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Case Reports
. 2024 Jun 21:14:e2024503.
doi: 10.4322/acr.2024.503. eCollection 2024.

Clear cell myomelanocytic tumor of ligamentum teres

Ariba Zaidi  1 , Debajyoti Chatterjee  2 , Venu Bhargav  3 , Vikas Gupta  3 , Ashim Das  2
Affiliations
Case Reports

Clear cell myomelanocytic tumor of ligamentum teres

Ariba Zaidi et al. Autops Case Rep. .

Abstract

Clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ligamentum teres is a rare hepatic tumor, a variant of the perivascular epithelioid cell tumor (PEComa) family. CCMMT is the rarest variant of hepatic PEComas. Only a few cases of CCMMT have been reported in the English literature. Because of its rarity, less is known about its biological behavior. We present a case of a 31-year-old female who complained of abdominal pain, bilious vomiting, and abdominal fullness over two months. The radiological impression was of focal nodular hyperplasia. The histological examination of the resection specimen revealed a well-circumscribed tumor arranged in fascicles, sheets, and a whorling pattern. The tumor cells were spindle to epithelioid shaped with abundant clear to pale eosinophilic cytoplasm. The tumor cells expressed both myoid (smooth muscle actin) and melanocytic (MelanA and HMB45) markers, while they were negative for hepatocytic and vascular markers. Thus, based on histology and immunohistochemistry, a diagnosis of CCMMT was made. This case presents the diagnostic challenges of CCMMT and discusses the differential diagnosis with a literature review.

Keywords: Immunohistochemistry; Liver Neoplasms; Perivascular Epithelioid Cell Neoplasms.

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Conflict of interest statement

Conflict of interest: None

Figures

Figure 1
Figure 1. Abdominal computed tomography scan showing a large, lobulated tumor involving the left lobe and part of the right lobe of the liver.
Figure 2
Figure 2. Gross image of the slices of the liver showing a large mass in the right lobe reaching up to the liver capsule with central hemorrhagic areas. Scale bar= 10cm.
Figure 3
Figure 3. A – A well-circumscribed tumor is seen, sharply demarcated from the normal liver (lower left part) (H&E, x40); B – The tumor cells are arranged in fascicles (H&E, x100); C – Tumor cells are epithelioid in shape and have clear to pale eosinophilic cytoplasm (H&E, x200); D – At places, the tumor cells are arranged in a whorling pattern around blood vessels (H&E, x200).
Figure 4
Figure 4. The tumor cells show strong expression of smooth muscle antigen (A – immunohistochemistry, x200), HMB-45 (B – immunohistochemistry, x400), and MelanA (C – immunohistochemistry, x400). (D) CD34 stain highlights the capillaries, while the tumor cells are negative (immunohistochemistry, x200).
See this image and copyright information in PMC

References

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