• 13 year, Female
  • Presented with severe pain in the abdomen for 20 days with vomiting and generalized itching without any jaundice
  • CT scan and USG abdomen showed a pancreatic head mass measuring 4.6 cm X 4.4 cm X 4.9 cm with Dilatation of Proximal CBD  with IHBRD and few sub centimeter sized mesenteric and paraaortic lymphnode
  • EUS revealed a large hypoechoic mass lesion measuring (4 cm X 3.5 cm) in the head of pancreas with a peripheral cystic component and central necrosis
  • EUS-FNA was performed with a 22 G needle under colour Doppler control (two pass) from the suspicious lesion in the pancreatic head mass and the peripheral cystic component and aspirated material was sent for cytological examination and immunohistochemistry study
  • Cytology report was suggestive of Small round cell tumor of pancreas

Expert Comments:

  • Small round cell tumor of the pancreas is a rare and highly aggressive neoplasm which is an often misdiagnosed neoplasm in children and adolescents.
  • The aggressiveness of the disease and propensity for serosal spread necessitates the accurate preoperative diagnosis for curative resection and determining the prognosis
  • Endoscopic ultrasound with guided biopsy of the tumor provides adequate samples for the cytology and immunohistochemistry of the tumor
  • Small round cell tumors express desmin that help to confirm the diagnosis on immunohistochemistry
  • Multidisciplinary approach involving  chemotherapy, extensive debunking surgery and abdominopelvic radiation have been reported to improve the survival of the patients

Take Home Message

  • Young adolescent patients presenting with pancreatic mass should be suspected of having small round cell tumor
  • There have been reported cases of failure to diagnose the small round cell tumor of pancreas preoperatively leading to suboptimal resection of the disease affecting the prognosis of the patients
  • However with endoscopic ultrasound guided biopsy of these tumors provides accurate diagnosis  preoperatively as well as assessment of locoregional nodal spread

References:

  1. Gerald WL, Rosai J. Case 2: Desmoplastic small round cell tumor with divergent differentiation. Pediatr Pathol.1989;9:177–83
  2. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP. Results of multimodal treatment for desmoplastic round cell tumors. J Pediatr Surg. 2005; 40:251–5.
Pancreatic head mass

Pancreatic head mass

Dilatation of Proximal CBD  with IHBRD

Dilatation of Proximal CBD with IHBRD

 

 

 

 

 

 

 

 

Hypoechoic mass lesion in the head of pancreas

Hypoechoic mass lesion in the head of pancreas

Color doppler

Color doppler

 

 

 

 

 

 

 

 

 

EUS guided FNA

EUS guided FNA

Small round cell tumor of pancreas

Small round cell tumor of pancreas

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