Brain Tumor Res Treat.  2022 Jul;10(3):200-205 . 10.14791/btrt.2022.0019.

Cerebral Metastases in Appendiceal Cancer: Comprehensive Review and Report of Rare Medullary Carcinoma Histology

• Mackel C
• Rosenberg H
• Varma H
• Vega R
• Stippler M

• KMID: 2532245
• DOI: http://doi.org/10.14791/btrt.2022.0019

Abstract

Appendiceal cancer is an extremely rare malignancy, and its metastatic spread to the brain is even more unusual. We describe a 47-year-old female who presented with a rare cerebral appendiceal carcinoma metastasis, a case that is further remarkable for representing the first histologic diagnosis of primary medullary carcinoma in the appendix. Based on a comprehensive review of the English literature using PubMed, Embase, and Google Scholar, only six other cases of cerebral appendiceal metastases have been described.

Keyword

Appendiceal cancer; Brain tumor; Medullary carcinoma; Metastasis

Figure

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  Fig. 1 Images of appendiceal carcinoma with cerebral metastasis. A: Axial T1-gadolinum enhanced MRI of the brain demonstrating a heterogeneously enhancing right frontal mass with extensive vasogenic edema prior to resection. B: Appendiceal mass identified on colonoscopy with stigmata of recent bleeding.

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  Fig. 2 Photomicrographs of the right frontal mass showing metastatic poorly differentiated carcinoma. A: Low power (H&E, ×4) shows a poorly differentiated neoplasm with prominent sheet-like growth and necrosis (bottom). B: Higher power (H&E, ×20) highlights poorly differentiated tumor cells with prominent nucleoli and mitotic activity. C: CDX-2 is positive in tumor cells, suggesting gastrointestinal origin. Scale bars = 200 µm (A), 50 µm (B), 100 µm (C).

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  Fig. 3 Photomicrographs of the appendix tumor biopsy, poor differentiated carcinoma, favoring medullary carcinoma. A: High power (H&E, ×20) highlights a poorly differentiated tumor with sheet-like growth, prominent nucleoli, and mitotic activity, similar to the brain lesion. B: Immunohistochemistry for mismatch repair protein MLH-1 shows loss in tumor cells with retention in adjacent non-neoplastic cells. C: Immunohistochemistry for mismatch repair protein PMS-2 shows loss in tumor cells with retention in adjacent non-neoplastic cells. Scale bars = 50 µm (A), 100 µm (B and C).

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  Fig. 4 Flow chart of search results for cerebral appendiceal metastases performed May 2022 using the databases of PubMed, Embase, and Google Scholar.

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