Rapid growth of an orbital hemangiopericytoma with atypical histopathological findings

Pacheco, Luiz F. Regis; Fernandes, Bruno F.; Miyamoto, Cristina; Maloney, Shawn C.; Arthurs, Bryan; Burnier, Miguel N.

doi:10.2147/opth.s47901

Cited by 3 publications

(1 citation statement)

References 7 publications

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“…However, the classical picture seen are solitary fibrous tumors: monotonous cellular proliferation without significant variability in cellularity and minimal collagenization was not seen in this case [22]. More importantly, hemangiopericytomas/solitary fibrous tumors are composed of ovoid to spindle-shaped cells separated by sinusoidal spaces, the so-called “staghorn” pattern, which was not observed [23]. Other factors that helped rule out an orbital melanoma were the lack of S-100 protein staining, significant cytological atypia, and mitotic activity [8].…”

Section: Discussionmentioning

confidence: 86%

Giant Perivascular Epithelioid Cell Tumor of the Orbit: A Clinicopathological Analysis and Review of the Literature

et al. 2018

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Aim: To describe and review the clinical, radiological, and histopathological characteristics of an orbital perivascular epithelioid cell tumor (PEComa). Methods: A systematic review of clinical records, radiological investigations, microscopic features, and immunohistochemical characteristics was done. Results: A 9-year-old female child presented with a year-long history of a large orbital mass associated with painless, progressive proptosis of the right eye. Radiologically, a well-defined orbital mass was seen with no intracranial extension. Excision was performed and histopathological examination showed uniform epithelioid cells in nests separated by thin fibrovascular septae. The tumor cells stained positively for Human Melanoma Black-45, but negatively for desmin, S-100, smooth muscle actin, MyoD1, microphthalmia-associated transcription factor, vimentin, CD10, CD31, and CD34 with a low proliferation index of 5–7%. Based on the tumor’s morphological and immunohistochemical characteristics, a diagnosis of giant orbital PEComa was made. No recurrence was seen at the last follow-up. Conclusions: PEComas are uncommon mesenchymal neoplasms that have typical histological features, with an immunohistochemical profile of negativity for epithelial markers and positivity for melanocytic markers. For benign PEComas, complete excision is advised. However, since PEComas elsewhere in the body have been known to be malignant, a close follow-up of such cases is recommended.

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Section: Discussionmentioning

confidence: 86%

Giant Perivascular Epithelioid Cell Tumor of the Orbit: A Clinicopathological Analysis and Review of the Literature

et al. 2018

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Seltene Erkrankungen der Orbita

Kisser

Heichel²,

Glien

2021

Laryngorhinootologie

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ZusammenfassungDieser Übersichtsartikel stellt eine Zusammenstellung seltener Erkrankungen der Orbita dar. Es werden angeborene Fehlbildungen und strukturelle Läsionen, entzündliche Erkrankungen, gutartige und bösartige epitheliale sowie nicht epitheliale Tumoren erörtert. Da in der Orbita zahlreiche Gewebetypen auf engstem Raum beieinanderliegen, kommen dort besonders viele Erkrankungen vor, sodass der Vollständigkeit und dem Tiefgang bei den einzelnen Abhandlungen natürliche Grenzen gesetzt sind. Die Kapitel beinhalten eine Übersicht über das jeweilige klinische Erscheinungsbild, wichtige Stichpunkte zur Diagnostik, Therapie und gegebenenfalls zur Prognose der Erkrankung. Dabei wurde versucht, die besonders charakteristischen Merkmale der einzelnen Erkrankungen herauszuarbeiten, sodass trotz der kurzen Darstellung die relevanten Aspekte enthalten sind.

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A Clinical Case of 5 Times Irradiated Recurrent Orbital Hemangiopericytoma

et al. 2021

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Orbital hemangiopericytomas (HPCs) are rare mesenchymal tumors with a high tendency to recur. Treatment options are quite limited in case of a relapse, but re-irradiation can be useful. Most of the available data limit the possibility of re-irradiation, while novel techniques (e.g., pencil beam proton therapy [PT]) open new approaches for the safe repeating of treatment. To the best of our knowledge, this is the first well-documented case of multi-times (>3) irradiation of tumors located intracranially. The case reported here describes orbital HPCs with proton irradiation performed two times since 1999 in a 30-year-old woman with a medical history as well as surgery followed by conventional radiotherapy (RT) and chemotherapy, and two times stereotactic RT (in 2009 and 2013).In 2016 the patient came to our hospital with the 3rd relapse of the tumor, located in the left orbit, with an intracranial part, involving cavernous sinus, which was close to the temporal lobe. The 4th course of irradiation was done in May to June 2016 by pencil beam PT. Radiation necrosis occurred after 2 years and was treated with bevacizumab (BVZ). Three years later, another relapse was treated with PT and BVZ. The 9-month follow-up showed complete tumor response without signs of brain toxicity. The patient died due to a brain abscess 1 year after the 5th irradiation. This case shows a possibility of irradiation, applied 5 times to the same location, with promising results and manageable toxicity.

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Rapid growth of an orbital hemangiopericytoma with atypical histopathological findings

Cited by 3 publications

References 7 publications

Giant Perivascular Epithelioid Cell Tumor of the Orbit: A Clinicopathological Analysis and Review of the Literature

Giant Perivascular Epithelioid Cell Tumor of the Orbit: A Clinicopathological Analysis and Review of the Literature

Seltene Erkrankungen der Orbita

A Clinical Case of 5 Times Irradiated Recurrent Orbital Hemangiopericytoma

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