Pigmented perivascular epithelioid cell tumor of the liver: Report of a rare case with brief review of literature

Patra, Susama; Vij, Mukul; Venugopal, K.; Kancherla, Ravindranath; Rela, Mohamed

doi:10.4103/0973-1482.113401

Cited by 21 publications

(12 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…[4,27] PEComas are characterized by their perivascular location, and the cells are with radial arrangement around the vascular lumen. Typically, cells around the vessels are epithelioid and spindle-shaped, resembling smooth muscles cell, and have abundant clear to eosinophilic granular cytoplasm.…”

Section: Discussionmentioning

confidence: 99%

Hepatic perivascular epithelioid cell tumor

et al. 2016

View full text Add to dashboard Cite

Rational:Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers. PEComas are found in a variety locations in the body, but up to now only approximately 30 cases about hepatic perivascular epithelioid cell tumor are reported in English language worldwide.Patient concerns:A 32-year-old woman was admitted in our hospital with intermittent right upper quadrant pain for 1 month and recent (1 day) progressive deterioration.Diagnoses:Based on the results of the laboratory examinations and the findings of the computed tomography, the diagnosis of hepatic hamartoma or the hepatocecullar carcinoma with hemorrhage was made.Interventions:The patient underwent a segmentectomy of the liver, and the finally diagnosis of hepatic PEComa was made with immunohistochemical confirmation with HMB-45 and SMA.Outcomes:There is no clinical or radiographic evidence of recurrence 9 months after surgery.Lessons:This kind of tumor is extremely rare and the natural history of PEComa is uncertain, as the treatment protocol for hepatic PEComa has not reached a consensus. But the main treatment of the disease may be surgical resection. Only after long term follow-up can we know whether the tumor is benign or malignant. It appears that longer clinical follow-up is necessary in all patients with hepatic PEComas.

show abstract

Section: Discussionmentioning

confidence: 99%

Hepatic perivascular epithelioid cell tumor

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Melanotic PEComas have been described at extracutaneous sites. 32,33 Most PEComas are cytologically bland. However, some display large tumor size, high-grade atypia ( Figure 6, B), dense cellularity, infiltrative growth, necrosis, significant mitotic activity (.1 per 50 high-power fields), or angiolymphatic invasion; cases with these concerning features warrant classification as PEComa of uncertain malignant potential if one feature is present, or malignant PEComa if multiple features are present.…”

Section: Cutaneous Pecomamentioning

confidence: 99%

Clear Cell Melanoma: A Cutaneous Clear Cell Malignancy

Pletneva

Andea

Palanisamy

et al. 2014

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

Clear cell melanoma is a rare clear cell malignancy. Accurate diagnosis of clear cell melanoma requires integration of immunohistochemical and morphologic findings, with molecular studies to rule out clear cell sarcoma. The differential diagnosis includes melanoma, carcinoma, perivascular epithelioid cell tumor, and epidermotropic clear cell sarcoma. We use a case of a lesion on the helix of an 86-year-old man as an example. Histologic examination revealed an ulcerated clear cell malignant tumor. Tumor cell cytoplasm contained periodic acid-Schiff-positive, diastase-sensitive glycogen. Tumor cells showed positive labeling for S100, HMB-45, and Melan-A, and negative labeling for cytokeratins, p63, and smooth muscle actin. Molecular studies demonstrated BRAF V600E mutation, copy gains at the 6p25 (RREB1) and 11q13 (CCND1) loci, and absence of EWSR1-ATF1 fusion. These findings supported a diagnosis of clear cell melanoma. The rare pure clear cell morphology occurs due to accumulation of intracytoplasmic glycogen. We review the differential diagnosis of clear cell melanoma and describe the utility of immunohistochemical and molecular studies in confirming this diagnosis.

show abstract

“…However, pigmented PEComas were reported in organs other than the skin and subcutaneous tissue. They are exceedingly rare neoplasms and have been reported in kidney, [30][31][32] pelvis, 33 liver, 34 and orbit. 35 Here, we describe the first case of cutaneous PEComa with focal melanin pigmentation histologically and discuss differential diagnoses of other entities, such as melanoma, renal clear cell carcinoma, clear hidradenoma, and paraganglioma.…”

Section: Introductionmentioning

confidence: 99%

Pigmented Perivascular Epithelioid Cell Tumor of the Skin

Navale

Asgari

Chen

2015

The American Journal of Dermatopathology

View full text Add to dashboard Cite

The authors present a first case of pigmented perivascular epithelioid cell tumor (PEComa) of the skin, which was misdiagnosed histopathologically as metastatic melanoma. PEComa are exceedingly rare neoplasms, and only a few cases have been reported in kidney, liver, and pelvis. Herein, we describe this very rare entity in the subcutaneous tissue of the flank with very similar if not identical morphology of renal clear cell carcinoma. Because of the presence of focal melanin pigmentation and strong positivity for HMB-45, the lesion was mistaken for metastatic melanoma. To the best of authors' knowledge, this is the first case of cutaneous PEComa with melanin pigmentation. Awareness about occurrence of the lesion in the subcutaneous tissue and its unique immunoprofile would be helpful to reach correct diagnosis and to distinguish this lesion from melanoma or metastatic renal clear cell carcinoma.

show abstract

Pigmented perivascular epithelioid cell tumor of the liver: Report of a rare case with brief review of literature

Cited by 21 publications

References 4 publications

Hepatic perivascular epithelioid cell tumor

Hepatic perivascular epithelioid cell tumor

Clear Cell Melanoma: A Cutaneous Clear Cell Malignancy

Pigmented Perivascular Epithelioid Cell Tumor of the Skin

Contact Info

Product

Resources

About