PEComa of the Nasal Cavity with Worrisome Histologic Features and Benign Behavior: A Case Report

Bocciolini, Corso; Fornelli, Adele; Casadei, Gian Piero; Cattani, Maria Grazia; Dall’Olio, Danilo

doi:10.1177/000348941312201110

Cited by 7 publications

(3 citation statements)

References 11 publications

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“…PEComas can arise in a variety of anatomic sites. According to our literature review, sites for head and neck PEComas include the nasal cavity [6,7,8,12,14,21,22,25], scalp [5,9,10], neck soft tissue [10], eye [4,11,15,16,19,24,27], oral cavity [14,20], larynx [6,17], pharynx [17,26], face [13], and skull base [18,23]. PEComas arising from sites in the skull base like the jugular foramen are extremely rare.…”

Section: Discussionmentioning

confidence: 99%

“…Our case met these histopathological and immunohistorical features, and ultimately a diagnosis of PEComa was made. Traditionally, PEComas were thought to be benign tumors, but malignant disease courses with local recurrence and distant metastases have been reported [4,8,9,10,14,17,18,23,26]. Folpe et al proposed that tumors with two or more of the following defining features are considered malignant: tumor size >5 cm, infiltrative growth pattern, high nuclear grade and cellularity, mitotic rate 1/50 HPF, necrosis, and vascular invasion [10].…”

Section: Discussionmentioning

confidence: 99%

“…The site of origin of this tumor is extremely variable; however, the head and neck are rare origin sites [3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27], and PEComas arising from the skull base are even more unusual [18,23]. In this report, we describe a case with a PEComa located in the jugular foramen.…”

Section: Introductionmentioning

confidence: 95%

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Malignant perivascular epithelioid cell tumor mimicking jugular foramen schwannoma: A case report and literature review

Komune

Masuda

Yasumatsu

et al. 2020

Heliyon

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Background: Perivascular epithelioid cell tumors (PEComas) of the skull base are extremely rare. Here we report the first description of a malignant PEComa mimicking jugular foramen schwannoma and presenting as Collet-Sicard syndrome, and we review the previous literature on PEComas of the head, neck and skull base. Case description: A 29-year-old woman presented with hoarseness, dysphagia, vomiting, and headache. She was first diagnosed with Collet-Sicard syndrome caused by thrombosis of the sigmoid and transverse sinuses. She was treated with anticoagulant therapy, and the hoarseness and paralysis of the accessory nerve improved. Later, at age 31, the hoarseness again worsened. At another hospital, enhanced computed tomography revealed a tumor in the jugular foramen extending to the neck and medially displacing the internal carotid artery. She was referred to our hospital for further examination and was diagnosed with jugular foramen schwannoma causing thrombosis of the sinuses. At the one-year follow-up, the tumor had grown rapidly and had started to surround the internal carotid artery. We therefore performed a tissue biopsy of the tumor in the jugular foramen and neck. Based on pathological analysis, we made a definitive diagnosis of malignant PEComa. Conclusions: It may be extremely challenging to reach an accurate diagnosis of PEComa in the skull-base region, which can cause a delay in treatment initiation. When atypical clinical features for a skull-base tumor are found, we recommend preliminary biopsy to obtain a definitive diagnosis and initiate an appropriate treatment strategy as early as possible.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Malignant perivascular epithelioid cell tumor mimicking jugular foramen schwannoma: A case report and literature review

Komune

Masuda

Yasumatsu

et al. 2020

Heliyon

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Perivascular epithelioid cell tumor (PEComa) of the pterygopalatine fossa

Dougherty

Payne

Gupta

et al. 2020

Clinical Case Reports

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Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal tumors associated with tuberous sclerosis. These tumors are typically treated with resection and rarely recur or exhibit malignant behavior. A 78‐year‐old woman presented with an incidentally discovered pterygopalatine fossa/retroantral mass. Excisional biopsy was performed and revealed pathology consistent with PEComa. Upon review of the literature, there have been 43 reported cases of PEComa of the head and neck. There is only one previously reported case of PEComa in the skull base, and none reported in the pterygopalatine fossa. Of note, the previously reported case of skull base PEComa involved an aggressive tumor with widespread metastasis. Here, we report the first case of a PEComa of the pterygopalatine fossa/retroantral region, which was treated conservatively. This rare pathology should be considered in the differential diagnosis for atypical skull base tumors.

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Mesenchymal Tumors

Franchi¹

2019

Pathology of Sinonasal Tumors and Tumor-Like Lesions

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PEComa of the Nasal Cavity with Worrisome Histologic Features and Benign Behavior: A Case Report

Cited by 7 publications

References 11 publications

Malignant perivascular epithelioid cell tumor mimicking jugular foramen schwannoma: A case report and literature review

Malignant perivascular epithelioid cell tumor mimicking jugular foramen schwannoma: A case report and literature review

Perivascular epithelioid cell tumor (PEComa) of the pterygopalatine fossa

Mesenchymal Tumors

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