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CASE REPORT Table of Contents  
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Obstructive Jaundice with skin involvement - an unusual presentation of Myeloid Sarcoma

1 Department of Medical Gastroenterology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India
2 Department of Hepatology, SCB Medical College and Hospital, Cuttack, Odisha, India

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Date of Submission15-Nov-2021
Date of Decision26-Apr-2022
Date of Acceptance28-Apr-2022
Date of Web Publication18-Oct-2022


Biliary obstruction secondary to malignancy is a common clinical problem. Rarely, biliary obstruction is due to leukemia, and obstructive jaundice in these patients usually presents late in the course of the disease. We present a rare case of a patient who presented with fever, jaundice, and pruritus with multiple nodular swellings in the left shoulder, left thigh, and lower back. Magnetic resonance cholangiopancreatography (MRCP) revealed periampullary mass lesion causing dilated common bile duct (CBD) and intrahepatic bile ducts; hence, endoscopic retrograde cholangiography with plastic stenting was done. Biopsy from the shoulder lesion revealed a mesenchymal tumor, and immunohistochemistry (IHC) confirmed the lesion as myeloid sarcoma. Myeloid sarcoma is an extramedullary tumor, a subtype of acute myeloid leukemia, and presentation as biliary lesions with multiple anatomical sites is very rare. The patient was started on chemotherapy after the normalization of bilirubin. The patient showed improvement of skin lesions and normalization of liver function test (LFT) after 3 weeks of chemotherapy.

Keywords: Acute myeloid leukemia (AML), myeloid sarcoma, obstructive jaundice

How to cite this URL:
Jeji PS, Patnaik SK, Behera MK, Narayan J, Sahu MK, Mishra D, Singh A, Pati GK. Obstructive Jaundice with skin involvement - an unusual presentation of Myeloid Sarcoma. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Sep 24]. Available from:

   Introduction Top

Malignant biliary obstruction is a common clinical entity encountered in clinical practice. Myeloid sarcoma of the bile ducts with obstructive jaundice is a very rare initial presentation.[1],[2] Myeloid sarcoma is a tumor mass of immature granulocytic cells occurring at an extramedullary site. It usually occurs during the course of acute myeloid leukemia (AML), though it also occurs in chronic myeloid leukemia (CML).[2]

Extramedullary manifestations of acute myeloid leukemia include leukemia cutis, organomegaly, meningeal leukemia, and tumor masses at any site. Among them, myeloid sarcoma is a rare extramedullary tumor composed of immature cells of the myeloid progenitor series, found in patients with myelodysplastic syndrome, CML, and AML and usually involves bone, periosteum, soft tissue, lymph nodes, and skin. Owing to its rarity as a cause of biliary obstruction, AML is seldom considered in the differential diagnosis of obstructive jaundice and is often misdiagnosed initially as cholangiocarcinoma.[3]

   Case Report Top

A 42-year-old male presented with jaundice for 15 days, associated with itching all over the body. No history of fever, pain abdomen, vomiting, diarrhea, or weight loss was present. On examination, there were multiple swellings present over the left shoulder, left thigh, and lower back. No localized tenderness or raised temperature was noted.

