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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

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A primary leiomyosarcoma of bone containing pseudoepithelial plexiform elements.

A Rigopoulou¹, M Vlychou, S J Ostlere

¹Department of Radiology, Nuffield Orthopaedic Centre, Oxford, OX3 7LD, UK.

Skeletal Radiology

|May 8, 2007

Summary

A rare uterine smooth muscle tumor variant, plexiform tumorlets, was found in a primary bone leiomyosarcoma. This is the first reported bone occurrence and only the second malignant case globally.

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Three-Dimensional Bone Extracellular Matrix Model for Osteosarcoma

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Published on: April 12, 2019

Area of Science:

Oncology
Pathology
Skeletal Tumors

Background:

Plexiform tumorlets are a rare, distinctive feature of smooth muscle tumors, predominantly observed in the uterus.
Malignant plexiform smooth muscle tumors are exceptionally rare, with limited documented cases.

Observation:

A primary leiomyosarcoma of the proximal humerus exhibited characteristic leiomyosarcoma features alongside plexiform tumorlets.
Tumor cells within the plexiform component and other cellular morphologies expressed smooth muscle actin, calponin, and cytokeratin.

Findings:

This case represents the first documented instance of a plexiform smooth muscle tumor originating in bone.
It is only the second reported malignant plexiform smooth muscle tumor and a rare extrauterine manifestation.
The tumor occurred in a male patient, adding to its unique characteristics.

Implications:

This finding expands the known anatomical sites for plexiform smooth muscle tumors.
Distinguishing this rare bone leiomyosarcoma variant from metastatic carcinomas with epithelial elements is crucial for accurate diagnosis and treatment.
Further research into the pathogenesis and behavior of extrauterine plexiform smooth muscle tumors is warranted.