Franchi, Alessandro

Gallo, Oreste

Panelos, J.

Scala, I.

English

Florence Research

20 December 2021Neoplasm with perivascular epithelioid cell differentiation (PEComa) of the nasal septum / J. Panelos; O. Gallo; I. Scala;A. Franchi. - In: OTOLARYNGOLOGY-HEAD AND NECK SURGERY. - ISSN 0194-5998. - STAMPA. - 141(2009), pp.543-544.Original Citation:Neoplasm with perivascular epithelioid cell differentiation (PEComa) of the nasalseptumTerms of use:Publisher copyright claim:(Article begins on next page)La pubblicazione è resa disponibile sotto le norme e i termini della licenza di deposito, secondo quanto stabilito dallaPolicy per l'accesso aperto dell'Università degli Studi di Firenze (https://www.sba.unifi.it/upload/policy-oa-2016-1.pdf)Availability:This version is available at: 2158/394692 since:Questa è la Versione finale referata (Post print/Accepted manuscript) della seguente pubblicazione:FLORERepository istituzionale dell'Università degli Studi diFirenzeOpen AccessOtolaryngology–Head and Neck Surgery (2009) 141, 543-544CASE REPORTPEComa of the nasal septumJohn Panelos, MD, Oreste Gallo, MD, Iacopo Scala, MD, andAlessandro Franchi, MD, Florence, ItalyNo sponsorships or competing interests have been disclosed forthis article.Neoplasms showing perivascular epithelioid cell differ-entiation (PEComas) constitute a group of tumors com-posed of distinctive perivascular cells that coexpress mela-nocytic and muscle markers.1 This family of tumorsincludes angiomyolipoma, clear cell sugar tumor of thelung, lymphangiomatosis, clear cell myomelanocytic tumorof the falciform ligament/ligamentum teres, and other un-usual clear cell tumors arising at a variety of visceral or softtissue sites.1-4 In recent years, there has been an increase inthe number of reported cases of PEComas, and it nowappears that these tumors may arise at any anatomic loca-tion. We report the second case of PEComa occurring in thenasal cavity.2A 50-year-old woman visited our hospital complainingof epistaxis, rhinorrhea, and unilateral nasal obstruction.Physical examination revealed a well-demarcated exophyticlesion attached to the anterior portion of the nasal septum bya relatively broad base. The patient’s clinical history wasotherwise unremarkable, and there was no family history oftuberous sclerosis. The lesion involved only the mucosa andcould be excised surgically without septal perforation. Re-covery was uneventful, and six years after excision, thepatient is alive and well without evidence of local recur-rence or metastatic disease.Microscopic examination revealed a well-circumscribedbut not encapsulated subepithelial lesion composed of fas-cicles and nests of plump epithelioid and spindled cellsseparated by a rich network of thin-walled capillary-sizedvessels (Fig 1). Tumor cells presented clear to granularlightly eosinophilic cytoplasm with small, centrally placed,round to ovoid nuclei. There were rare mitoses and nonecrosis. The overlying respiratory epithelium did not showany alteration.The immunohistochemical stains showed a diffuse vi-mentin and -smooth muscle actin positivity, whereasdesmin and HMB-45 were focally positive (Fig 2). Epithe-lial membrane antigen, cytokeratins, CD10, CD31, CD34,S-100 protein, melanoma-associated antigen recognized byT cells (MART-1), estrogen receptor, and progesterone re-ceptor immunostains were negative. Furthermore, aboutReceived December 15, 2008; revised February 9, 2009; accepted March 10,0194-5998/$36.00 © 2009 American Academy of Otolaryngology–Head and Necdoi:10.1016/j.otohns.2009.03.009three percent of the neoplastic cells were in cell cycle, asestimated with the Ki-67 marker.Ultrastructurally, neoplastic cells contained numerousdense granules, whereas bundles of microfilaments wererecognized in the peripheral cytoplasm of few neoplasticelements. The institutional review board of our hospitalapproved this study.DISCUSSIONPEComas have been described only rarely in the head andneck region, and they involved the nasal cavity,2 the softtissues,3 and the oral mucosa.5 The differential diagnosis ofnasal PEComa includes several entities. Malignant mela-noma can be distinguished from PEComa by the expressionof S-100 protein and the absence of actin immunoreactivity,even if rare spindled melanomas may show limited actinexpression and S-100–negative melanomas have been de-scribed. In such cases, perivascular