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Uterine PEComa: appraisal of a controversial and increasingly reported mesenchymal neoplasm

By Oluwole Fadare

Abstract

In recent years, a group of tumors that have been designated "perivascular epithelioid cell tumors" (PEComa) have been reported with increasing frequency from a wide variety of anatomic locations. The uterus and retroperitoneum appear to be the most frequent sites of origin for these lesions. PEComas belong to an identically named family of tumors comprised of conventional angiomyolipomas, clear cell sugar tumors, lymphangiomyomatosis and clear cell myomelanocytic tumor of the falciform ligament/ligament teres, and are also known as PEComa-NOS. This article is a primer for clinicians on the most salient clinicopathologic features of uterine PEComas, as most of the debate and discussion have taken place in the pathologic literature. The author appraises in detail the current state of knowledge on PEComas of the uterus based on a review of published data on the 44 previously reported cases, and comments on areas of controversy. The latter are centered predominantly on the significant morphologic and immunophenotypic overlap that exists between uterine PEComa and some smooth muscle tumors of the uterus. The clinicopathologic features of cases reported as epithelioid smooth muscle tumors and cases reported as uterine PEComas are compared and contrasted, and a practical approach to their reporting is proposed

Topics: Commentary
Publisher: BioMed Central
OAI identifier: oai:pubmedcentral.nih.gov:2278149
Provided by: PubMed Central
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    1. (2005). A: A uterine leiomyosarcoma that became positive for HMB45 in the metastasis. Ann Diagn Pathol
    2. (2004). A: Epithelioid angiomyolipoma of the uterus: a case report. J Reprod Med
    3. (2004). A: Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract.
    4. (2004). A: Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa.
    5. (1999). AG: HMB-45 immunoreactivity is present in both uterine leiomyomas and normal myometrium [abstract]. Mod Pathol
    6. (2007). Albores-Saavedra J: HMB-45 reactivity in conventional uterine leiomyosarcomas.
    7. (1994). Andrion A: Clear cell ("sugar") tumor of the lung is a lesion strictly related to angiomyolipoma–the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology
    8. (2004). Angiomyolipoma of the uterus associated with tuberous sclerosis: case report and review of the literature. Gynecol Oncol
    9. (1995). C: Rodent model of reproductive tract leiomyomata. Clinical and pathological features.
    10. (2006). CD: PEComa: what do we know so far? Histopathology
    11. (2008). CD: Sclerosing PEComa: Clinicopathologic Analysis of a Distinctive Variant With a Predilection for the Retroperitoneum. Am J Surg Pathol in press.
    12. (2007). Chintapalli KN: Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: cross-sectional imaging findings.
    13. (1996). Clear cell "sugar" tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells.
    14. (2008). Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma.
    15. (2001). Couly GF: The cephalic neural crest provides pericytes and smooth muscle cells to all blood vessels of the face and forebrain. Development
    16. (1999). De Antoni E: A uterine pecoma: a case report.
    17. (1943). Die Geschwulste und Gewebsmissbildungen der Nierenrinde. II Midteilung. Die mesenchymalen Neubildungen. Virchows Arch
    18. (1996). DJ: Alternative neural crest cell fates are instructively promoted by TGFbeta superfamily members. Cell
    19. (2001). Epithelioid smooth muscle tumors of the uterus [abstract]. Mod Pathol
    20. (1997). Epithelioid smooth muscle tumors of the uterus. A clinicopathologic study of 80 cases. [abstract]. Mod Pathol
    21. (2003). ES: Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. Pathol Int
    22. (1998). F: Epithelioid lymphangioleiomyomatosis-like tumour of the uterus in a patient without tuberous sclerosis: a lesion mimicking epithelioid leiomyosarcoma. Histopathology
    23. (2007). F: PEComas: the past, the present and the future. Virchows Arch in press.
    24. (2007). F: Perivascular epithelioid cell tumor (PEComa) in the genitourinary tract. Adv Anat Pathol
    25. (1996). F: Perivascular epithelioid cell.
    26. (2003). FF: The tuberous sclerosis complex and its highly variable manifestations.
    27. (1991). Fiore-Donati L: Clear cell tumor and angiomyolipoma [Letter]. Am J Surg Pathol
    28. (2005). G: Perivascular epithelioid cell (PEC) tumors of the uterus: a clinicopathologic study of two cases with aggressive features. Mod Pathol
    29. (2003). Gittenberger-de Groot AC: Folic acid and homocysteine affect neural crest and neuroepithelial cell outgrowth and differentiation in vitro. Dev Dyn
    30. (1997). GM: The perivascular epithelioid cell and related lesions. Adv Anat Pathol
    31. (2005). Gonzalez-Tejero C: The PEComa Tumor: Could it be considered an independent neoplastic entity. J Gynecol Surg
    32. (2000). Hendrickson MR: Smooth muscle, endometrial stromal, and mixed Müllerian tumors of the uterus. Mod Pathol
    33. (1976). HJ: Mesenchymal tumors of the uterus. VI. Epithelioid smooth muscle tumors including leiomyoblastoma and clear-cell leiomyoma: a clinical and pathologic analysis of 26 cases. Cancer
