Journal of Pathology and Translational Medicine

Cytologic Findings of Alveolar Soft Part Sarcoma Presenting with Multiple Pulmonary Masses: A Case Report with Review of Literature.: Na Rae Kim, Jae Y Ro, Eun Kyung Cho, Mi Jin Kim, Jungsuk An, Seung Yeon Ha; Korean J Pathol. 2011;45(1):119-124.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.1.119

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Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor of uncertain origin, and it has a strong propensity for metastasis to the lungs, bones and brain. We report upon an unusual case of ASPS, presenting as multiple lung nodules with no other detectable primary site, in a 44-year-old man. A fine needle aspiration of the nodules yielded scattered, discohesive cells, each containing an eccentrically displaced nucleus and prominent nucleolus, on a granular background. Tumor cells with numerous bared nuclei, and occasional sheets of epithelioid cells were also found. Under the cytological diagnosis of an unclassified epithelioid malignant tumor, resection of the lung nodules was performed. The histologic findings were consistent with ASPS, showing positive TFE3-nuclear immunoreactivity. There is limited literature concerning cytological findings associated with pulmonary ASPS: especially in cases where the primary site is unknown. Here, we present a cytological review of pulmonary ASPS, investigating the significance of TFE3 staining in the diagnosis of ASPS.

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Alveolar soft part sarcoma: A case report with emphasis on some unusual cytological features
Neelam Sood, Minakshi Gulia
Diagnostic Cytopathology.2018; 46(2): 170. CrossRef

Array-comparative Genomic Hybridization Analysis of Alveolar Soft Part Sarcoma.: Jeung Il Kim, Kyung Un Choi, Hyun Jeong Kang, Dong Hoon Shin, In Sook Lee, Tae Yong Moon, Won Taek Kim; Korean J Pathol. 2009;43(3):231-237.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.3.231

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Abstract PDF: BACKGROUND
Alveolar soft part sarcomas (ASPSs) are rare, histologically distinctive soft tissue sarcomas of unknown origin. Although ASPSs are characterized by a specific alteration, der(17)t(X;17)(p11;q25), the entire spectrum of genetic events underlying the pathogenesis of ASPS is unclear. Using array-based comparative genomic hybridization (array-CGH), we examined the DNA copy number changes in ASPS.
METHODS
Array-CGH, composed of 4,030 clones, was performed in two samples of fresh frozen tumor tissues from a 29-year-old male and a 16-year-old female.
RESULTS
We identified 16 commonly altered chromosomal regions involving 25 genes. Eleven altered regions were located on chromosome Xp (Xp22.33, Xp22.11, Xp11.3, Xp11.3-Xp11.23, Xp22.2, Xp22.12, Xp22.31, Xp22.32, Xp21.1, Xp21.3, and Xp11.4). Additional regions with an increased copy number were observed at 1q25.1, 7q35, 12p12.1, and 17p11.2. Loss was found in only one region of chromosome 22q11.23. Several genes located within the amplified region of Xp included GYG2, ARSD, ARSE, ARSH, UBE1, USP11, PCTK1, ARAF, SYN1, TIMP1, XK, PDK3, PCYT1B, PHEX, ARX, RPS6KA3, TMSB4X, TMEM27, BMX, and KAL1.
CONCLUSIONS
This was the first application report of genome-wide copy number changes by BAC array-CGH in ASPSs. Our study showed unique genomic regions and new candidate genes that suggest a neural origin and are associated with tumor pathogenesis in ASPSs.

Alveolar Soft Part Sarcoma of the Uterine Cervix: A case report.: Ghee Young Kwon, Yeon Lim Suh; Korean J Pathol. 1996;30(10):933-938.

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Abstract PDF: We report a case of alveolar soft part sarcoma(ASPS) of the uterine cervix in a 57-year-old female. She was presented with vaginal bleeding and underwent total hysterectomy. A 1.1cm-sized, polypoid mass was found at the uterine cervix. Microscopically, it had shown an alveolar arrangement of tumor cells and characteristic Periodic acid-Schiff-positive, diastase-resistant, intracytoplasmic granules. Distinct cytoplasmic crystals were found on the ultrastructural examination and tumor cells showed immunoreactivity for smooth muscle actin, myoglobin and neuron-specific enolase, while nonreactive for desmin, vimentin and S-100 protein. Myogenic origin can be suggested with these immunohistochemical results.

Metastatic Alveolar Soft Part Sarcoma Presented with a Dura Mass: A Case Report.: Sun Young Kwon, Hyun Chang Joo, Kun Young Kwon, Sang Sook Lee, Chul Ho Sohn, Man Bin Yim, Sang Pyo Kim; Korean J Pathol. 2001;35(2):180-183.

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Abstract PDF: Alveolar soft part sarcoma, accounting for less than 1% of all soft tissue sarcomas, is known for late metastases to the lung, bone and brain. We have examined a case of metastatic alveolar soft part sarcoma to the brain in a 34-year-old woman. Computed tomography of the brain revealed a well enhancing dura based mass of the left temporal area. The patient had a previous history of surgery for alveolar soft part sarcoma from the right thigh at the age of 24 years. She underwent total removal of the dural mass under the clinical impression of the meningioma. Grossly, a well-circumscribed, pale tan and solid mass was 3.4 x 3.2 x 2.4 cm and easily detached from the attached dura. Histologically, the tumor showed typical features of alveolar soft part sarcoma. Immunohistochemically, tumor cells were positive for muscle related antibodies. Ultrastructural examination showed rod-shaped membrane bound crystals and electron-dense granules in the cytoplasm of tumor cells.

