Cutaneous 'fibroma‐like' perivascular epithelioid cell tumor: A case report and review of literature
- Resource Type
- Authors
- Eugene Guerrero Odoño; Kong-Bing Tan; Victor Kwan Min Lee; Sok Yan Tay
- Source
- Journal of Cutaneous Pathology. 47:548-553
- Subject
- Pathology
medicine.medical_specialty
Histology
medicine.diagnostic_test
business.industry
Benign fibrous histiocytoma
Dermatology
medicine.disease
Perivascular Epithelioid Cell
Pathology and Forensic Medicine
body regions
Lesion
stomatognathic diseases
030207 dermatology & venereal diseases
03 medical and health sciences
Tuberous sclerosis
0302 clinical medicine
Stroma
030220 oncology & carcinogenesis
Biopsy
medicine
Desmin
medicine.symptom
Fibroma
business
- Language
- ISSN
- 1600-0560
0303-6987
Perivascular epithelioid cell tumors (PEComas) are a group of lesions sharing the common features of co-expression of melanocytic and myogenic markers, with focal association of the cells with vascular walls. The PEComa group exhibits a wide range of morphologies. A "fibroma-like" variant of PEComa has been recently described. The case reported herein is that of an infant with tuberous sclerosis complex (TSC) presenting with a lip mass. Excisional biopsy showed a moderately cellular tumor composed of spindled to stellate cells embedded within a collagenized stroma. The cells showed focal perivascular accumulation and positivity for both melanocytic (HMB-45) and myogenic (desmin) markers. This is the fifth reported case of "fibroma-like" PEComa in literature and the youngest patient to date. All of the "fibroma-like" PEComas were found in patients with tuberous sclerosis-hence, the diagnosis of this entity should prompt a workup for TSC; conversely, a fibroma-like lesion in a patient with TSC or with TSC-related conditions should be evaluated using melanocytic and myogenic markers. Melanocytic and myogenic markers are also useful in differentiating "fibroma-like" PEComa from other differential diagnoses such as fibroma and benign fibrous histiocytoma.