Alveolar Soft Part Sarcoma

Zarrin-Khameh, Neda; Kaye, Kim Sutker

doi:10.5858/2007-131-488-asps

Cited by 54 publications

(6 citation statements)

References 37 publications

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“…Microscopically, uniform rounded and often polygonal cells are separated by dense fibrous trabeculae into nests and sometimes show loss of cellular cohesion, resulting in a pseudo-alveolar pattern (Figure 3A). The findings of positive diastase-resistant periodic acid-Schiff (PAS) staining combined with a cytoplasmic crystalloid structure helps support the diagnosis (26). In terms of immunohistochemistry findings, ASPS are typically positive for Cathepsin K and can be focally positive for desmin, while being largely negative for cytokeratin, EMA, HMB-45, melan-A, and synaptophysin.…”

Section: Pathological Discussionmentioning

confidence: 97%

Multidisciplinary sarcoma tumor board: adolescent and young adult soft tissue sarcoma—myxoid liposarcoma and alveolar soft part sarcoma

Jayachandran¹,

Patel²,

Hu³

et al. 2020

Chin Clin Oncol

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Section: Pathological Discussionmentioning

confidence: 97%

Multidisciplinary sarcoma tumor board: adolescent and young adult soft tissue sarcoma—myxoid liposarcoma and alveolar soft part sarcoma

Jayachandran¹,

Patel²,

Hu³

et al. 2020

Chin Clin Oncol

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“…These characteristic intracytoplasmic crystals may be seen in at least 80% of ASPS cases. 3,4 Absence of the crystals does not rule out ASPS. The main differential diagnoses are similar to cytology (mentioned earlier).…”

Section: Discussionmentioning

confidence: 98%

“…In these two cases, intracytoplasmic PAS‐positive diastase‐resistant (PAS‐D) rhomboid or needle‐shaped crystals are presented in cells in histology sections. These characteristic intracytoplasmic crystals may be seen in at least 80% of ASPS cases . Absence of the crystals does not rule out ASPS.…”

Section: Discussionmentioning

confidence: 98%

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Fine‐needle aspiration of alveolar soft part sarcoma: Histologic correlation and aberrant CD68 expression

Zhou

et al. 2018

Diagnostic Cytopathology

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Alveolar soft part sarcoma is a rare highly malignant neoplasm of the soft tissue and usually occurs in the lower extremities of children and young adults. We report two cases of alveolar soft part sarcoma: a 24-year-old Latino man with a 10-cm neck mass and a 56-year-old Latino woman with a recurring thigh mass. Fine-needle aspiration and a core biopsy were performed on both, which was followed by tumor resection on the man. The smears displayed numerous loosely cohesive or single large cells with abundant granular cytoplasm, round nuclei, vesicular chromatin, and occasional prominent nucleoli. Periodic and Schiff (PAS)-positive, diastaseresistant rhomboid, or needle-shaped crystals were present. Both tumors had diffuse and strong nuclear TFE3 expression and aberrant cytoplasmic CD68 expression. Fluorescence in situ hybridization analysis was performed in the first case, which detected a characteristic translocation t(X;17)(p11;q25). The diagnosis of alveolar soft part sarcoma was rendered in both cases.Herein, we present the cytology, histology, immunohistochemistry, and molecular findings and discuss the differential diagnosis. K E Y W O R D S aberrant CD 68 expression, alveolar soft part sarcoma, crystal, fine-needle aspiration, TFE3 expression 1 | INTRODUCTION Alveolar soft part sarcoma (ASPS) is a rare highly malignant neoplasm and accounts for 0.2%-0.9% of the soft tissue sarcoma. 1 A female predominance is seen in patients younger than 30 years. 2,3 ASPS usually arises from deep soft tissue of the thigh or buttock. It usually affects the head and neck region such as tongue or orbit in infants and children. 2 Primary ASPS of visceral organs has also been reported. 4 ASPS usually presents as a solitary, painless, slowly growing soft tissue mass. Rarely, it may have an aggressive course. 5 The local recurrence rate is 20%-30%. 6 The most common metastatic sites are the brain and the lung. Current metastases in the brain and lung occur in approximately 30% of the patients. Prognosis is poor with survival of 12-17 months. 7,8 Cytogenetic study of ASPS has shown the unbalanced translocation der(17)t(X;17)(p11;q25) producing the fusion of the N-terminal region of the alveolar soft part sarcoma locus (ASPL) gene on17q25 to the C-terminal of transcription factor E3 (TFE3) on Xp11. This results in two types of fusion transcripts (ASPL-TFE3 types 1 and 2), which lead to tumorigenesis by transcriptional deregulation. 9 The overexpressed fusion protein can be detected by immunohistochemistry (IHC) with anti-TFE3 antibody. 10,11 There is limited published literature regarding the cytological features of ASPS. Extremely rare cases with diffuse immunoreactivity for CD68 in ASPS have been reported. 12,13 2 | CASE REPORTS 2.1 | Clinical history Case 1: The patient was a 24-year-old Latino man with a history of intellectual disability due to cerebral palsy presented with a posterior neck mass for a year. The mass had been enlarging over a few months. Physical examination revealed a 10 cm well-circumscribed soft mass arising...

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“…A diagnosis is typically made through a combination of imaging studies, such as MRI or CT scans, and a tumour tissue biopsy. TFE-3 is an important immunohistochemical stain that stains the nuclei of tumour cells [ 3 ]. Despite its indolent behaviour, ASPS has a high potential for metastasis to the lungs, bones, liver, soft tissue, and brain.…”

Section: Introductionmentioning

confidence: 99%

Alveolar Soft Part Sarcoma of the Tongue: A Rare Tumor at an Unusual Location

Qureshi,

Shalaby,

Al Hasani

et al. 2023

Cureus

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Alveolar soft part sarcoma (ASPS) is a rare type of soft tissue sarcoma that typically affects adolescents and young adults, though it can occur at any age. We report a case of ASPS of the tongue, which is extremely rare at this location. The patient presented with a polypoidal lesion on the tongue, a biopsy of which showed granular and alveolar morphology. A definitive diagnosis was not rendered due to limited tissue. The case was discussed with the treating surgeon, and excision was recommended with clear margins. Excision of the lesion showed typical ASPS. A TFE-3 immunohistochemical stain was done, which showed strong immunoreactivity, thereby confirming the diagnosis of ASPS. This tumour is rare, and its presence in the tongue makes it extremely infrequent.

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Alveolar Soft Part Sarcoma

Cited by 54 publications

References 37 publications

Multidisciplinary sarcoma tumor board: adolescent and young adult soft tissue sarcoma—myxoid liposarcoma and alveolar soft part sarcoma

Multidisciplinary sarcoma tumor board: adolescent and young adult soft tissue sarcoma—myxoid liposarcoma and alveolar soft part sarcoma

Fine‐needle aspiration of alveolar soft part sarcoma: Histologic correlation and aberrant CD68 expression

Alveolar Soft Part Sarcoma of the Tongue: A Rare Tumor at an Unusual Location

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