Orbital alveolar soft-part sarcoma: Clinico-pathological profiles, management and outcomes

Mulay, Kaustubh; Ali, Mohammad Javed; Honavar, Santosh G; Reddy, Vijay Anand P.

doi:10.4103/0973-1482.136570

Cited by 14 publications

(5 citation statements)

References 39 publications

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“…The exact tissue of origin of ASPS is not clear, however, a few studies suggest that the tumor arises from the extraocular muscles, pointing toward a myogenic origin. [ 5 9 12 ] In our series, the recti muscles were found to be involved in two patients. The histopathology is characterized by a pseudo-alveolar pattern consisting of round to polygonal tumor cells having distinct borders and epithelioid appearance separated by delicate fibrovascular septae.…”

Section: Discussionmentioning

confidence: 59%

“…[ 5 7 12 ] The visual acuity was impaired in only one out of nine patients reported by Mulay et al . [ 12 ] However, in our study, three patients had optic nerve compression noted on imaging and another patient had extensive mass leading to a total loss of vision in the affected eye.…”

Section: Discussionmentioning

confidence: 99%

“…[ 5 14 ] PAS-positive, diastase-resistant intracytoplasmic crystals and granules are characteristic and are present in 80% of the cases. [ 12 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 17 18 ] ASPS is known to metastasize late in the course of the disease years after the initial diagnosis which mandates a long-term follow-up. [ 12 ]…”

Section: Discussionmentioning

confidence: 99%

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Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma – An experience from a tertiary eye-care center

Koka

Singh

Nisar

et al. 2021

Indian Journal of Ophthalmology

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Purpose: To describe the clinic-radiological, pathological profile, and management outcomes of primary alveolar soft-part sarcoma (ASPS) of the orbit. Methods: A retrospective analysis of all histopathologically proven cases of orbital ASPS that presented between May 2016 and September 2019 was done. Data collected included demographics, clinical features, imaging characteristics, metastatic workup, management, and follow-up. Results: Five patients, of which four were males, presented to us during the study period. The mean age of presentation was 12.6 years (range 3–22 years). The most common presenting features were abaxial proptosis ( n = 4) and diminished vision ( n = 4). Imaging showed a well-defined orbital mass in all patients with internal flow voids in three. Preoperative percutaneous embolization with cyanoacrylate glue was done in these three patients owing to high vascularity. Four patients underwent complete tumor excision. One patient underwent exenteration. Histopathology showed polygonal tumor cells arranged in a pseudo-alveolar pattern and Periodic Acid-Schiff (PAS) positive crystals in the cytoplasm in all patients. One patient had systemic metastasis at presentation and developed a local recurrence after 3 months. No recurrence or metastasis was noted in the remaining four patients at a mean final follow-up of 11.2 months (range 5–15 months). Conclusion: ASPS is a rare orbital neoplasm that poses a diagnostic and therapeutic challenge. Imaging might show a soft-tissue tumor with high vascularity. Multidisciplinary management with interventional radiologists for preoperative embolization of vascular lesions helps minimize intraoperative bleeding and aids in complete tumor resection. A localized orbital disease carries a better prognosis.

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Section: Discussionmentioning

confidence: 59%

Section: Discussionmentioning

confidence: 99%

“…[ 5 14 ] PAS-positive, diastase-resistant intracytoplasmic crystals and granules are characteristic and are present in 80% of the cases. [ 12 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 17 18 ] ASPS is known to metastasize late in the course of the disease years after the initial diagnosis which mandates a long-term follow-up. [ 12 ]…”

Section: Discussionmentioning

confidence: 99%

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Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma – An experience from a tertiary eye-care center

Koka

Singh

Nisar

et al. 2021

Indian Journal of Ophthalmology

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“…Morphologically, ASPS (especially the solid type) may resemble PEComas in some ways. Furthermore, the orbit is a relatively common location for ASPS and is also seen in the pediatric age group [20, 21]. However, the cytoplasmic crystals encountered in >80% of ASPSs are absent in PEComas [8].…”

Section: Discussionmentioning

confidence: 99%

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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Pathology of the Orbit: Neoplasms

Logunova

Roden

Salomão

2022

Albert and Jakobiec's Principles and Practice of Ophthalmology

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Orbital alveolar soft-part sarcoma: Clinico-pathological profiles, management and outcomes

Abstract: Early diagnosis with a multidisciplinary, multimodal approach of treatment is quintessential in achieving a long disease-free survival in patients with ASPS.

Cited by 14 publications

References 39 publications

Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma – An experience from a tertiary eye-care center

Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma – An experience from a tertiary eye-care center

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

Pathology of the Orbit: Neoplasms

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