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  Table of Contents    
CASE REPORT  
Year : 2016  |  Volume : 59  |  Issue : 2  |  Page : 223-226
Sclerosing angiomatoid nodular transformation of spleen masquerading as carcinoma breast metastasis: Importance of splenic biopsy in obviating splenectomy


1 Department of Endocrinology, Post Graduate Institute of Medical Education and Research and Dr. Ram Manohar Lohia Hospital, Lucknow, Uttar Pradesh, India
2 Department of Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India
3 Department of Histopathology, Sir Ganga Ram Hospital, New Delhi, India

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Date of Web Publication9-May-2016
 

   Abstract 

Metastasis to spleen is rare and usually occurs in the setting of extensive multivisceral metastatic disease. A 60-year-old lady with appropriately managed early breast cancer (breast conservative surgery for Grade-2 infiltrative ductal carcinoma [0.7 cm × 0.5 cm diameter]) in 2006, was detected to have splenic incidentaloma (1.4 cm × 0.8 cm) in November 2012, which was fluoro-deoxy-glucose (FDG) avid on positron emission tomography. Fine needle spiration cytology (FNAC) was normal. More than doubling of lesion size by March 2015 (3.83 cm × 3.03 cm diameter) with persistent FDG positivity lead to Tru-Cut biopsy of spleen, which revealed multiple nodular areas of congestion and hemorrhage, composed of sinusoids (CD31+, CD8+, and CD34), capillaries (CD31+, CD8, and CD34+), and small veins (CD31+, CD8, and CD34), fibrosis, around these nodular areas along with numerous histiocytes (CD68+) consistent with diagnosis of sclerosing angiomatoid nodular transformation (SANT) of spleen. SANT is a benign, reactive vascular transformation of spleen, notorious to mimic metastasis, the cause of its 18FDG avidity due to its rich content of macrophages and myofibroblasts, usually diagnosed postsplenectomy. This report highlights the importance of splenic biopsy over FNAC is diagnosing splenic incidentalomas, which can help prevent splenectomy, and hence the associated morbidity. This is the first report of SANT in carcinoma breast mimicking metastasis.

Keywords: Carcinoma breast, incidentaloma, metastasis, positron emission tomography, sclerosing adenomatoid nodular transformation, spleen

How to cite this article:
Dutta D, Sharma M, Mahajan V, Chopra P. Sclerosing angiomatoid nodular transformation of spleen masquerading as carcinoma breast metastasis: Importance of splenic biopsy in obviating splenectomy. Indian J Pathol Microbiol 2016;59:223-6

How to cite this URL:
Dutta D, Sharma M, Mahajan V, Chopra P. Sclerosing angiomatoid nodular transformation of spleen masquerading as carcinoma breast metastasis: Importance of splenic biopsy in obviating splenectomy. Indian J Pathol Microbiol [serial online] 2016 [cited 2020 Aug 10];59:223-6. Available from: http://www.ijpmonline.org/text.asp?2016/59/2/223/182034



   Introduction Top


Metastasis to spleen, in general, is rare and usually occurs in the setting of late-stage extensive multivisceral metastatic disease from melanoma, lung, ovarian, and gastrointestinal malignancy.[1] Sclerosing angiomatoid nodular transformation (SANT) is a rare, recently recognized, nonneoplastic transformation of spleen, of obscure etiology, documented most commonly in females (female:male = 2:1) >50 years age.[1],[2] Most patients with SANT are asymptomatic and are usually initially detected as a splenic incidentaloma.[1] In view of the suspicion of metastasis (especially in the setting of previous malignancy), most patients undergo splenectomy, and SANT is usually a postoperative histopathologic diagnosis, thus exposing the patient to future risk of fulminant infections related to immunodeficiency secondary to splenectomy.


