Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2019  |  Volume : 62  |  Issue : 4  |  Page : 602--604

Myeloid sarcoma of bile ducts presenting as obstructive jaundice – A case report


Ayushi Agarwal1, Tina Dadu2, VP Bhalla3, Veena Malhotra1,  
1 Department of Histopathology, BLK Hospital, Pusa Road, New Delhi, India
2 Department of Hematology, BLK Hospital, Pusa Road, New Delhi, India
3 Department of Digestive and Liver Diseases, BLK Hospital, Pusa Road, New Delhi, India

Correspondence Address:
Veena Malhotra
Department of Histopathology, BLK Hospital, Pusa Road, New Delhi
India

Abstract

Myeloid sarcoma is an extra medullary manifestation of acute myeloid leukemia (AML). Primary involvement of the biliary tract with myeloid sarcoma presenting as obstructive jaundice without evidence of leukemia is very rare. Here we present a case of 72 year old lady, who initially presented with features of biliary obstruction and was clinically considered as cholangiocarcinoma. She was diagnosed as myeloid sarcoma involving right and left hepatic duct, common bile duct (CBD) on histopathological and immunohistochemistry (IHC) examination after surgical resection. Since she did not show evidence of leukemia on peripheral blood and bone marrow examination treatment was deferred. However, she developed full blown picture of AML within two months and succumbed to her disease. We conclude that obstructive jaundice can be the presenting symptom in myeloid sarcoma without evidence of AML in peripheral blood and bone marrow. However, these cases have to be treated aggressively to obtain remission.



How to cite this article:
Agarwal A, Dadu T, Bhalla V P, Malhotra V. Myeloid sarcoma of bile ducts presenting as obstructive jaundice – A case report.Indian J Pathol Microbiol 2019;62:602-604


How to cite this URL:
Agarwal A, Dadu T, Bhalla V P, Malhotra V. Myeloid sarcoma of bile ducts presenting as obstructive jaundice – A case report. Indian J Pathol Microbiol [serial online] 2019 [cited 2021 Jun 23 ];62:602-604
Available from: https://www.ijpmonline.org/text.asp?2019/62/4/602/269069


Full Text



 Introduction



Myeloid sarcoma also known as granulocytic sarcoma or chloroma is a tumor mass consisting of myeloid blasts occurring at extramedullary sites. Its detection is considered equivalent to diagnosis of AML. Primary involvement of the biliary tract with myeloid sarcoma is very rare. It can be clinically misdiagnosed as cholangitis or cholangiocarcinoma.

 Case Presentation



A 72-year-old lady was admitted with complaints of itching, icterus, passage of dark colored urine since 20 days. Liver function tests showed serum direct bilirubin of 13.4 mg/dL and elevated AST, ALT, and ALP liver enzymes. Ultrasonography was suggestive of common bile duct (CBD) block. Multiphase contrast enhanced computed tomography showed extra hepatic biliary obstruction with circumferential thickening of CBD and common hepatic duct (CHD) extending up to the confluence along with enlarged portal lymph node. CA19.9 level was 717.60 u/mL and CEA level was 3.23 ng/ml. Endoscopic ultrasound guided fine needle aspiration of portal lymph node showed few atypical cells. Patient was clinically considered as case of cholangiocarcinoma. Patient underwent exploratory laparotomy, cholecystectomy, and CBD excision with Roux-en-Y hepatico-jejunostomy. Resection margins were sent for frozen section for margin clearance. Margins showed atypical lymphoid cells. However, definite diagnosis was deffered. Subsequently paraffin embeded sections from left, right, and common hepatic ducts and CBD showed sheets of large atypical possibly lymphoid cells with convoluted nucleus [Figure 1]a. On IHC, atypical cells showed diffuse positivity for leukocytic common antigen (LCA) [Figure 1]b. They were negative for PAN CK, CD3, CD20, CD79a, PAX -5, CD56, and ALK-1. IHC for myeloperoxidase (MPO) showed diffuse cytoplasmic positivity in atypical cells [Figure 1]c. Proliferative index was about 50% [Figure 1]d. A diagnosis of myeloid sarcoma was made. One of the lymph nodes also showed focal area with infiltration by abnormal cells with similar morphology as seen in the biliary ducts. Bone marrow aspiration and biopsy at that time did not show evidence of blasts cells. Biopsy was hypercellular (55% cellularity). However, no increase in blast cells was seen even on CD34 immunostain.{Figure 1}