    34. (2007). HM: Perivascular epithelioid cell tumor of the uterus: report of three cases. Chin Med J (Engl)
    35. (2001). HMB45+ hyalinized epithelioid tumor of the uterus is linked to epithelioid leiomyoma rather than to PEC-omas.
    36. (2000). Hyalinized Uterine Mesenchymal Neoplasms with HMB-45-Positive Epithelioid Cells: Epithelioid Leiomyomas or Angiomyolipomas? Report of Four Cases
    37. (2006). JD: Uterine lipoleiomyomas: a clinicopathologic study of 50 cases.
    38. (2001). JK: Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. Mod Pathol
    39. (2002). JW: Sonographic evaluation of a uterine angiolipoleiomyoma.
    40. (2008). K: CD1a expression in PEComas. Pathol Int
    41. (1993). Lachard A: Uterine angiomyolipoma associated with pregnancy. Gynecol Oncol
    42. (2003). Late pulmonary metastasis in uterine PEComa.
    43. (2002). Le Douarin NM: Morphogenesis of the branchial vascular sector. Trends Cardiovasc Med
    44. (1996). Lipoleiomyomatous tumors of the uterus: a heterogeneous group? Histopathological study of five cases. Pathol Int
    45. (1995). Lymphangiomyomatosis of the uterus associated with tuberous sclerosis and malignant neoplasia of the female genital tract: a report of two cases.
    46. (2005). McCluggage WG: Uterine leiomyosarcoma with HMB45+ clear cell areas: report of two cases. Histopathology
    47. (1998). ME: In vitro system for differentiating pluripotent neural crest cells into smooth muscle cells.
    48. Mesenchymal tumors and related lesions.
    49. (1995). MR: Extrapulmonary lymphangioleiomyomatosis and lymphangiomatous cysts in tuberous sclerosis complex. Mayo Clin Proc
    50. Neoplasms with perivascular epithelioid cell differentiation (PEComas). In World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone Edited by:
    51. (2007). Parwani AV: Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature. Diagn Pathol
    52. (2002). PB: An immunohistochemical analysis of endometrial stromal and smooth muscle tumors of the uterus: a study of 54 cases emphasizing the importance of using a panel because of overlap in immunoreactivity for individual antibodies.
    53. (2004). Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. World J Surg Oncol
    54. (2008). Perivascular epithelioid cell tumor [PEComa] of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv Anat Pathol
    55. (2004). Perivascular Epithelioid Cell Tumor Not Otherwise Specified (PEComa-NOS)
    56. (2004). Perivascular epithelioid cell tumor of the uterus: a case report.
    57. (2006). Perivascular epithelioid cell tumor of the uterus: a case report. Pathologica
    58. (2005). Perivascular epithelioid cell tumor of the uterus: report of four cases.
    59. (2007). Perivascular Epithelioid Cell Tumors and Smooth Muscle Tumors of the Uterus [Letter]. Am J Surg Pathol
    60. (1986). Priest JB: Clear cell leiomyoma (leiomyoblastoma) of the uterus: ultrastructural observations. Ultrastruct Pathol
    61. (2003). Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature. Gynecol Oncol
    62. (1989). RJ: Pigmented myomatous neurocristoma of the uterus. Arch Pathol Lab Med
    63. (2002). RL: Perivascular epithelioid cell tumor ('PEComa') of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors.
    64. (2000). RT: HMB-45 immunoreactivity in uterine mesenchymal tumors [abstract]. Mod Pathol
    65. (1994). RV: A comparative immunohistochemical study of uterine smooth muscle neoplasms with emphasis on the epithelioid variant. Hum Pathol
    66. (2001). Sasano H: Uterine angiomyolipoma: case report and review of the literature. Pathol Int
    67. (2005). SM: Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus. J Pediatr Hematol Oncol
    68. (2006). Soslow R: Expression of melanocytic ("PEComa") markers in smooth muscle tumors of the uterus: an immunohistochemical analysis of 86 cases [abstract]. Mod Pathol
    69. (2000). SW: Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: a novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults.
    70. (2005). SW: Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.
    71. (2006). Tavassoli FA: Mesenchymal lesions of the uterine cervix. Pathol Case Reviews
    72. (1996). Teng NN: Lymphangioleiomyomatosis of the uterus simulating high-stage endometrial stromal sarcoma. Gynecol Oncol
    73. (1989). TL: The clear-cell variant of uterine epithelioid leiomyoma. An immunohistologic and ultrastructural study. Arch Pathol Lab Med
    74. (2008). Tornos C: "Malignant" uterine perivascular epithelioid cell tumor, pelvic lymph node lymphangioleiomyomatosis, and gynecological pecomatosis in a patient with tuberous sclerosis: a case report and review of the literature.
    75. (2000). TW: HMB-45 immunoreactivity in uterine mesenchymal tumors. Mod Pathol
    76. (2006). Unusual variants of malignant melanoma. Mod Pathol
    77. (1995). Uterine leiomyosarcoma with clear cell areas.
    78. (2006). Uterine perivascular epithelioid cell tumour presenting as a cervical mass. J Pak Med Assoc
    79. (1997). WR: Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients.
    80. (2007). Yeung RS: Activation of the mTOR pathway in sporadic angiomyolipomas and other perivascular epithelioid cell neoplasms. Hum Pathol
    81. (2006). YJ: Comparative genomic hybridization study of perivascular epithelioid cell tumor: molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm. Hum Pathol

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