   Case Report Top


A 60-year-old lady with carcinoma breast diagnosed 9 years back, following mammography and fine needle aspiration (FNA) of suspicious lesion in the right breast, underwent breast conservative surgery along with level-3 axillary lymph node excision in April 2006. Histopathologic diagnosis was Grade-2 infiltrative ductal carcinoma (0.7 cm × 0.5 cm diameter) with clear margins, estrogen-receptor positive, progesterone-receptor positive, Her 2 negative, and without any evidence of nodal metastasis. She received locoregional external beam radiotherapy (45 Gy) followed by tamoxifen for 5 years, stopped in 2011. In November 2012, she was detected to have a hypoechoic splenic incidentaloma (1.4 cm × 0.8 cm diameter) during ultrasonography abdomen for right hypochondriac pain. Sequential ultrasonography (6-monthly) revealed a gradual increase in lesion size, which was 1.8 cm × 1.4 cm in November 2014, when it was detected to be fluoro-deoxy-glucose (FDG) avid (peak standard uptake value 3.8) on positron emission tomography (PET). Subsequent splenic FNA cytology was unremarkable. Further follow-up revealed continuing increase in lesion size. Hence, repeat FDG-PET, done in March 2015, revealed isolated well-defined hypodense lesion in spleen (3.83 cm × 3.03 cm diameter) with mildly increased FDG uptake [Figure 1]. Since the patient refused to consent for splenectomy, a repeat ultrasonography guided FNA followed by Tru-Cut biopsy of spleen was done. FNA cytology was again unremarkable. Histopathology of biopsy specimen was significant for multiple nodular areas of congestion and hemorrhage along with diffuse fibrosis, focal calcification, and hemosiderin deposition (Gamma–Gandy bodies) around these nodular areas. Nuclear atypia, mitosis, and necrosis were absent [Figure 2]. Immunohistochemistry revealed the nodules to be composed of sinusoids (CD31+, CD8+, and CD34), capillaries (CD31+, CD8, and CD34+), and small veins (CD31+, CD8, and CD34) along with numerous histiocytes (CD68+) in the internodular areas consistent with a diagnosis of SANT [Figure 3]. (1). Hence, splenectomy was deferred, and the patient was followed-up conservatively.
Figure 1: (a) Computed tomography abdomen showing hypodense splenic incidentalomas 3.83 cm × 3.03 cm diameter; (b) positron emission tomography and computed tomography showing the splenic incidentalomas to be weakly 18F-deoxy-glucose avid; (c) Positron emission tomography whole body scan showing abnormally increased fluoro-deoxy-glucose uptake only in spleen (solid black arrow). Normal fluoro-deoxy-glucose uptake in brain, myocardium, and bladder is also seen (hollow black arrows)

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Figure 2: (a) Splenic core biopsy showing multinodular appearance (H and E, ×4); (b) high power view of the nodules showing slit-like vascular spaces and many erythrocytes surrounded by fibrosis (hematoxylin and eosin [H and E, ×20]); (c) The intervening stroma showing inflammatory cells, calcifications, and hemosiderin-laden macrophages (H and E, ×20); (d) hemosiderin deposition highlighted by special stain for iron (Pearls Prussian blue, ×10)

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Figure 3: (a) Immunohistochemistry with CD31 showing positively stained small veins arranged in intricate mesh-like pattern (×40); (b) immunohistochemistry with CD34 showing positively stained capillaries (×40); (c) immunohistochemistry with CD8 showing positively stained splenic sinusoids (×40); (d) macrophages in the intervening stroma highlighted with immunohistochemistry for CD68 (×40)