Karyotyping done at this time showed normal karyotype. As the patient was asymptomatic after surgery and peripheral blood and bone marrow did not show evidence of leukemia, the patient was reluctant for further treatment. However, after 2 months, patient showed bicytopenia and 98% blasts in the peripheral blood. The patient was reluctant for a bone marrow and karyotyping. However, flow cytometry was done on peripheral blood, which showed immunophenotype consistent with AML (CD38+, CD13+, CD33+, CD117+, and MPO+). Molecular studies by polymerase chain reaction (PCR) followed by fragment analysis showed NPM 1 positivity. Other markers done for AML genetic profile, including BCR- ABL, inv16, t(8:21), and FLT3 mutation done by PCR were negative. In spite of an isolated NPM1 positivity, which is usually associated with good prognosis, patient went rapidly downhill with almost 0% mature cells in peripheral blood and expired within 10 days before any therapy could be started.

 Discussion



Obstructive jaundice as initial presentation of myeloid sarcoma is very rare. Myeloid sarcoma can occur concurrently in cases of AML, cases of AML in remission after treatment, chronic myeloid leukemia in blast crisis, and in myelodysplastic syndrome.[1] Neiman et al. reported 61 cases of granulocytic sarcoma of which 15 (22%) occurred with no known disease and 13 out of these developed leukemia within 1–49 months after diagnosis (mean: 10.5 months).[2]

Obstructive jaundice as presenting symptom of AML is rare. This can be due to leukemic infiltration of biliary tract or by compression of bile ducts by mass lesion. Infiltration of bile ducts by blast cells causing obstruction can clinically mimic cholangiocarcinoma. Our case was also clinically considered as cholangiocarcinoma. Histological examination of the resected specimen raised suspicion of a lymphoma involving biliary tract. IHC panel applied could not confirm the diagnosis of lymphoma as markers for both B- and T-lymphoid cells, NK T-cells, and ALCL were negative. Cytokeratin for poorly differentiated carcinoma was also negative. Finally, staining for MPO was done which clinched to the diagnosis of myeloid sarcoma. An extensive IHC panel is required in these cases to reach the diagnosis. Cases presenting an obstructive jaundice on simultaneous examination of peripheral blood and bone marrow show evidence of AML.[1],[3],[4],[5],[6],[7] Rarely, they are nonleukemic.[8],[9],[10],[11] However, all these cases are followed by full blown picture of AML within few months.[12]

As our case did not show evidence of AML in peripheral blood and marrow at the time of diagnosis and appeared asymptomatic after surgery, patient was reluctant for further treatment. She developed full blown picture of AML within 2 months; however, her general condition did not allow any chemotherapy treatment. Our case illustrates that myeloid sarcoma is to be aggressively treated as AML even in the absence of peripheral blood and bone marrow findings. Nonleukemic patients of granulocytic sarcoma initially administered chemotherapy show longer disease free intervals and overall survival.[9],[11]

Presence of jaundice, hepatic dysfunction may hamper the treatment by impairing tolerance to chemotherapeutic drugs. Chemotherapy along with endoscopic nasobiliary drainage has been considered as treatment modality in these cases. Lee et al.[1] reported a case where induction chemotherapy initially increased jaundice, which resolved after simultaneous nasobiliary drainage and remission was achieved by giving full doses of cytarabine and idarubicin. However, Rajesh et al.[13] reported a case where nasobiliary drainage failed to relieve jaundice and Goor et al.[5] reported resolution of presumptive biliary leukemic infiltrate with chemotherapy in their case without drainage procedure.

Acute leukemia presenting as obstructive jaundice has been reported in pediatric cases as well. Rajeshwari et al.[10] reported two cases, one at the age of 1 year and another of 10 months. Both had aggressive disease and succumbed to illness in short period. They also cited a case reported by Lang et al. of 4-year-old male child with mass at pancreatic head-associated biliary obstruction where remission could be achieved by chemotherapy followed by bone marrow transplantation.

 Conclusion



To conclude, patients of myeloid sarcoma presenting as obstructive jaundice in the absence of evidence of disease in peripheral blood and bone marrow are to be treated aggressively like AML, as these patients are likely to have rapid fatal course in absence of timely management. The exact diagnosis on histopathological examination needs careful evaluation of morphological features and optimal immunohistochemistry panel.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgements

Authors acknowledge secretarial help provided by Ms. Noora Baweja.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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