Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland

Pancreatic gastrointestinal stromal tumors (PGISTs) are exceptionally rare, accounting for <5% of extra-gastrointestinal stromal tumors (EGISTs) and <1% of malignant pancreatic neoplasms. We present a unique case of concurrent double primary malignancies in a 46-year-old female with a history...

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Main Authors: Vania Myralda Giamour Marbun, Indah Jamtani, Ening Krisnuhoni, Sonar Soni Panigoro
Format: Article
Language:English
Published: Wiley 2023-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2023/8274226
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author Vania Myralda Giamour Marbun
Indah Jamtani
Ening Krisnuhoni
Sonar Soni Panigoro
author_facet Vania Myralda Giamour Marbun
Indah Jamtani
Ening Krisnuhoni
Sonar Soni Panigoro
author_sort Vania Myralda Giamour Marbun
collection DOAJ
description Pancreatic gastrointestinal stromal tumors (PGISTs) are exceptionally rare, accounting for <5% of extra-gastrointestinal stromal tumors (EGISTs) and <1% of malignant pancreatic neoplasms. We present a unique case of concurrent double primary malignancies in a 46-year-old female with a history of recurrent myoepithelial carcinoma of the parotid gland, managed through surgical resection and adjuvant therapy. She presented with an enlarging abdominal mass, initially suggestive of pancreatic metastasis. Immunohistochemical analysis revealed positive staining for smooth-muscle actin (SMA) and CD34 in both parotid and pancreatic tissues. Importantly, CD117 expression was confined to the pancreatic tissue, confirming the diagnosis of PGIST rather than metastasis. Subsequently, a splenic-sparing distal pancreatectomy was performed, followed by immediate imatinib therapy. This case underscores the potential for the coexistence of rare primary malignancies with unique histopathological characteristics and organ involvement. When encountering a newly developed lesion in a distant organ, surgeons must consider the possibility of metastasis to guide therapeutic decision-making. Early diagnosis and appropriate intervention are paramount, particularly in the case of PGIST, given its infrequent presentation and clinical complexities.
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spelling doaj-art-786b86b87dcc47b29bf9f3598554e1bb2025-02-03T11:53:20ZengWileyCase Reports in Medicine1687-96352023-01-01202310.1155/2023/8274226Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid GlandVania Myralda Giamour Marbun0Indah Jamtani1Ening Krisnuhoni2Sonar Soni Panigoro3Faculty of MedicineConsultant of Oncology Division of General Surgery Department Faculty of MedicineStaff of Pathology Anatomy DepartmentStaff of Digestive Division of General Surgery DepartmentPancreatic gastrointestinal stromal tumors (PGISTs) are exceptionally rare, accounting for <5% of extra-gastrointestinal stromal tumors (EGISTs) and <1% of malignant pancreatic neoplasms. We present a unique case of concurrent double primary malignancies in a 46-year-old female with a history of recurrent myoepithelial carcinoma of the parotid gland, managed through surgical resection and adjuvant therapy. She presented with an enlarging abdominal mass, initially suggestive of pancreatic metastasis. Immunohistochemical analysis revealed positive staining for smooth-muscle actin (SMA) and CD34 in both parotid and pancreatic tissues. Importantly, CD117 expression was confined to the pancreatic tissue, confirming the diagnosis of PGIST rather than metastasis. Subsequently, a splenic-sparing distal pancreatectomy was performed, followed by immediate imatinib therapy. This case underscores the potential for the coexistence of rare primary malignancies with unique histopathological characteristics and organ involvement. When encountering a newly developed lesion in a distant organ, surgeons must consider the possibility of metastasis to guide therapeutic decision-making. Early diagnosis and appropriate intervention are paramount, particularly in the case of PGIST, given its infrequent presentation and clinical complexities.http://dx.doi.org/10.1155/2023/8274226
spellingShingle Vania Myralda Giamour Marbun
Indah Jamtani
Ening Krisnuhoni
Sonar Soni Panigoro
Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland
Case Reports in Medicine
title Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland
title_full Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland
title_fullStr Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland
title_full_unstemmed Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland
title_short Rare Double Primary Malignancies: A Pancreatic Gastrointestinal Stromal Tumor Mimicking as a Metastatic Lesion of Myoepithelial Carcinoma of Parotid Gland
title_sort rare double primary malignancies a pancreatic gastrointestinal stromal tumor mimicking as a metastatic lesion of myoepithelial carcinoma of parotid gland
url http://dx.doi.org/10.1155/2023/8274226
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