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   Discussion Top


Neoplastic involvement of spleen is rare, (except in case of primary tumors of reticuloendothelial system)[3] due to mechanical factors impeding splenic implantation of neoplastic cells through hematogenous route (sharp angle of splenic artery, high blood flow), lack of afferent lymphatics, impervious nature, and constant contraction of capsule and the inhibitory effect of the splenic microenvironment (strong local immunosurveillance).[3],[4] History of treated carcinoma breast raised the possibility of splenic metastasis in our patient. Factors against metastasis were an early diagnosis of breast cancer (T1aN0M0), appropriate management, long disease-free interval (9 years), slow growth of splenic lesion, and its persistence as a solitary lesion even after 2.5 years follow-up. However, worrying features were doubling of the lesion size over 2.5 years and persistent low-grade FDG avidity. Isolated splenic metastasis from breast carcinoma is extremely rare with only 3 cases reported until date,[3],[5],[6] all of which had a more advanced primary disease with one or more poor prognostic factors such as axillary lymph node involvement, locoregional spread, lack of hormone receptor expression, or strong family history.[3],[5],[6] Lack of willingness for splenectomy lead to guided FNA followed by core biopsy (especially since previous FNA was nondiagnostic) which lead to a diagnosis of SANT. It must be highlighted that splenic biopsy in not a commonly done procedure due to the small associated risk of bleeding and the fear of spillage of tumor cells, if the tumor is malignant. The same should always be discussed with patients to enable them to take an informed decision. In our patient, we safely performed ultrasonography-guided splenic biopsy utilizing a coaxial system, caudocranial approach with closure of the biopsy tract after completing the procedure. Splenic biopsy was justified in our patient as there was a significant preprocedure likelihood of a benign pathology, the desire for conservative management from the patient along with the lack of willingliness for splenectomy. The final diagnosis of SANT, a benign pathology, obviated splenectomy in our patient. One of the serious complications of splenectomy is overwhelming postsplenectomy infection (mortality due to fulminant sepsis: 4.4% in children and 0.9% in adults), secondary to immunosuppression due to loss of body's largest lymphoid tissue reserve, which has a key role in antibody formation.[7] Several reports have suggested percutaneous image-guided biopsy of spleen to be safe and effective.[8],[9],[10] Keogan et al. observed complication rates of <2% following image-guided percutaneous biopsy on 20 patients, which is well within the limits of those associated with biopsies of other abdominal organs.[8] In addition, needle-tract seeding is an exceedingly rare complication with an estimated occurrence rate of <0.01% following percutaneous abdominal fine-needle biopsy.[9],[10]

The most common primary splenic tumors are benign vascular neoplasia of spleen (most commonly hemangioma).[11] SANT has been classically described by Martel et al. (largest series, 25 patients) who believed it to be a reactive process to unknown stimuli including trauma.[1] SANT has previously been described in literature as “cord capillary hemangioma” or “multinodular hemangioma.”[12] Its typical histopathological appearance of vascular nodules having angiomatoid appearance in a fibrous stroma helps differentiating SANT from other vascular lesions of spleen (hamartoma, hemangioma, littoral cell angioma, lymphangioma, and hemangioendothelioma). SANT has been classically reported to be asymptomatic.[1] The pathogenesis of SANT is obscure. It is believed that passive congestion of splenic pulp secondary to trauma or unknown causes leads to sinus endothelial cell damage, fibrin deposition, and inflammation resulting in pseudotumor appearance or SANT.[1] On macroscopic examination of spleen postsplenectomy, SANT has been described as well circumscribed, nonencapsulated, bosselated mass with multiple nodules, and whitish stroma, firmer than rest of the spleen.[1] SANT perhaps represent a one-end spectrum of splenic hamartoma, the cause of the similar immunohistochemistry.[13] Stromal sclerosis in SANT has led to it being linked to IgG4-related sclerosing disease.[13]

On imaging studies (ultrasonography, computed tomography, and magnetic resonance imaging), SANT is usually hypodense with peripheral contrast enhancement, sometimes described to have a hypovascular center with an enhancing rim and radiating vascularized tissue penetrating from the periphery toward the center of the lesion (spoke wheel pattern), especially on delayed postcontrast imaging.[2] This appearance has been attributed to central stellate fibrous stroma with fibrous septa separating angiomatoid nodules.[1],[2] Similar to our patient, there are only 2 previous reports of FDG avidity of SANT.[2],[14] Thacker et al. hypothesized that the abundance of hemosiderin-laden macrophages, myofibroblasts, lymphocytes, and plasma cells, may explain this increased FDG avidity.[2]

