Biomaterial Database

Primary Thyroid Gland Alveolar Soft Part Sarcoma. 2020

Rumeal D Whaley, and Lester D R Thompson

Department of Pathology & Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor of unknown histogenesis generally characterized by the der(17)t(X;17)(p11.2;q25) translocation which results in the ASPSCR1-TFE3 gene fusion. Primary ASPS of the thyroid gland has not yet been reported. During oncology follow-up for breast cancer, a pulmonary nodule and thyroid gland mass were identified in a 71-year-old Korean male. Thyroid ultrasound showed a 5.7 cm left thyroid gland mass. After several fine needle aspirations, a thyroid gland lobectomy was performed after documenting only non-caseating granulomatous inflammation in a biopsy of the lung nodule. A 7.6 cm bulging nodular thyroid gland mass was identified, showing significant destructive invasion. Alveolar nests of large polygonal, eosinophilic, granular neoplastic cells were separated by vascularized stroma. Colloid was absent. Tumor necrosis and increased mitoses were identified. The neoplastic cells were positive with TFE3 and CD68, but negative with pancytokeratin, thyroglobulin, TTF-1, napsin-A, calcitonin, PAX8, CAIX, S100 protein, HMB45, SMA, and desmin. FISH confirmed a TFE3 gene rearrangement. The differential includes several primary thyroid gland epithelial neoplasms, paraganglioma, PEComa, melanoma, crystal storage disease, and metastatic carcinomas, especially Xp11 translocation renal cell carcinoma. The patient has refused additional therapy, but is alive without tumor identified (primary or metastatic).

UI	MeSH Term	Description	Entries
D008297	Male		Males
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D013964	Thyroid Neoplasms	Tumors or cancer of the THYROID GLAND.	Cancer of Thyroid,Thyroid Cancer,Cancer of the Thyroid,Neoplasms, Thyroid,Thyroid Adenoma,Thyroid Carcinoma,Adenoma, Thyroid,Adenomas, Thyroid,Cancer, Thyroid,Cancers, Thyroid,Carcinoma, Thyroid,Carcinomas, Thyroid,Neoplasm, Thyroid,Thyroid Adenomas,Thyroid Cancers,Thyroid Carcinomas,Thyroid Neoplasm
D015321	Gene Rearrangement	The ordered rearrangement of gene regions by DNA recombination such as that which occurs normally during development.	DNA Rearrangement,DNA Rearrangements,Gene Rearrangements,Rearrangement, DNA,Rearrangement, Gene,Rearrangements, DNA,Rearrangements, Gene
D016609	Neoplasms, Second Primary	Abnormal growths of tissue that follow a previous neoplasm but are not metastases of the latter. The second neoplasm may have the same or different histological type and can occur in the same or different organs as the previous neoplasm but in all cases arises from an independent oncogenic event. The development of the second neoplasm may or may not be related to the treatment for the previous neoplasm since genetic risk or predisposing factors may actually be the cause.	Neoplasms, Metachronous,Neoplasms, Metachronous Second Primary,Neoplasms, Therapy-Related,Neoplasms, Treatment-Related,Second Malignancy,Second Neoplasm,Second Primary Neoplasms,Therapy-Associated Neoplasms,Therapy-Related Cancer,Treatment-Associated Neoplasms,Treatment-Related Cancer,Cancer, Second Primary,Metachronous Neoplasms,Metachronous Second Primary Neoplasms,Neoplasms, Therapy-Associated,Neoplasms, Treatment-Associated,Second Cancer,Second Primary Neoplasms, Metachronous,Therapy-Associated Cancer,Therapy-Related Neoplasms,Treatment-Associated Cancer,Treatment-Related Neoplasms,Cancer, Second,Cancer, Therapy-Associated,Cancer, Therapy-Related,Cancer, Treatment-Associated,Cancer, Treatment-Related,Cancers, Second,Cancers, Second Primary,Cancers, Therapy-Associated,Cancers, Therapy-Related,Cancers, Treatment-Associated,Cancers, Treatment-Related,Malignancies, Second,Malignancy, Second,Metachronous Neoplasm,Neoplasm, Metachronous,Neoplasm, Second,Neoplasm, Second Primary,Neoplasm, Therapy-Associated,Neoplasm, Therapy-Related,Neoplasm, Treatment-Associated,Neoplasm, Treatment-Related,Neoplasms, Second,Neoplasms, Therapy Associated,Neoplasms, Therapy Related,Neoplasms, Treatment Associated,Neoplasms, Treatment Related,Second Cancers,Second Malignancies,Second Neoplasms,Second Primary Cancer,Second Primary Cancers,Second Primary Neoplasm,Therapy Associated Cancer,Therapy Associated Neoplasms,Therapy Related Cancer,Therapy Related Neoplasms,Therapy-Associated Cancers,Therapy-Associated Neoplasm,Therapy-Related Cancers,Therapy-Related Neoplasm,Treatment Associated Cancer,Treatment Associated Neoplasms,Treatment Related Cancer,Treatment Related Neoplasms,Treatment-Associated Cancers,Treatment-Associated Neoplasm,Treatment-Related Cancers,Treatment-Related Neoplasm
D051778	Basic Helix-Loop-Helix Leucine Zipper Transcription Factors	A family of transcription factors that contain regions rich in basic residues, LEUCINE ZIPPER domains, and HELIX-LOOP-HELIX MOTIFS.	BHLH-Zip Transcription Factors,BHLHLZ Transcription Factors,BHLH Zip Transcription Factors,Basic Helix Loop Helix Leucine Zipper Transcription Factors,Transcription Factors, BHLH-Zip,Transcription Factors, BHLHLZ
D018234	Sarcoma, Alveolar Soft Part	A variety of rare sarcoma having a reticulated fibrous stroma enclosing groups of sarcoma cells, which resemble epithelial cells and are enclosed in alveoli walled with connective tissue. It is a rare tumor, usually occurring between 15 and 35 years of age. It appears in the muscles of the extremities in adults and most commonly in the head and neck regions of children. Though slow-growing, it commonly metastasizes to the lungs, brain, bones, and lymph nodes. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1365)	Alveolar Soft Part Sarcoma,Alveolar Soft-Part Sarcoma
D018567	Breast Neoplasms, Male	Any neoplasms of the male breast. These occur infrequently in males in developed countries, the incidence being about 1% of that in females.	Male Breast Neoplasms,Neoplasms, Breast, Male,Breast Cancer, Male,Breast Carcinoma, Male,Breast Tumors, Male,Male Breast Cancer,Neoplasms, Male Breast,Tumors, Breast, Male,Breast Neoplasm, Male,Breast Tumor, Male,Cancer, Male Breast,Carcinoma, Male Breast,Male Breast Carcinoma,Male Breast Neoplasm,Male Breast Tumor,Male Breast Tumors,Neoplasm, Male Breast,Tumor, Male Breast,Tumors, Male Breast