Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

University of Michigan Health System, Ann Arbor, MI, USA.
Head and Neck Pathology 05/2011; 5(3):233-40. DOI: 10.1007/s12105-011-0268-9
Source: PubMed


PEComas are a family of neoplastic lesions that share overlapping morphology, immunohistochemistry, and ultrastructure that include angiomyolipoma, lymphangioleiomyomatosis, clear cell "sugar" tumor of the lung as well as similar tumors occurring in a variety of visceral, cutaneous and soft tissue sites throughout the body. The defining histopathological features are epithelioid cells with a perivascular distribution containing clear to pale eosinophilic granular cytoplasm and a round-to-oval centrally located nucleus with an inconspicuous nucleolus. Immunohistochemically, coexpression of melanocytic (HMB-45 and/or Melan-A) and myoid markers are characteristic. In the present study, we describe three PEComas occurring in the head and neck (nasal cavity and larynx) and discuss the behavior of these distinctive tumors and review the literature of head and neck PEComas. The importance of recognizing this entity will ensure its consideration in the differential diagnosis of tumors of the head and neck with a similar morphology. The histogenesis of PEComas still remains elusive and additional cases with a prolonged follow up remain important to accurately determine the behavior of these distinctive tumors. Complete surgical excision still remains the treatment of choice for histologically benign PEComas.

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Available from: Leon Barnes, Apr 10, 2014
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    • "The World Health Organization (WHO) defines PEComas as ''mesenchymal tumors composed of histologically, ultra structurally, and immunohistochemically distinctive perivascular epithelioid cells C [9]. PEComas are a family of related mesenchymal neoplasms that include angiomyolipoma (renal and extrarenal variants), lymphangiomyomatosis, clear cell 'sugar' tumor of the lung, clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ligamentum teres, primary extrapulmonary ''sugar'' tumor and clear cell tumors of diverse sites [10] [11] [12] [13] [14] [15] [16] [17]. "
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    ABSTRACT: Although angiomyolipoma (AML) is a relatively rare entity, it is the most common benign mesenchymal neoplasm of the kidney. To highlight the clinicopathological characteristics of AML and to assess the role of Human Melanoma Black-45 (HMB-45), Melan-A, smooth muscle actin (SMA), S-100 and cytokeratin in its diagnosis. The study included 15 cases of AML. Clinical and radiological data were retrieved from the archival files and all cases were subjected to a histopathological evaluation as well as immunohistochemical staining for HMB-45, Melan-A, SMA, S-100, and cytokeratin. AML was more common in females (female:male=4:1), the mean age was 53.9±6.45years. 60% of patients were symptomatic while the remaining 40% were asymptomatic. A statistically significant relationship was found between size of the tumor and the presence of the symptoms (P=0.02). Patients with tumor size less than 4cm were asymptomatic, while those with tumor size larger than 4cm had different symptoms. Thirteen cases were classic AML, while 2 cases were epithelioid AML. Classic AML demonstrated admixture of fatty tissue, thick-walled blood vessels, and smooth muscle, while epithelioid AML was composed mainly of epithelioid cells and contained no fat. HMB-45 was positive in all cases of AML (100%), Melan-A was positive in 13/15 (87%) while SMA was positive in 11/15 (73%) of AML with variable staining intensity. All cases of AML were negative for S-100 and cytokeratin. AMLs have characteristic clinicopathological and immunohistochemical features and their recognition is crucial for proper diagnosis and treatment.
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    • "Our case is an angiomyolipoma that had originated from the vocal process of the arytenoid and posterior of the the vocal cord. Only a few laryngeal angiomyolipoma cases are available in the literature [6] [7] [8]. "
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    ABSTRACT: Angiomyolipoma is a rare benign mesenchymal tumor, which is mostly renal in origin. A sixty-year-old male patient with the diagnosis of angiomyolipoma located in the larynx has been presented here, and the literature is reviewed.
    Full-text · Article · Oct 2011
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    ABSTRACT: Primary perivascular epithelioid cell neoplasms (PEComas) of bone are rare mesenchymal tumors. Histologically, they are composed predominantly of perivascular epithelioid cells and have the capacity to metastasize. PEComas have been reported within intra-abdominal and intra-pelvic organs. To the best of our knowledge, only seven primary PEComas of bone have been described in the English literature. We present two cases of PEComa of bone, one arising from the distal fibula and one from the acetabulum. Both were treated by surgical excision and one also received adjuvant chemotherapy.
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