis of frontal bone in an immunocompetent young patient p. 483
Valentina Liverotti, Mauro Dobran, Davide Nasi, Alessandro Di Rienzo
DOI:10.4103/IJPM.IJPM_694_17  PMID:31361248
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Primary pulmonary meningioma p. 486
Pradeep Vaideeswar, Jayashri Chaudhari
DOI:10.4103/IJPM.IJPM_310_18  PMID:31361249
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Composite lymphoma comprising mantle cell lymphoma and Epstein-Barr virus positive classic Hodgkin lymphoma: A rare case p. 488
Shobhna Sharma, Vishal Singh, Dipti Bisaria, Rajiv Tangri
DOI:10.4103/IJPM.IJPM_34_18  PMID:31361250
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Primary epithelioid angiosarcoma of the spleen p. 491
Shufei Wei, Yongliang Han, Ting Li, Xiao Xu
DOI:10.4103/IJPM.IJPM_170_18  PMID:31361251
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Bone marrow granuloma in a child with pyrexia of unknown origin: A clue for diagnosis of brucellosis p. 493
Renu Suthar, Deepak Bansal, Deepti Suri, Prashant Sharma, Pallab Ray
DOI:10.4103/IJPM.IJPM_7_18  PMID:31361252
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Extracranial metastasis in a IDH- wild type glioblastoma p. 495
Richa Goyal, Preeti J John, Shweta Bhatoa, Raman Arora
DOI:10.4103/IJPM.IJPM_432_18  PMID:31361253
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Multifocal recurrent conjunctival melanoma with diffuse primary acquired melanosis p. 497
Richa Goyal, Preeti Joseph John, Yogesh Gauba, Anjana Kumari
DOI:10.4103/IJPM.IJPM_15_18  PMID:31361254
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A rare case of recurrent vascular aneurysmal bone cyst of the orbit in a 4-year-old child p. 499
Urmi Mukherjee, Aarti Tyagi, Anuj Khurana, Arun Saroha, Himanshu Arora
DOI:10.4103/IJPM.IJPM_317_18  PMID:31361255
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Ameloblastic carcinoma: A diagnostic dilemma p. 501
Sankalp Sancheti, Puneet Kaur Somal, Sourav Sarkar
DOI:10.4103/IJPM.IJPM_121_18  PMID:31361256
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An unusual presentation of mediastinal myelolipoma: A Radiologico-Pathological correlation p. 503
Ritesh Sachdev, Ruchika Kumar Goel, Shalini Goel, Kulbir Ahlawat
DOI:10.4103/IJPM.IJPM_295_18  PMID:31361257
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A case of multifocal EBV associated gastric carcinoma with focal squamous differentiation p. 505
Kim Ji-Hoon, Eom Dae-Woon
DOI:10.4103/IJPM.IJPM_174_18  PMID:31361258
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Hepatocellular carcinoma presenting as rib lesion: A diagnostic dilemma p. 507
Subhashis Mitra, Amiya Jhunjhunwala, Hema Chakraborty
DOI:10.4103/IJPM.IJPM_413_18  PMID:31361259
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Fibrous hamartoma of infancy with pseuodoangiomatous pattern p. 509
Anshul Singh, Preeti Singh, Mamta Bhatt, Vatsala Misra
DOI:10.4103/IJPM.IJPM_272_18  PMID:31361260
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Hodgkin's lymphoma in a patient with acute lymphoblastic leukemia while on maintenance: A rare second malignant neoplasm p. 510
Sugeeth M Thambi, Rony Benson, Sreejith G Nair, Jayasudha A Vasudevan, Rekha A Nair
DOI:10.4103/IJPM.IJPM_87_18  PMID:31361261
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Sequential surprises; Non endemic mycoses revealing immunodeficiency p. 512
Mansoor C Abdulla, Syed Mustaq, Ram Narayan
DOI:10.4103/IJPM.IJPM_721_17  PMID:31361262
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Biofilms: Fungal perspective p. 514
Nidhi Singla, Neelam Gulati, Jagdish Chander
DOI:10.4103/IJPM.IJPM_20_18  PMID:31361263
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NEW HORIZON Top

Catechism (Quiz 5) p. 516
Bharat Rekhi
DOI:10.4103/0377-4929.263509  PMID:31361264
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