Sustentaculoma

Definition

There is a single case report of an adrenal tumour, thought to be composed of sustentacular cells1.

Epidemiology

The case report is of a 54 year old woman.

Macroscopic appearances

The tumour is well demarcated but not encapsulated.

Histopathology

The tumour is composed of spindle cells with eosinophilic cytoplasm which form ill-defined nests. The tumour lacks necrosis, atypia or mitotic activity.

Immunohistochemistry

	Vimentin	1/11
	S-100	1/11
	CD56	1/11
	GFAP	0/11
	Chromogranin	0/11
	Synaptophysin	0/11
	cytokeratin	0/11
	EMA	0/11
	inhibin	0/11
	HMB-45	0/11
	Melan-A	0/11
	CD1a	0/11
	CD3	0/1: background lymphocytes were positive1
	CD20	0/11
	CD21	0/11
	CD23	0/11
	CD35	0/11
	CD45	0/11
	CD68	0/11
	Ki-67	1.5% of cells1

Ultrastructure

The spindle cells show interdigitating cytoplasmic processes and lack a basement lamina.

Differential diagnosis

Phaeochromocytoma: positive for chromogranin and synaptophysin.
Schwannoma: shows Antoni A and B areas. Cells are surrounded by a basal lamina ultrastructurally.
Dendritic cell neoplasm: follicular dendritic cells tumours are positive for CD21 and CD35.
Extracranial meningioma: would be positive for EMA.
Melanoma: would probably be positive for Melan-A and HMB-45.

References

1 Lau SK, Romansky SG,Weiss LM Sustentaculoma: report of a case of a distinctive neoplasm of the adrenal medulla. Am J Surg Pathol 2006; 30:268-73

This page last revised 10.4.2006.