CASE DETAIL


General Pathology  »   ADRENAL GLANDS (XXIII)  »   Neoplasms (iii)

PHAEOCHROMOCYTOMA AND HIBERNOMA

Museum No: XXIII:iii:19   

Year: 1964

 

Click thumbnails below for enlargements


Clinical data:

The patient was a 42 year old white man, who had been treated for two years before admission for hypertension and progressive limitation of cardiac reserve, during which period he lost approximately 16kg in weight. He was admitted to Groote Schuur hospital in biventricular failure, and apparently had urinary vanillylmandelic acid (VMA) excretions of 10.4 and 25.4 mg/24 hours (i.e. grossly elevated). His autopsy confirmed the presence of hypertensive cardiovascular disease.

Macroscopy:

His left adrenal contained this brown tumour (shown here) containing several small irregular yellow areas and two small haemorrhagic foci. Overall the appearances were typical of a phaeochromocytoma, naked-eye. Continuous with the tumour and with the area of gland adjacent to the tumour, and originally extending posteriorly to the left kidney and completely covering the posterior aspect of the left kidney, is lobulated fatty tissue (originally dark brown). The lobules are connected by flimsy, loose connective tissue.(Interestingly there is a small amount of non-neoplastic fat present which, as often happens with prolonged fixation, has gone a patchy orange colour.)

Microscopy:

Microscopically appearing as foetal fat, the fatty mass is consistent with the diagnosis of a hibernoma.

Comments:

While there is no disputing the original commentary that surgery would likely have proved curative in this case, not many would currently agree with the corollary that “all patients with hypertension disease should be investigated for the presence of a phaeochromocytoma”. (See also commentary on specimen XXIII:iii:15.)


Hibernoma is an uncommon tumor of brown fat, and has been described only in a few case reports. There is a recognised association with phaeochromocytoma. This example has now lost the dark brown colour originally noted at autopsy, but does appear more “lobular and greasy”, as described in the AFIP series quoted below.
The quoted authors below assembled 170 cases from the records of the Soft Tissue Registry of the Armed Forces Institute of Pathology in the USA. Of the 170, 99 were men, and overall the mean age was 38 years (range 2 - 78y). The commonest site was the thigh (50), with the abdomen / retroperitoneum being the site in 10. The average size was 9.3cm in diameter (range 1 - 24cm). The tumours were microscopically “typical” in 140 cases, but three other variants were described as well. None of the 66 cases (39%), which had a follow-up for a mean of 7.7 years (range 6m - 28y), had a recurrence or metastasis, and no patient died of their disease.

 

References:

Furlong MA et al. The morphologic spectrum of hibernoma: A clinicopathologic study of 170 Cases. Am J Surg Pathol. 2001. 25:809-14.

English JT, Patel SK, Flanagan MJ. Association of phaeochromocytomas with brown fat tumours. Radiology 1973; 107:279-281