The complete blood count (CBC) report had all parameters within the normal range. A liver function test (LFT) showed total bilirubin of 18 mg/dL with predominant conjugated bilirubinemia and alkaline phosphatase (ALP) around 200 IU/L. A repeat LFT after 72 h showed a further increase in total bilirubin to 23 mg/dL and ALP rising to 243 IU/L. Serum LDH was also slightly elevated at 240 U/L. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and CA 19-9 were all within the normal range. Ultrasound abdomen was done, which showed dilated common bile duct (CBD), although the lumen was clear. Hence, magnetic resonance cholangiopancreatography (MRCP) was done which suggested a periampullary mass lesion with a double duct sign. Hence, contrast-enhanced computed tomography (CECT) was done, which showed periampullary lesion dilatation of CBD and intrahepatic biliary radicles (IHBR) with few hypo enhancing lesions in the renal cortex [Figure 1]. Endoscopy was done showing significant bulging of the ampulla. In view of the obstructive pattern of jaundice and CECT findings, endoscopic retrograde cholangiography (ERCP) was done showing an impression of ampullary mass with distal CBD stricture, and plastic stenting was done for the same [Figure 2]. Biopsy from the thigh region revealed sheets of large cells with round, oval to cleaved nuclei with variable prominent nucleoli and scant cytoplasm, which was suggestive of the possibility of either non-Hodgkin's lymphoma or malignant round cell tumor. However, immunohistochemistry (IHC) markers were positive for CD 45, CD 68, CD 99, CD 117, and myeloperoxidase (MPO) suggestive of myeloid sarcoma [Figure 3].
Figure 1: (a) CECT abdomen showing well-defined oval-shaped enhancing mass in periampullary region with dilated CBD. Multiple hypoenhancing lesions involving the cortex of the right kidney; (b) MRCP with MRI abdomen showing periampullary mass; (c) MRCP showing dilated CBD with IHBR; (d) MRCP coronal section showing dilated CBD with IHBR dilatation and periampullary mass

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Figure 2: (a and b) ERCP images showing ampullary mass with distal CBD stricture with posphincterotomy and stent placement; (c and d) Cholangiogram images showing dilated CBD due to stricture

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Figure 3: (a) Spindle shaped tumor cells taking up the stain for CD 117 marker in IHC; (b) MPO positive stain in tumor cells as seen with a blue nucleus and brown cytoplasm

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Hematology consultation was taken in view of the above findings, and a bone marrow biopsy was done. Bone marrow aspiration showed 10% blasts with MPO positivity, and morphologically myelodysplasia-related changes were not evident in other lineages. The flow cytometry analysis was positive for cytoplasmic MPO, CD117, HLA-DR, CD 13, and CD 33 with dim positivity for CD45, CD34, CD 64, CD 11c, CD 14, CD 4, and CD 2, suggestive of blasts of myeloid lineage. A fluorescent in situ hybridization (FISH) study from bone marrow does not reveal any recurrent cytogenetic abnormalities like t(8;21)(q22;q22.1);RUNX1-RUNX1T1, inv (16)(p13.1q22) or t(16;16)(p13.1;q22);CBFB-MYH11, t(9;22)(q34.1;q11.2);BCR-ABL1, t(15;17)(q22;q11-12);PML-RARA, t(9;11)(p21.3;q23.3);KMT2A-MLLT3. Positron emission tomography-computed tomography (PET-CT) was done showing hypermetabolic cervical and axillary nodes. Hypermetabolic heterogeneously enhancing soft-tissue masses were noted in multiple areas all showing evidence of nodal and extranodal soft tissue metastatic disease.

The patient was started on chemotherapy by the hematology department. Dexamethasone was given for 10 days along with cytarabine, and the patient was discharged. After the second cycle of chemotherapy with dexamethasone and cytarabine, LFT showed significant improvement after 3 weeks with total bilirubin reduced to around 2 mg/dL, normal aspartate transaminase (AST) and alanine transaminase (ALT) values and alkaline phosphatase (ALP) also reducing to 140 IU/L.

   Discussion Top

Obstructive jaundice is a common problem occurring from an obstruction to the passage of conjugated bilirubin from the liver to the intestine, and malignancy accounts for 64% of cases of obstructive jaundice. Although malignant causes of obstructive jaundice are much more common than benign causes, the single most common cause has been found to be choledocholithiasis in a recent study.[4],[5] Secondaries in the liver have an association with obstructive jaundice in 5%–7% of cases.[5] Several case reports of cholestasis and acute liver failure due to hematological malignancies have been described, secondary to lymphomas, lymphoid leukemias, or acute myeloid leukemia.[6]

