Humans ; Sarcoma, Alveolar Soft Part/secondary* ; Lung Neoplasms/pathology* ; Soft Tissue Neoplasms/pathology*

Perivascular epithelioid cell tumor (PEComa) is a very rare mesenchymal tumor with a distinctive morphology and immunophenotype. PEComas usually harbor TSC2 alterations, although TFE3 translocations, which occur in MiT family translocation renal cell carcinoma and alveolar soft part sarcoma, are also possible. We recently experienced a case of PEComa with TFE3 expression arising in the breast. An 18-year-old female patient presented with a right breast mass. Histologically, the tumor consisted of epithelioid cells with alveolar structure and showed a diffuse strong expression of HMB45 and TFE3. TSC2 was preserved. Melan A and smooth muscle actin were negative. To our knowledge, this is the first Korean case of PEComa of the breast that intriguingly presented with TFE3 expression.
Actins ; Adolescent ; Breast ; Carcinoma, Renal Cell ; Epithelioid Cells ; Female ; Humans ; MART-1 Antigen ; Muscle, Smooth ; Perivascular Epithelioid Cell Neoplasms ; Sarcoma, Alveolar Soft Part

The 20th National Assembly of Korea was called to order on April 13, 2016. The National Assembly is invested with the authority to play a significant role in influencing healthcare policy. There are many pending issues in healthcare policy that the Assembly needs to address in this term. The low fertility rate and population aging that Korea faces can exert a major impact on the healthcare system, as aging will lead to an expansion of non-communicable diseases, which can place a high burden on society. This requires strengthening the social security system, including National Health Insurance. A paradigm shift is necessary, in which healthcare providers are organized in the National Health Insurance using approaches such as the Accountable Care Organization (ACO) and Patient_Centered Medical Home (PCMH), and people are offered choices similar to the US Medicare Advantage Program. Additionally, it is essential that healthcare accessibility is enhanced by covering catastrophic medical costs and guaranteeing patient safety through quality improvement so that healthcare costs are kept within an affordable range. Therefore, the 20th National Assembly must overcome differences in ideology to improve the healthcare system with the aim of promoting national health.
Accountable Care Organizations ; Aging ; Birth Rate ; Delivery of Health Care ; Health Care Costs ; Health Personnel ; Health Policy* ; Humans ; Korea ; Medicare Part C ; National Health Programs ; Patient Safety ; Patient-Centered Care ; Quality Improvement ; Social Security

Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma, and frequently, metastases are found at diagnosis. In patients with metastatic or unresected ASPS, systemic treatment is extremely limited, because conventional chemotherapeutic agents have not been effective in most cases. A novel agent inhibiting angiogenesis, pazopanib, has been proven to be effective for metastatic soft tissue sarcoma in a second-line setting. However, the efficacy of pazopanib in ASPS has not yet been reported. A 22-year-old man presented with right calf ASPS and multiple lung metastases. Pazopanib as a second-line treatment showed significant tumor response. To the best of our knowledge, this is the first report of the effectiveness of pazopanib in ASPS.
Diagnosis ; Humans ; Lung ; Neoplasm Metastasis ; Sarcoma ; Sarcoma, Alveolar Soft Part* ; Viperidae ; Young Adult

OBJECTIVETo study the clinicopathologic features and differential diagnosis of alveolar soft part sarcoma (ASPS).

METHODSThe clinical data and pathologic features of 48 cases of ASPS were evaluated. Immunohistochemical study, PAS staining and fluorescence in-situ hybridization (FISH) were carried out in selected examples. Relevant literature was reviewed.

RESULTSAmongst the 48 cases studied, there were 17 males and 31 females, with male-to-female ratio of 1.0∶1.8. The age of patients ranged from 2 to 60 years (median=26 years). The tumor was most commonly located in deep soft tissue, especially that of lower extremities. Histologically, the tumor cells were arranged in alveolar or solid patterns and separated by sinusoidal vessels. They were large and contained abundant eosinophilic granules or crystals in cytoplasm. The nuclei were round to polygonal and vesicular, often with prominent nucleoli. Intravascular tumor extension was common. Some cases showed necrosis, hemorrhage and cystic changes. Immunohistochemical study showed that the tumor cells were positive for TFE3 (100%, 33/33). FISH assay was carried out in 4 cases and all of them had TFE3-ASPL gene fusion.

CONCLUSIONSASPS is a rare malignant neoplasm, often occurs in young patients. TFE3 is a useful immunohistochemical marker for diagnosis. The diagnosis is further confirmed by other markers.