accentuation of tumorcells and strong and diffuse melanocytic marker positivityare additional important features. Epithelioid PEComa maybe confused with metastatic clear cell renal carcinoma. TheFigure 1 The tumor consisted of a uniform population of spin-dle and epithelioid cells mostly showing a clear cytoplasm.2009.k Surgery Foundation. All rights reserved.544 Otolaryngology–Head and Neck Surgery, Vol 141, No 4, October 2009expression of melanocytic markers and the lack of reactivityfor cytokeratins and CD10 are decisive for the diagnosis ofthe former. Paraganglioma is characterized by a more or-ganoid growth pattern and positivity for protein S-100,chromogranin A, and synaptophysin. Morphologic evalua-tion is the cardinal criterion in distinguishing PEComasfrom true smooth muscle tumors because the role of immu-nohistochemistry in such differential diagnosis is lessstraightforward. PEComas show clear to lightly eosino-philic cytoplasm and round to oval nuclei with small nucle-oli instead of the diffuse cytoplasmic eosinophilia and the“cigar-shaped” nuclei of the smooth muscle tumors that typ-ically lack the delicate capillary network seen in PEComas. Insinonasal hemangiopericytoma, neoplastic cells show am-phophilic to eosinophilic cytoplasm and are organized inshort fascicles, often around vascular channels ranging fromcapillary size to large sinusoidal spaces, thus mimicking thehistologic picture of PEComa. Both tumors are positive formyogenic markers, but sinonasal hemangiopericytoma doesnot express melanocytic markers.Clinically, the vast majority of PEComas follow a benigncourse, although a subset of these tumors behaves in amalignant fashion. Malignant PEComas can be very aggres-sive, with clinical behavior similar to a high-grade sarcoma.In a recently published study, criteria for malignancy weresuggested,3 but, until more cases with long clinical fol-low-up are evaluated in a systematic fashion, firm criteriaFigure 2 Focal immunostaining for HMB45 is present.for malignancy remain uncertain. Our patient is alive andwell without evidence of local recurrence or metastaticdisease six years after the surgical excision of the lesion,indicating a benign clinical course.AUTHOR INFORMATIONFrom the Departments of Human Pathology and Oncology (Drs Panelosand Franchi) and Oto-neuro-ophtalmologic Surgery (Drs Gallo and Scala),University of Florence, Florence, Italy.Corresponding author: Alessandro Franchi, MD, Department of HumanPathology and Oncology, Viale G.B. Morgagni 85, 50134 Firenze, Italy.E-mail address: Franchi@unifi.it.AUTHOR CONTRIBUTIONSJohn Panelos, acquisition of histopathological data, writing of the manu-script, final approval of the version to be published; Oreste Gallo, acqui-sition of clinical data, review of the manuscript, final approval of theversion to be published; Iacopo Scala, acquisition of clinical data, reviewof the manuscript, final approval of the version to be published; Alessan-dro Franchi, acquisition of histopathological data, writing of the manu-script, final approval of the version to be published.DISCLOSURESCompeting interests: None.Sponsorships: None.REFERENCES1. Folpe AL. Neoplasms with perivascular epithelioid cell differentiation(PEComas). In: Fletcher CDM, Unni KK, Mertens F, editors. WorldHealth Organization classification of tumours: pathology and geneticsof soft tissue and bone. Lyon, France: IARC Press; 2002. p. 221–2.2. Banerjee SS, Eyden B, Trenholm PW, et al. Monotypic angiomyoli-poma of the nasal cavity: a heretofore undescribed occurrence. IntJ Surg Pathol 2001;9:309–15.3. Folpe A, Mentzel T, Lehr HA, et al. Perivascular epithelioid cellneoplasms of soft tissue and gynecologic origin: a clinicopathologicstudy of 26 cases and review of the literature. Am J Surg Pathol2005;29:1558–75.4. Govender D, Sabaratnam RM, Essa AS. Clear cell “sugar” tumor of thebreast; another extrapulmonary site and review of the literature. Am JSurg Pathol 2002;26:670–5.5. Koutlas IG, Pambuccian SE, Jessurun J, et al. Perivascular epithelioidcell tumor of the oral mucosa. Arch Pathol Lab Med 2005;129:690–3.

Neoplasm with perivascular epithelioid cell differentiation (PEComa) of the nasal septum

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Neoplasm with perivascular epithelioid cell differentiation (PEComa) of the nasal septum

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