Dataset Information

Synchronous Gastrointestinal Stromal Tumor (GIST) with pancreatic adenocarcinoma: A case report.

ABSTRACT:

Introduction

Gastrointestinal Stromal Tumors (GIST) are rare mesenchymal neoplasm of gastrointestinal tract. Stomach is the most common site affected by GIST compared to other places in gastrointestinal track. The coexistence of GIST with another malignancy represents a rare phenomenon with few literature reported.

Case presentation

We present here 65 years old patient with stomach GIST and synchronous pancreatic adenocarcionoma discovered during surgery for suspected pancreatic mucinious cystadenoma. Distal pancreaticosplenectomy with excision of GIST Tumor & wedge resection of stomach was done. Histopathological examination of resected specimens reported the margins are clear.

Discussion

In this article we discuss on the option of systemic therapy versus upfront surgery and their outcome benefit based on literature review.

Conclusion

The coexistence of GIST with pancreatic adenocarcinoma is a rare condition. High clinical analysis needed during laparotomy for GIST to detect a synchronous tumor. In a case of GIST the surgeon should recognize the possibility of another tumor with different histological origin. Surgical excision is the mainstay of therapy and it has proven to be curative for our patient. . Due to its rare occurrence and limited literature further studies has to be done on GIST with other synchronous tumor to help the surgeon to manage the patient optimally.

SUBMITTER: A/L Chandrasekaran T

PROVIDER: S-EPMC9142392 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

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Publications

Synchronous Gastrointestinal Stromal Tumor (GIST) with pancreatic adenocarcinoma: A case report.

A/L Chandrasekaran Terukumar T Sahid Nik Amin NA Maiyauen Thanesh Kumar TK

Annals of medicine and surgery (2012) 20220411

<h4>Introduction</h4>Gastrointestinal Stromal Tumors (GIST) are rare mesenchymal neoplasm of gastrointestinal tract. Stomach is the most common site affected by GIST compared to other places in gastrointestinal track. The coexistence of GIST with another malignancy represents a rare phenomenon with few literature reported.<h4>Case presentation</h4>We present here 65 years old patient with stomach GIST and synchronous pancreatic adenocarcionoma discovered during surgery for suspected pancreatic m ...[more]

PMID: 35638052

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Project description:BackgroundGastrointestinal stromal tumor (GIST) is the most common primary mesenchymal neoplasm of the gastrointestinal tract. Mutations of KIT and platelet-derived growth factor receptor alpha have been well characterized in GISTs. Patients with KIT mutations are generally sensitive to treatment with tyrosine kinase inhibitors. However, some patients with GIST, while initially sensitive to TKIs, gain resistance in later stages of treatment. Heterologous rhabdomyomsarcomatous dedifferentiation of advanced GISTs after long-term imatinib mesylate (IM) therapy has been reported. In these cases, the underlying molecular mechanism of tumor progression and transformation is unclear.Case presentationWe report one such patient with rhabdomyosarcomatous dedifferentiation of a GIST without metastatic disease after brief 3-month therapy with IM. The tumor was composed of two distinct phenotypes, a CD117 negative region with rhabdomyosarcomatous differentiation directly adjacent to a CD117 positive classic GIST region. Molecular analysis identified the activating KIT exon 11 mutation in both regions, indicating a common origin for both phenotypes. Additionally, the dedifferentiated component contained two synonymous variants in platelet-derived growth factor receptor alpha and KIT. The increased number of synonymous variants in the rhabdomyosarcomatous region may reflect increased genetic instability of this tumor that may have resulted in the loss of CD117 expression in the dedifferentiated component.ConclusionThis study adds to the growing consensus that rhabdomyosarcomatous GIST progresses from a common GIST primary tumor. The role of IM in this progression is uncertain; however short duration of IM treatment in this study supports the hypothesis that rhabdomyosarcomatous GIST progression is not a consequence of IM therapy. Furthermore, we provide additional information supporting the observation that CD117 negative rhabdomyosarcomatous transformation maintains the activating KIT variant without KIT expression.

Dataset Information

Synchronous Gastrointestinal Stromal Tumor (GIST) with pancreatic adenocarcinoma: A case report.

Introduction

Case presentation

Discussion

Conclusion

Publications

Synchronous Gastrointestinal Stromal Tumor (GIST) with pancreatic adenocarcinoma: A case report.

Similar Datasets

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