Cervix
Mesenchymal / mixed epithelial & mesenchymal tumors
Alveolar soft part sarcoma
Author: Branko Perunovic, D.M.
Last author update: 1 May 2007
Last staff update: 12 April 2024 (update in progress)
Copyright: 2007-2024, PathologyOutlines.com, Inc.
PubMed Search: Alveolar soft parts sarcoma cervix
Table of Contents
Definition / general | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Perunovic B. Alveolar soft part sarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cervixalveolarsoftpartssarcoma.html. Accessed April 19th, 2024.
Definition / general
- Very rare
- Usually ages 30 to 40 years
- Associated with abnormal uterine bleeding
- Patients often do well, but may die of metastatic disease
Case reports
- 8 year old girl with alveolar soft part sarcoma of the uterine cervix (Acta Pathol Jpn 1993;43:204)
- 35 year old woman with alveolar soft part sarcoma of the uterine cervix (Arch Pathol Lab Med 1989;113:1179)
- 39 year old woman whose tumor demonstrated TFE3+ (Int J Gynecol Pathol 2005;24:131)
Microscopic (histologic) description
- Well circumscribed tumor with loss of central cohesion causing a pseudoalveolar pattern
- Nests are separated by thin - walled, sinusoidal vascular spaces
- Cells are large with distinct cell borders, resembling gemistocytic astrocytes
- Contain PAS+ diastase resistant intracytoplasmic crystals
- Small nuclei with prominent nucleoli
Microscopic (histologic) images
Images hosted on other servers:
Nests of tumor cells with PAS+ crystals
Positive stains
Molecular / cytogenetics description
- t(X;17)(p11;q25) - TFE3 - ASPL fusion transcript
Electron microscopy description
- Rhomboid, rod - shaped or spicular crystals with a regular lattice pattern and electron dense secretory granules
- Crystals consist of filaments 6 nm in diameter, arranged in parallel arrays with periodicity of 10 nm
- Basal lamina surrounds groups of tumor cells with prominent mitochondria, glycogen and lipid
Differential diagnosis
- Clear cell carcinoma: often papillary or cystic with hobnail cells, cytoplasm is more clear, may have focal PAS+ areas in cytoplasm, but diastase sensitive
- Metastatic renal cell carcinoma
- Paraganglia: solid nests of neuroendocrine cells surrounded by S100+ sustentacular cells; negative for muscle markers, no PAS+ diastase resistant crystals
Additional references
