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Granulocytic sarcoma, also known as myeloid sarcoma, is an extra-medullary tumoral aggregation of immature granulocytic cells¹. Although rare, it is a known presentation of acute leukemia, especially in association with acute myeloid leukemia and less commonly associated with other myeloproliferative disorders and myelodysplastic disorders¹. It may be detected simultaneously with leukemia, during the evolution of the disease, and sometimes at relapse after bone marrow transplantation². Rarely, it is detected before clinical signs of leukemia appear.  

It can involve any body site; however, soft tissues, lymph nodes, and gastrointestinal tract are among the commonly affected sites1. Rarely central nervous system and genitourinary system may be involved. Clinical features are diverse and depend on the size and site of the tumor. Based on the diversity of location and clinical presentation, the diagnosis of this tumor becomes tricky. However, the association with acute leukemia is an important clue that should clinch the diagnosis. 

Here we report a case of pancreatic mass in a patient presenting as cholestatic jaundice, which was eventually diagnosed as granulocytic sarcoma. Subsequent evaluation of the patient showed myeloid blasts in peripheral blood cells and on immunophenotyping  a diagnosis of Acute Myeloid Leukemia with monocytic differentiation (M5) was made.

A 45 year-old male presented to the Gastroenterology outpatient department with a history of vague pain abdomen and cholestatic jaundice for two months associated with significant anorexia and weight loss of 5 kg over two months. Past history was significant for left-sided pleural effusion one and a half years back for which he received anti-tubercular treatment. Physical examination showed stable vitals. Pallor and icterus were present, along with significant bilateral cervical, axillary, and inguinal lymphadenopathy. On abdominal examination, there was cystic firm palpable gall bladder and mild splenomegaly.

Laboratory evaluation showed hemoglobin of 4.7 gm/dL, markedly raised leukocytes 200×1000/cumm, and platelet count 1.08 lakhs/ml. Peripheral blood film showed the presence of 76% blast cells. Liver function tests revealed features of cholestasis with total bilirubin 6.2 mg/dl predominantly conjugated bilirubin, elevated alkaline phosphatase (ALP) 1805 U/l (<97 U/l), aspartate aminotransferase (AST) 150 IU/l, alanine aminotransferase (ALT) 210 IU, lactate dehydrogenase (LDH) 490 U/L. Amylase and lipase levels were within normal limits. CA 19.9 was within normal limits (8.25 IU/L). Ultrasonography revealed dilated CBD of 12 mm, with biliary radicles and mass lesion in the head of pancreas. The patient underwent a CT scan, which showed soft tissue mass arising from the pancreatic head of size 5×4.7 cm with resultant compression of the intra-pancreatic common bile duct, multiple enlarged portal and peri-pancreatic nodes largest measuring 10 mm, and bilateral IHBR dilation with patent ductal confluence.

Endoscopic- Ultrasound (EUS) revealed an ill-defined hypo-echoic mass lesion of size 5×4.5 cm in relation to the head and uncinate process of pancreas compressing the CBD (Figure 1). EUS guided FNA smears showed sheets of blasts surrounding normal pancreatic acini (Figure 2).  

Immunophenotyping of the peripheral blood blasts showed 76% blasts positive for CD13, CD33, CD117, CD14, and HLA DR. Hence, a diagnosis of Acute myeloid leukemia with monocytic differentiation was made (Figure 3). The patient underwent biliary stenting and started on induction therapy with cytarabine and daunorubicine.

Granulocytic Sarcoma is also called chloroma, which refers to the green color picked up by the neoplasm cells due to the presence of myeloperoxidase enzyme². Granulocytic sarcoma generally is diagnosed simultaneously or can occur occasionally following the onset of Acute Myeloid Leukemia (AML) in 3-10% of patients³. Rarely, granulocytic sarcoma is the first manifestation of AML. Our case is an exceptional one in this regard as the presenting symptoms of the patients were due to the presence of pancreatic mass, later diagnosed to be a granulocytic sarcoma, and subsequently, AML was diagnosed. Pancreatic granulocytic sarcoma cases have been rarely reported in the literature with only 10 cases, to our knowledge^4,5. Granulocytic sarcoma can occur in patients of all ages with slight male dominance (male: female – 1.2: 1). The presence of granulocytic sarcoma in patients with AML worsens the overall prognosis, and only 24% of patients were reported to survive for two years after the initial diagnosis with overall median survival of 7 to 20 months³. These observations emphasize the fact that clinicians should consider the diagnosis of chloroma when a soft tissue mass is identified in a patient suspected to have leukemia. However, in the case reported herein, as granulocytic sarcoma was the first manifestation of AML, diagnosis may be challenging.

Young age and normal CA 19.9 levels of our patient may have been a clinical indication that emphasizes the value of cytological examination to rule out the other differential diagnosis in this patient, especially pancreatic adenocarcinoma. A diagnosis of granulocytic sarcoma is challenging especially if not considered clinically and requires an expert pathologist. Histology reveals markedly increased blast cells, promyelocytes, and myelocytes.

To conclude, it is important to consider granulocytic sarcoma when confronted with a pancreatic mass in association with splenomegaly, generalized lymphadenopathy, or cytopenias. Although rare, recognizing this diagnosis may assist the pathologist in making the correct diagnosis on the biopsy specimen, and facilitate appropriate treatment and prognostication.