This is perhaps the first report of SANT spleen in breast carcinoma, mimicking metastasis. There is only a single report until the date of SANT being diagnosed with percutaneous core biopsy of spleen.[12] In rest all the cases reported until date, SANT was diagnosed postoperatively following splenectomy, due to fear of neoplasm/metastasis. This report intends to highlight that isolated splenic metastasis is extremely rare, the characteristic features of SANT spleen which can mimic metastasis, the need for image-guided splenic biopsy as a definitive alternative to splenectomy in patients with suspected splenic metastasis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Martel M, Cheuk W, Lombardi L, Lifschitz-Mercer B, Chan JK, Rosai J. Sclerosing angiomatoid nodular transformation (SANT): Report of 25 cases of a distinctive benign splenic lesion. Am J Surg Pathol 2004;28:1268-79.  Back to cited text no. 1
    
2.
Thacker C, Korn R, Millstine J, Harvin H, Van Lier Ribbink JA, Gotway MB. Sclerosing angiomatoid nodular transformation of the spleen: CT, MR, PET, and 99(m) Tc-sulfur colloid SPECT CT findings with gross and histopathological correlation. Abdom Imaging 2010;35:683-9.  Back to cited text no. 2
    
3.
Sufficool K, Wang J, Doherty S. Isolated splenic metastasis from carcinoma of the breast: A case report. Diagn Cytopathol 2013;41:914-6.  Back to cited text no. 3
    
4.
Bartolotti M, Franceschi E, Di Battista M, Esposti RD, Castaldini L, Baccarini P, et al. Cytologically confirmed splenic metastases in breast cancer. Future Oncol 2012;8:1495-500.  Back to cited text no. 4
    
5.
Iype S, Akbar MA, Krishna G. Isolated splenic metastasis from carcinoma of the breast. Postgrad Med J 2002;78:173-4.  Back to cited text no. 5
    
6.
Barreca M, Angelini D, Gallo A, Puntillo F, Amodio PM, Fernandes E. Single asymptomatic splenic metastasis of breast carcinoma: Report of a clinical case. G Chir 2001;22:227-8.  Back to cited text no. 6
    
7.
Akkucuk S, Aydogan A, Gokce H, Davran R, Karcioglu M. Splenic hematoma mimicking angiosarcoma: A case report. Case Rep Oncol Med 2012;2012:183458.  Back to cited text no. 7
    
8.
Keogan MT, Freed KS, Paulson EK, Nelson RC, Dodd LG. Imaging-guided percutaneous biopsy of focal splenic lesions: Update on safety and effectiveness. AJR Am J Roentgenol 1999;172:933-7.  Back to cited text no. 8
    
9.
Smith EH. Complications of percutaneous abdominal fine-needle biopsy. Review. Radiology 1991;178:253-8.  Back to cited text no. 9
    
10.
Yarmohammadi H, Nakamoto D, Faulhaber PF, Miedler J, Azar N. Inflammatory pseudotumor of the spleen: Review of clinical presentation and diagnostic methods. J Radiol Case Rep 2011;5:16-22.  Back to cited text no. 10
    
11.
Gutzeit A, Stuckmann G, Dommann-Scherrer C. Sclerosing angiomatoid nodular transformation (SANT) of the spleen: Sonographic finding. J Clin Ultrasound 2009;37:308-11.  Back to cited text no. 11
    
12.
Krishnan J, Danon A, Frizzera D. Use of anti-factor VIII-related antigen (F8) and QBEN10 (CD34) antibodies helps classify the benign vascular lesions of the spleen. Mod Pathol 1993;6:94A.  Back to cited text no. 12
    
13.
Koreishi AF, Saenz AJ, Fleming SE, Teruya-Feldstein J. Sclerosing angiomatoid nodular transformation (SANT) of the spleen: A report of 3 cases. Int J Surg Pathol 2009;17:384-9.  Back to cited text no. 13
    
14.
Lee D, Wood B, Formby M, Cho T. F-18 FDG-avid sclerosing angiomatoid nodular transformation (SANT) of the spleen: Case study and literature review. Pathology 2007;39:181-3.  Back to cited text no. 14
    

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Correspondence Address:
Deep Dutta
Department of Endocrinology, Post Graduate Institute of Medical Education and Research and Dr. Ram Manohar Lohia Hospital, New Delhi - 110 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.182034

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