PEComa of the nasal septum

Department of Human Pathology and Oncology, University of Florence, Florence, Italy.
Otolaryngology Head and Neck Surgery (Impact Factor: 2.02). 10/2009; 141(4):543-4. DOI: 10.1016/j.otohns.2009.03.009
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    • "The PEComa family of tumors is now felt to be comprised of AML, CCST, LAM, and less well-characterized PEComas of a variety of other anatomic origins, for which the term " perivascular epithelioid cell tumor not otherwise specified " (PEComa-NOS) has been proposed [6]. PEComa-NOS have been described in a variety of anatomic locations, including the colon [8] [9], pancreas [5] [10], retroperitoneum [11] [12], heart [13] [14], adrenal gland [15], breast [16], eye [17], biliary tract [18], bone [19], urinary bladder [20] [21], skull base [22], liver [23] [24] [25], uterus [26], cervix [27], skin [28], nasopharynx [29] [30], Sarcoma upper airway [31], and soft tissues [32] [33]. The majority of the published literature regarding PEComa-NOS is in the form of case reports and series, with a focus on the presentation and distinguishing pathologic features of the disease. "
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    ABSTRACT: Purpose. Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors characterized by a myomelanocytic phenotype, and PEComas occurring in “nonclassic” anatomic distributions are known as perivascular epithelioid cell tumor not otherwise specified (PEComa-NOS). This review aims to compile and analyze cases of PEComa-NOS in an effort to better define their natural history. Design. We evaluated all 234 cases of PEComa-NOS reported in the English literature, extracting information regarding diagnostic features, treatment approaches, and outcomes. Multivariate analysis of a number of variables evaluable on pathologic review was performed to refine preexisting risk stratification criteria. Outcomes for patients receiving nonsurgical treatment are also reported. Results. Primary tumor size ≥5 cm (P = 0.02) and a high (1/50 HPF) mitotic rate (P < 0.0001) were the only factors significantly associated with recurrence following surgical resection. Cytotoxic chemotherapy and radiation therapy have shown little benefit in treating PEComa-NOS; mTOR inhibition is emerging as a treatment option. Conclusion. Progress has been made in understanding the natural history and molecular biology of PEComa-NOS. This review further clarifies risk of recurrence in this disease, allowing clinicians to better risk stratify patients. Further work should focus on applying this knowledge to making treatment decisions for patients with this disease.
    Sarcoma 04/2012; 2012:541626. DOI:10.1155/2012/541626
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    • "PEComas of the nasal cavity are extremely rare and a total of four cases (including the present case) have been reported in the literature to date, all in females 3-5. The age of the three previously reported cases ranged from 34 to 79 years, with a mean age 54.3 years. "
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    ABSTRACT: Perivascular epithelioid cell neoplasms are a group of rare tumours reported in various organs under a variety of designations. Such tumours are of interest primarily because of the distinctive morphology of their cell population and their immunoreactivity with melanocytic and myoid markers. There is a strong association between perivascular epithelioid cell neoplasms and tuberous sclerosis complex. Perivascular epithelioid cell neoplasms very rarely occur in the upper aero-digestive tract. To date only three cases of nasal perivascular epithelioid cell neoplasms have been reported in the literature. The present report refers to a 22-year old woman, without any stigmata of tuberous sclerosis complex, with early onset of a polypoid nasal mass with pathological and immunohistochemical features entirely compatible with those of a perivascular epithelioid cell neoplasm.
    Acta otorhinolaryngologica Italica: organo ufficiale della Società italiana di otorinolaringologia e chirurgia cervico-facciale 04/2012; 32(3):198-201. · 1.64 Impact Factor
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    ABSTRACT: Neoplasms showing perivascular epithelioid cell differentiation (PEComas) are uncommon tumors of the sinonasal tract. They are often misdiagnosed as angiomyolipoma or a simple benign tumor or polyp. We present a further case of a sinonasal PEComa and review the literature in an attempt to ascertain their malignant potential. Published evidence on invasiveness and characteristics were defined on systematic review. MEDLINE and EMBASE were searched from 1966 and 1980, respectively, to week 3 of December 2010. Publications reporting PEComa or angiomyolipoma were sought. Only those describing a sinonasal origin were included. Demographics, anatomic site, local invasion, recurrence rates, and mortality were recorded. A case report of a locally invasive intranasal PEComa is described. In addition to the case we present, 12 case reports were located (n = 13). The mean age of patients was 59.6 (SD, 14.98 years) years. The location was within the right sinonasal tract in 54% of cases, the left sinonasal tract in 38% of cases, and not reported in 8% of cases. Treatment focused on local surgical excision and this was achieved endoscopically in 100% of cases. Recurrence rate was 8%. Mean follow-up was 17.4 (SD, 20.68 months) months. Invasion was noted in 23% of cases. There was a single death recorded. PEComas of the paranasal sinuses and skull base appear to have a biological behavior different from simple benign angiomyolipomas reported elsewhere in the body. PEComa may be more intermediate or malignant in clinical behavior.
    American Journal of Rhinology and Allergy 05/2012; 26(3):213-7. DOI:10.2500/ajra.2012.26.3760 · 1.81 Impact Factor
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