UNTREATEDHYPERNEPHROMAOF THIRTYITHREEYEARS

MARK HOROWITZ, M.D. * HARRY W. HERR, M.D. VICTOR REUTER, M.D.

From the Urology Service, Department of Surgery, and Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York

ABSTRACT-The literature cites long-term (>lO years) survivals after surgi- cal treatment for renal cell carcinoma compared with few untreated cases with similar survival. We report the longest survival between time of diagnosis and documented distant metastasesfrom an untreated renal cell cancer.

Untreated renal cell cancer is regarded as a le- thal disease since five-year survival is 2 per- cent.’ Ljungberg et a1.2 reported a case of renal cell carcinoma in a solitary kidney treated by nephrectomy thirty-one years after verification of its histopathology. We report a patient sur- viving thirty-three years with a documented untreated papillary renal cell cancer.

Case Report In 1957, a forty-nine-year-old white man

presented with sharp epigastric pain radiating to the left flank. There was no history of trauma. Intravenous pyelogram demonstrated a mass in the left retroperitoneum. The patient underwent exploration, evacuation of hema- toma, and left kidney biopsy. Histology was in- terpreted as a papillary adenoma (Fig. l), and the patient received no further treatment.

The patient was well until 1990, when at age eighty-three he complained of weight loss and a palpable mass on left side of neck. Biopsy speci- men of a supraclavicular lymph node showed papillary carcinoma (Fig. 2). A CT scan re- vealed a large mass replacing the left kidney. The paraffin-embedded tissues from the origi- nal kidney biopsy in 1957 were retrieved and reviewed. The specimen showed a small papil- lary carcinoma indistinguishable from the

‘State University of New York Health Science Center at Brooklyn, New York.

278

FIGURE 1. Papillary adenocarcinoma of kidney (hematoxylin and eosin, original magnification x 60).

FIGURE 2. Papillary adenocarcinoma of supra- clavicular lymph node (hematoxylin and eosin, orig- inal magnification x 180).

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metastasis seen in the cervical lymph node. Pa- tient is currently being followed expectantly.

Comment

This case demonstrates the difficulty in dis- tinguishing the biology of renal cortical adenoma from that of renal cell carcinoma on the basis of histologic appearance. In 1938, Bell3 studied a series of renal “adenoma-car- cinemas.” He showed that 35 percent, 72 per- cent, and 85 percent of tumors 3 cm to 6 cm, 6 cm to 10 cm, and more than 10 cm, respec- tively, demonstrated later metastases. He con- cluded that “the small tumors are early stages .of the large growths and that no certain distinc- tions can be made between adenoma and car- cinoma.” He classified those nodules less than 3 cm as adenomas and those larger as carcino- mas. The difficulty with accepting this working definition of adenoma and carcinoma is that the literature contains reports of metastases from tumors less than 3 cm, and tumors larger than 10 cm exhibiting relatively benign behav- ior. There are no gross, microscopic, ultrastruc-

tural, or histochemical features that reliably al- low distinction between so-called cortical adenomas and renal adenocarcinomas.

The survival of our untreated patient is the longest reported in the literature. The “typical” renal carcinoma shows a tubular, acinar, al- veolar, or papillary histologic pattern. These patterns convey little, if any, prognostic infor- mation in adults. Despite the lethal nature of most untreated renal cell cancers, this urologic neoplasm more often than other tumors proves to be enigmatic, capricious, and the “exception to the rule.”

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(DR. HERR)

References

1. Riches E: The natural history of renal tumors, in: l’hmors of the Kidney and Ureter, Baltimore, Williams and Wilkins Co., 1964, pp 124-134.

2. Ljungberg B, et al: Renal cell carcinoma in a solitary kid- ney: late nephrectomy after 35 years and analysis of tumor deoxy- ribonucleic acid content, J Urol 139: 350 (1987).

3. Bell ET: A classification of renal tumors with observation on the frequency of the various types, J Uro139: 238 (1938).

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