A few studies in children too showed obstructive jaundice as the presenting feature of acute myeloid leukemia. It may be due to a stricture of the biliary tree or a granulocytic sarcoma compressing the biliary tree.[6] Myeloid sarcoma also known as granulocytic sarcoma or chloroma is a tumor mass consisting of myeloid blasts occurring at extramedullary sites and is an extramedullary manifestation of AML. Primary involvement of the biliary tract with myeloid sarcoma is very rare and can be clinically misdiagnosed as cholangitis or cholangiocarcinoma. Obstructive jaundice may be due to granulocytic sarcomas compressing the biliary tree or stricture of the biliary tree.[7],[8]

Goor et al.[9] reported a case of a 36-year-old man presenting with abdominal pain and jaundice, imaging showed dilatation of the intrahepatic bile ducts and a normal common bile duct with no calculi. A peripheral blood smear was diagnostic of AML. After remission-inducing chemotherapy, there was a complete regression of jaundice, which had not recurred at the 12-month follow-up. Another case report showed a 72-year-old lady presenting with features of biliary obstruction and was clinically considered as cholangiocarcinoma. She was diagnosed with myeloid sarcoma involving the right and left hepatic duct and common bile duct on histopathological and IHC examination after surgical resection. As 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 2 months and died.[7] A recent case report from Canada revealed painful obstructive jaundice in a 36-year-old male who was found to have myeloid sarcoma after detailed investigations.[10] As was the case in our patient and a few others, CBC was normal, and the diagnosis was established on biopsy and IHC evaluation. Hence, hematological malignancy as an etiology of obstructive jaundice always should be kept, even with normal blood counts.

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Conflicts of interest

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   References Top

Lokich JJ, Kane RA, Harrison DA, McDermott WV. Biliary tract obstruction secondary to cancer: Management guidelines and selected literature review. J Clin Oncol 1987;5:969-81.  Back to cited text no. 1
Brunning RD, McKenna RW. Tumors of bone marrow, third series, fascicle 9. Washington, DC: Armed Forces Institute of Pathology; 1994. Atlas of tumor pathology; p. 19–37.  Back to cited text no. 2
Gupta AK, Singh A, Goel S, Tank R. Profile and pattern of obstructive jaundice cases from a tertiary care teaching hospital of Uttar Pradesh. Int Surg J 2017;4:743-6.  Back to cited text no. 3
Sharma MP, Ahuja V. Aetiological spectrum of obstructive jaundice and diagnostic ability of ultrasonography: A clinician's perspective. Trop Gastroenterol 1999;20:167-9.  Back to cited text no. 4
Khan ZA. Clinical profile of patients with obstructive jaundice: A surgeon's perspectives. Int Surg J 2019;28:1876-80.  Back to cited text no. 5
Rajeswari B, Ninan A, Prasannakumari SN, Parukuttyamma K. Acute myeloid leukemia presenting as obstructive jaundice. Indian Pediatr 2012;49:414-6.  Back to cited text no. 6
Agarwal A, Dadu T, Bhalla VP, Malhotra V. Myeloid sarcoma of bile ducts presenting as obstructive jaundice – A case report. Indian J Pathol Microbiol 2019;62:602-41.  Back to cited text no. 7
[PUBMED]  [Full text]  
Rajesh G, Sadasivan S, Hiran KR, Nandakumar R, Balakrishnan V. Acute myeloid leukemia presenting as obstructive jaundice. Indian J Gastroenterol 2006;25:93-94.  Back to cited text no. 8
[PUBMED]  [Full text]  
Goor Y, Goor O, Michalewitcz, R, Cabili S. Acute myeloid leukemia presenting as obstructive jaundice. J Clin Gastroenterol 2002;34;485-6.  Back to cited text no. 9
Patterson M, Wu Y, Niazi M. Myeloid sarcoma presenting as obstructive jaundice. Case Rep Gastroenterol 2021:695-702.  Back to cited text no. 10

Correspondence Address:
Manas K Behera,
SCB Medical College and Hospital, Cuttack, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_1108_21


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