Alveolar Soft Part Sarcoma of the Lung Diagnosed by Fine Needle Aspiration Cytology: A Case Report .: Dae Su Kim, Young Lyun Oh, Young Hyeh Ko; Korean J Cytopathol. 1998;9(2):187-192.

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Abstract PDF: Alveolar soft part sarcoma(ASPS) is a rare malignant neoplasm with a distinct clinicopathologic entity of which fine needle aspiration(FNA) cytologic findings have been described in only a few reports. Although patients usually present with an isolated soft-tissue mass in the extremity, metastasis can occur in about 13 % of total cases and the most frequent metastatic site is the lung. We have recently experienced a FNA cytologic case of ASPS in the lung. A 23-year-old female patient was admitted to this hospital due to 2-month-history of cough. She had been good in health before the visit. Chest computed tomography revealed multiple, variable sized, bilateral pulmonary nodules. Physical examination and other staging work up revealed no other lesions except for pulmonary nodules. A percutaneous transthoracic FNA was performed from the pulmonary nodules. The smear was cellular and most cells were arranged singly. In addition, a few clusters lined by thin-walled vasculature with a pseudoalveolar pattern were present. Some of the tumor cells were large and polygonal to oval with abundant granular or vacuolated cytoplasm. Most cells were naked nuclei showing finely granular chromatin pattern with prominent central nucleoli.

Fine Needle Aspiration Cytology of Alveolar Soft Part Sarcoma: A Case Report .: Hye Seung Han, In Seo Park, Jee Young Han, Joon Mee Kim, Young Bae Kim, Tae Sook Hwang, Young Chae Chu; Korean J Cytopathol. 2000;11(2):115-119.

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Abstract PDF: Alveolar soft part sarcoma is a rare soft tissue tumor. Few cases of fine needle aspiration cytology have been reported in the literature. We experienced a case of recurrent alveolar soft part sarcoma of the right thigh diagnosed by fine needle aspiration cytology in a 47-year-old man. Cytologic findings showed single cells and clusters associated with thin walled vasculature in a distinct pseudo-alveolar pattern. The tumor cells exhibited round or ovoid abundant granular cytoplasm and large pleomorphic nuclei with prominent central nucleoli.

Fine Needle Aspiration Cytology of Alveolar Soft Part Sarcoma: A Case Report.: Joon Hyuk Choi, Young Ran Shim, Duk Seop Shin, Kil Ho Cho; Korean J Cytopathol. 2006;17(1):69-74.

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Abstract PDF: Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma, which occurs predominantly in adolescents and young adults. The cytological characteristics of this condition have been described only rarely in the literature. Here, we report a case of alveolar soft part sarcoma. A 28-year-old man presented with a mass in his right buttock, which had persisted for three years. The mass was subjected to a fine needle aspiration cytology (FNAC). The smears were cellular. The observed tumor cells were round or polygonal, and exhibited vesicular nuclei with prominent nucleoli and finely granular cytoplasm. Naked nuclei were frequently detected. Tumor cells were arranged singularly, but occasionally in a pseudoalveolar pattern.

Bilateral Mammary Metastasis of Alveolar Soft Part Sarcoma: A Case Report.: Soo Youn Cho, Ho Chang Lee, Chong Jai Kim, Min Suk Kim, Sun Hoo Park, Eui Keun Ham, In Ae Park; Korean J Pathol. 2003;37(5):365-368.

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Abstract PDF: An alveolar soft part sarcoma(ASPS) is a rare malignant soft tissue tumor, which metastasizes to the lung, bone and brain. Recently, we encountered an unusual case of a metastatic ASPS to the bilateral breasts in a 27-year-old woman. She had undergone surgery for an ASPS in her right thigh two years ago, which metastasized to the breast on three occasions, 15 months, 20 months and two years after surgery.

Intracerebral Metastasis of Alveolar Soft Part Sarcoma: A case report and study on its histogenesis.: Young Chae Chu, Joon Mee Kim, Young Bae Kim, Tae Sook Hwang; Korean J Pathol. 1992;26(5):510-516.

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Abstract PDF: We describe a case metastatic alveolar soft part sarcoma of the brain in a 20-year old man. Alveolar soft part sarcoma is slowly growing tumor which almost shows conspicuous vascular invasion and have a high incidence of blood-borne metastasis. The principal metastatic sites are the lungs, followed by the skeleton and brain. Cerebral metastases may be the first manifestation of the disease and are more common with alveolar soft part sarcoma than with any other type of soft tissue sarcoma. The light-and electron-microscopic and immunohistochemical findings are described. Periodic acid-Schiff-positive, diastase resistant, intracytoplasmic crystals, pathognomonic for alveolar soft part sarcoma, are present. The cells expressed immunoreactivity for vimentin, desmin, neuron-specific enolase, S-100 protein and renin. The results of our own immunohistochemical examinations is suggestive of myoendocrine origin.

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