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

IF 0.8 Q3 MEDICINE, GENERAL & INTERNAL Case Reports in Medicine Pub Date : 2023-12-26 DOI:10.1155/2023/8274226
V. Marbun, Indah Jamtani, E. Krisnuhoni, S. S. Panigoro
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Abstract

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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罕见的双原发性恶性肿瘤:模仿腮腺肌上皮癌转移灶的胰腺胃肠道间质瘤
胰腺胃肠道间质瘤(PGISTs)异常罕见,占胃肠道外间质瘤(EGISTs)的<5%,占胰腺恶性肿瘤的<1%。我们报告了一例独特的并发双原发恶性肿瘤病例,患者是一名 46 岁女性,曾患复发性腮腺肌上皮癌,通过手术切除和辅助治疗得到控制。她出现腹部肿块增大,最初提示为胰腺转移。免疫组化分析显示,腮腺和胰腺组织中的平滑肌肌动蛋白(SMA)和 CD34 均呈阳性染色。重要的是,CD117的表达仅限于胰腺组织,这证实了PGIST而非转移的诊断。随后,患者接受了保脾远端胰腺切除术,并立即接受了伊马替尼治疗。该病例强调了具有独特组织病理学特征和器官受累的罕见原发性恶性肿瘤并存的可能性。当遇到远处器官新发病灶时,外科医生必须考虑转移的可能性,以指导治疗决策。早期诊断和适当干预至关重要,尤其是在 PGIST 的情况下,因为它并不常见且临床复杂。
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来源期刊
Case Reports in Medicine
Case Reports in Medicine MEDICINE, GENERAL & INTERNAL-
CiteScore
1.70
自引率
0.00%
发文量
53
审稿时长
13 weeks
期刊最新文献
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