Cytopathological diagnosis of alveolar soft part sarcoma, a rare soft tissue neoplasm

Agarwal, Shipra; Gupta, Ruchika; Iyer, V. K.; Mathur, Sandeep; Ray, Ruma

doi:10.1111/j.1365-2303.2010.00796.x

Cited by 11 publications

(25 citation statements)

References 7 publications

(34 reference statements)

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“…Individual tumor cells have abundant finely vacuolated cytoplasm, resembling histiocytes, with indistinct cell borders, central to eccentric nuclei and prominent eosinophilic nucleoli. Cytoplasmic fragility, detached cytoplasmic fragments, and presence of numerous bare nuclei in a granular background are consistent features, bare nuclei being of diagnostic importance and absent in most lesions that enter in the differential diagnosis . Intranuclear cytoplasmic inclusions and binucleation are other less consistently identified features .…”

Section: Discussionsupporting

confidence: 71%

“…Vague acinar pattern and three‐dimensional structures with endothelial wrapping, corresponding to the tumor nests on histology, are less commonly observed . Metachromatic basement membrane‐like material is seen between cells within tumor fragments and can be thick or thin thread‐like, as in our case . Individual tumor cells have abundant finely vacuolated cytoplasm, resembling histiocytes, with indistinct cell borders, central to eccentric nuclei and prominent eosinophilic nucleoli.…”

Section: Discussionmentioning

confidence: 57%

“…Tumor cells are dispersed singly or in small discohesive aggregates with multiple tumor nuclei embedded in a syncytium of granular cytoplasm. Vague acinar pattern and three‐dimensional structures with endothelial wrapping, corresponding to the tumor nests on histology, are less commonly observed . Metachromatic basement membrane‐like material is seen between cells within tumor fragments and can be thick or thin thread‐like, as in our case .…”

Section: Discussionmentioning

confidence: 93%

“…Diagnosis of ASPS on cytology remains a challenge due to its rarity . Aspirates are moderately cellular, with a hemorrhagic background attributed to the rich vascularity.…”

Section: Discussionmentioning

confidence: 93%

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Metastatic alveolar soft part sarcoma of the lung: Metastatic alveolar soft part sarcoma of the lung‐a morphologic pitfall on cytology and aberrant CD10 expression on histology

Nambirajan

Jain

Malik

et al. 2015

Diagnostic Cytopathology

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Alveolar soft part sarcoma (ASPS) is a rare aggressive soft tissue sarcoma of young adults, typically arising in the deep soft tissue of lower extremities. Although cytomorphology is characteristic enough for an accurate diagnosis in typical clinical scenarios, problems arise when it occurs in older patients, atypical sites, or in primary evaluation at metastatic sites. A 48-year-old smoker presented with breathlessness and headache for 2 months. Imaging showed a heterogeneous enhancing lesion of 6 cm × 6 cm in right middle lobe of lung, smaller miliary nodules in bilateral lungs, multiple bilateral cerebral lesions, and a mass of 3 cm × 3 cm in the left thigh. Primary lung carcinoma with brain and thigh metastases was the clinical diagnosis. Fine-needle aspiration smears of the lung lesion showed cohesive fragments of large cells with a prominent traversing branching capillary network and discohesion at periphery resulting in a pseudo-papillary appearance. Tumor cells had fine granular to vacuolated cytoplasm, frayed borders, and prominent nucleoli. Trucut biopsy from the same showed a tumor arranged in nests composed of large polygonal cells, immunopositive for CD10. Possibility of metastatic renal cell carcinoma (RCC) was offered. Abdominal imaging was, however, normal. Core biopsy from thigh showed a similar tumor, immunonegative for epithelial markers, with cytoplasmic periodic-acid-schiff positive rhomboid crystals, clinching the final diagnosis of ASPS with lung and brain metastases. There is considerable morphological and immunohistochemical overlap between ASPS and RCC. Bare nuclei on air dried smears, binucleation, metachromatic basement membrane material are subtle pointers toward ASPS.

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Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 93%

“…Diagnosis of ASPS on cytology remains a challenge due to its rarity . Aspirates are moderately cellular, with a hemorrhagic background attributed to the rich vascularity.…”

Section: Discussionmentioning

confidence: 93%

See 2 more Smart Citations

Metastatic alveolar soft part sarcoma of the lung: Metastatic alveolar soft part sarcoma of the lung‐a morphologic pitfall on cytology and aberrant CD10 expression on histology

Nambirajan

Jain

Malik

et al. 2015

Diagnostic Cytopathology

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“…As cytology findings are overlapped with the cytological characteristics of other tumors, others tumor such as granular cell tumor (GCT), epithelioid sarcoma, paraganglioma, melanoma, clear cell sarcoma of the soft part, metastatic clear renal cell carcinoma (RCC), or adrenocortical carcinoma should also be considered in differential diagnosis. The characteristic cytomorphologic features for differential diagnosis have been discussed previously . The key points to distinguish these tumors are described here.…”

Section: Discussionmentioning

confidence: 88%

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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Alveolar Soft Part Sarcoma

Gambarotti

2014

Atlas of Musculoskeletal Tumors and Tumorlike Lesions

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Cytopathological diagnosis of alveolar soft part sarcoma, a rare soft tissue neoplasm

Abstract: The knowledge of cytological features may aid in diagnosing this rare tumour correctly on FNA smears, thus enabling correct patient management.

Cited by 11 publications

References 7 publications

Metastatic alveolar soft part sarcoma of the lung: Metastatic alveolar soft part sarcoma of the lung‐a morphologic pitfall on cytology and aberrant CD10 expression on histology

Metastatic alveolar soft part sarcoma of the lung: Metastatic alveolar soft part sarcoma of the lung‐a morphologic pitfall on cytology and aberrant CD10 expression on histology

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

Alveolar Soft Part Sarcoma

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