Adolescent ; Adult ; Basic Helix-Loop-Helix Leucine Zipper Transcription Factors ; genetics ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Gene Fusion ; Humans ; In Situ Hybridization, Fluorescence ; Male ; Middle Aged ; Oncogene Proteins, Fusion ; genetics ; Sarcoma, Alveolar Soft Part ; diagnosis ; pathology ; Young Adult

PURPOSE: To assess real-world treatment patterns, health care utilization, costs, and survival among Medicare enrollees with locally advanced/unresectable or metastatic gastric cancer receiving standard first-line chemotherapy. MATERIALS AND METHODS: This was a retrospective analysis of the Surveillance, Epidemiology, and End Results-Medicare linked database (2000~2009). The inclusion criteria were as follows: (1) first diagnosed with locally advanced/unresectable or metastatic gastric cancer between July 1, 2000 and December 31, 2007 (first diagnosis defined the index date); (2) > or =65 years of age at index; (3) continuously enrolled in Medicare Part A and B from 6 months before index through the end of follow-up, defined by death or the database end date (December 31, 2009), whichever occurred first; and (4) received first-line treatment with fluoropyrimidine and/or a platinum chemo-therapy agent. RESULTS: In total, 2,583 patients met the inclusion criteria. The mean age at index was 74.8+/-6.0 years. Over 90% of patients died during follow-up, with a median survival of 361 days for the overall post-index period and 167 days for the period after the completion of first-line chemotherapy. The mean total gastric cancer-related cost per patient over the entire post-index follow-up period was United States dollar (USD) 70,808+/-56,620. Following the completion of first-line chemotherapy, patients receiving further cancer-directed treatment had USD 25,216 additional disease-related costs versus patients receiving supportive care only (P<0.001). CONCLUSIONS: The economic burden of advanced gastric cancer is substantial. Extrapolating based on published incidence estimates and staging distributions, the estimated total disease-related lifetime cost to Medicare for the roughly 22,200 patients expected to be diagnosed with this disease in 2014 approaches USD 300 millions.
Aged* ; Delivery of Health Care ; Diagnosis ; Drug Therapy ; Epidemiology ; Follow-Up Studies ; Humans ; Incidence ; Medicare ; Medicare Part A ; Platinum ; Retrospective Studies ; Stomach Neoplasms* ; United States

Head ; pathology ; Head and Neck Neoplasms ; diagnosis ; Humans ; Neck ; pathology ; Sarcoma, Alveolar Soft Part ; diagnosis

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcomas, this sarcoma occurs rarely in the larynx. Herein, we describe an unusual case of ASPS occurring in the larynx. The patient was a 46-year-old woman who presented with a more than 2-year history of right pyriform fossa mass and progressive hoarseness for three months. The endoscopic examination of the larynx revealed a mass in the right pyriform fossa with smooth surface and well-defined margin. Computed tomography demonstrated a heterogeneous tumor in the right pyriform fossa, and involving the paralaryngeal space, measuring approximately 2.1 cm x 1.7 cm x 2.6 cm, which was resected via lateral neck approach. Histopathology and immunohistochemistry revealed an ASPS. To ours knowledge, this is the first reported case of ASPS arising in pyriform fossa.
Female ; Hoarseness ; Humans ; Immunohistochemistry ; Larynx ; pathology ; Middle Aged ; Sarcoma, Alveolar Soft Part ; pathology ; Tomography, X-Ray Computed

Alveolar soft part sarcoma (ASPS) is an extremely rare malignant soft tissue sarcoma primarily affecting young patients. It usually occurs in the lower extremities, although it can occur in soft tissue anywhere in the body. However, to our knowledge, there has been no case of primary ASPS originating from the kidney in the literature. We herein present the imaging and clinical features of an ASPS which occurred in a 16-year-old male presented as a palpable mass in the left side of the abdomen.
Adolescent ; Biopsy ; Diagnostic Imaging/methods ; Humans ; Kidney/pathology ; Kidney Neoplasms/*diagnosis/pathology ; Male ; Rare Diseases/*diagnosis/pathology ; Sarcoma, Alveolar Soft Part/*diagnosis/pathology

Alveolar soft part sarcoma (ASPS) of the uterine cervix is a rare malignancy, and 21 cases have been reported the literature from every language (including our case). Herein, we describe a 17-yearold female patient who presented with active vaginal bleeding. Pelvic examination revealed a 1.6 x1.0x0.5-cm-sized soft mass protruding from the uterine cervix. The final pathological diagnosis was ASPS of the uterine cervix. Immunohistochemically, tumor cells were strongly nuclear positive for transcription factor E3. The patient remained disease free for 24 months without adjuvant therapy. The prognosis of ASPS in the cervix is considerably better than that of ASPS in soft tissues due to early clinical detection, small size, and resectability. ASPS should be considered in the differential diagnosis of an unusual epithelioid neoplasm showing organoid appearance with mild cytologic atypia and no/rare mitotic figures, particularly in young women. Pathologists should be aware of those unusual locations where ASPS may originate.
Cervix Uteri* ; Diagnosis ; Diagnosis, Differential ; Female ; Gynecological Examination ; Humans ; Immunohistochemistry ; Organoids ; Prognosis ; Sarcoma, Alveolar Soft Part* ; Transcription Factors ; Uterine Hemorrhage ; Viperidae