Lipoblastic Nerve Sheath Tumour

Definition

This is a rare nerve sheath tumour containing mature adipocytes and signet-ring lipoblasts

Histopathology

The tumour shows the morphology of a Schwannoma or neurofibroma. Mature adipocytes and signet-ring cell lipoblasts are present in a random distribution.

Immunohistochemistry

	S-100	5/51
	Vimentin	5/51
	CD34	0/51
	CD56	0/51
	SMA	0/51
	Desmin	0/51
	EMA	0/51
	Cytokeratin	0/51
	bcl-2	0/51

Ultrastructure

The one case in which the lipoblasts could be identified at an ultrastructural level confirmed the present of lipid-laden vacuoles.

Differential diagnosis of tumours showing a signet ring cell morphology

Mucinous signet-ring cell adenocarcinoma
- gastric signet ring cell carcinoma
- lobular carcinoma of breast
Liposarcoma
Malignant melanoma
Gastrointestinal stromal tumour
Meningioma
Chondrosarcoma
Desmoplastic small round cell tumour
Uterine lipoleiomyoma

References

1 Plaza JA, Wakely PE, Jr.,Suster S. Lipoblastic nerve sheath tumors: report of a distinctive variant of neural soft tissue neoplasm with adipocytic differentiation. Am J Surg Pathol 2006; 30:337-44

This page last revised 10.4.2006.