Extraosseus Ewing's Sarcoma in Pancreas: A Review.

Dharti Patel,Aravind Reddy,Nitish Singh Nandu

doi:10.7759/cureus.7505

Dharti Patel, Aravind Reddy + Show 1 more

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https://doi.org/10.7759/cureus.7505

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Abstract

Primitive neuroectodermal tumors (PNET, previously referred to as peripheral neuroepithelioma) are rare malignant tumors with various degrees of differentiation belonging to the Ewing’s family of sarcomas. They are classified as round cell tumors arising from soft tissues. In rare instances, PNETs may arise from solid organs containing neuroendocrine cells of kidney, bladder, heart, lungs, parotid glands and pancreas. Most cases occur in the second decade of life with a slight preponderance in males. PNET of the pancreas is an aggressive tumor with multiple recurrences and a relatively poor prognosis. These tumors should be considered in the differential diagnosis, especially in a diagnosed pancreatic tumor in individuals less than 35 years of age. Due to the nature of the tumor, surgery with subsequent chemoradiation are widely accepted modalities despite the poor prognosis. In this article, we review 25 cases of extraosseous Ewing’s sarcoma (ES) of the pancreas which to the best of our knowledge, enlists most cases reported in the literature thus far.

Highlights

BackgroundEwing sarcoma is a poorly differentiated, aggressive, malignant, round cell tumor without cellular or structural differentiation more commonly diagnosed in children younger than ten
Peripheral primitive neuroectodermal tumors rarely arise in organs and it is extremely uncommon for PNETs to originate in the pancreas
Batsakis et al divided the PNET family of tumors into the following three groups, based on the origin of the tissue- central nervous system tumors including primitive neuroectodermal tumors in the brain and spinal cord, tumors derived from the autonomic nervous system called neuroblastomas, and tumors originating outside the central and autonomic nervous system called Peripheral primitive neuroectodermal tumors (PPNETs) [4]

Summary

Introduction

Ewing sarcoma is a poorly differentiated, aggressive, malignant, round cell tumor without cellular or structural differentiation more commonly diagnosed in children younger than ten. There are no definite guidelines for the pathological criteria for the diagnosis of the peripheral PNETs. The combination of clinical signs and symptoms such as abdominal pain with an abdominal mass, jaundice, vomiting, dyspepsia, pathological characteristics like sheets of small round blue cells with hyperchromatic nuclei and scant cytoplasm; immune-histochemical features stained positive for CD99, O13, HBA71, 12E7, RFB1 and neural markers like Neuron Specific Enolase (NSE), Chromogranin A, Synaptophysin; cytogenetic analysis for MIC-2 gene and t [11;22] [q24; q12] suggest a diagnosis of PNETs. Histological features and distinction from other small round cell tumors are the principle criteria for the diagnosis of PNET. But not adjacent structures; resected lymph Distal pancreatomy,Adjuvant nodes not involved, but LVI chemotherapy with VDC present; examining alternating with cisplatin and pathologist’s ‘‘best etoposide diagnosis’’ was PNET with divergent differentiation. Abdominal pain and menstrual bleeding, breast development and pubic hair; markedly elevated estradiol levels

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Conclusions

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