Alveolar Soft-Part Sarcoma of Paranasal Sinuses

Abstract

A 23-year-old woman presented with symptoms of nasal obstruction of 5 years'duration, originally diagnosed as sinusitis. Clinical examination revealed bilateral polyps in the maxillary sinus, from which a biopsy was taken. This showed a tumor with a well-defined nesting (Zellballen) pattern (Figure 1), composed of large cells, some of them of giant size, with an acidophilic cytoplasm with ragged edges. The nuclei were large, and the nucleoli were very prominent ( Figure 2). Despite the high degree of cytologic atypia, mitotic activity was practically nil. The PAS (periodic acid-Schiff) stain with diastase control showed the presence, in many of the tumor cells, of a PAS-positive diastase-resistant material in the Golgi region ( Figure 3). Some of these clumps had an appearance suggestive of early crystal formation (Figure 3, arrows).The main differential diagnosis was between alveolar soft-part sarcoma, paraganglioma, and PEComa (perivascular epithelioid cell tumor). The negativity for HMB-45, Melan-A, and the neuroendocrine markers (synaptophysin, chromogranin, and neuron-specific enolase) ruled out the latter 2 alternatives. The diagnosis of alveolar soft-part sarcoma was fully supported by the strong nuclear positivity for TFE3 (transcription factor E3; Figure 4), which Figure 1. Large tumor cells with acidophilic cytoplasm forming nests Figure 2. Moderate nuclear pleomorphism in tumor cells represents the immunohistochemical expression of the chromosomal unbalanced translocation der(17)t(X;17) (p11.2;q25), which results in the ASPL-TFE3 gene fusion.The upper aerodigestive tract is a relatively common site for this rare tumor. Tumors in this location tend to occur in younger patients and to have a nesting rather than alveolar pattern of growth, which may render their recognition difficult. Surgical excision combined with chemotherapy or radiation therapy is the recommended treatment. 1-4

Alveolar Soft-Part Sarcoma in Olfactory Cleft: A Case Report and Clinicopathological Review

“…11 Sinonasal involvement has been reported in 7 cases. 4,6,8,[13][14][15][16] Only one of these cases involved the nasal septum and cribriform plate. 6 In this study, we report a case of ASPS presented as a cribriform plate mass.…”

“…1 Moreover, in regard to molecular genetics, ASPS is characterized by unbalanced translocations of der(17)t(X;17)(p11;q25) that result in the ASPL-TFE3 gene fusion. 13 To date, there is no consensus on the best treatment plan for sinonasal ASPS, but radical surgical excision with tumor-free margins is preferred. 4 Radiotherapy, which produces improved local control, is recommended following subtotal surgical removal.…”

“…1 Moreover, in regard to molecular genetics, ASPS is characterized by unbalanced translocations of der(17)t(X;17)(p11;q25) that result in the ASPL-TFE3 gene fusion. 13…”

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

“…ASPS has been described to occur mostly in the extremities of young adults, [1,2] the head and neck region in children, [3] and other locations rarely and in isolation. [4][5][6][7][8] Orbital involvement is rare with only few reports published in literature. [9][10][11][12][13] Although it has an indolent clinical course, ASPS is known to metastasize.…”