Abstract

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Adenocarcinoma of the rete testis is a rare tumour with a varied presentation. A total of 44 cases have been reported [1]. Of these testicular tumours, primary nongerm cell tumours account for a minority of cases. Among this, adenocarcinoma originating in the rete testis is still rarer. It is a highly malignant tumour with poor prognosis. It is generally diagnosed clinically as a solid or cystic swelling. However, confirmation is made only on histological diagnosis.

Introduction

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It is a highly malignant tumour with poor prognosis. It is generally diagnosed clinically as a solid or cystic swelling. However, confirmation is made only on histological diagnosis. The diagnostic criteria of Nochomovitz for adenocarcinoma rete testis include:-
(1) Tumour origin around the hilum
(2) No involvement of the tunica
(3) Demonstration of transition from normal to tumour epithelium
(4) No evidence of teratoma or any other primary tumour elsewhere in the body [2, 3].

Case Report(s)

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A 75 year old man presented with painless swelling in the left side of the scrotum of 20 days duration. There was history of blunt trauma during fall. It was hard, proximal to left testis of 5X5 cm in sized, non fluctuant non transilluminant. Overlying skin was free.
A diagnosis of chronic haematocele was made. US abdomen was normal. Blood parameters were normal. Patient was taken up for surgery and left sided scrotum was explored. Blood mixed fluid with necrotic material was found. Left sided orchidectomy was done. Drain was placed and wound was closed.
Histology revealed features of adenocarcinoma of rete testis.
Patient was advised further treatment which he refused. After one month patient presented multiple cutaneous swellings which were hard, nontender over forearm and abdominal wall.
Biopsy of forearm cutaneous secondaries was done – histology confirmed cutaneous adenocarcinoma secondaries.

Discussion

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Carcinoma of the rete testis is a rare tumour, occurring most commonly in the fourth and fifth decades of life. However, patients may range from 20 to 80 years of age. A total of 44 cases have been reported, three of the ,youngest cases (ages 20 yrs, 21 yrs, and 34 yrs) had a prior history of undescended testis[4-6]. The carcinoma can affect either side, and occasionally may be bilateral. All races are equally affected. Carcinoma of the rete testis has usual association with hydrocele, epididymitis, inguinal hernia and occasionally, with undescended testis.
The tumour is confirmed only on histological diagnosis, which is governed by the Nochomovitz criteria. This includes:
(1) Location of the tumour around the hilum;
(2) No involvement of the tunica;
(3) Histological evidence of tumour origin from the rete testis and demonstration of transition from normal to tumour epithelium; and
(4) No evidence of teratoma or any other primary tumour elsewhere in the body [2, 3].
On gross examination, the tumour may show a solid, nodular or cystic surface. Microscopically, there are three patterns. The papillary pattern, which is commonest, represents glomeruli with numerous microvilli projecting into cystic spaces. The rete epithelium shows deep narrow nuclear invaginations, apparent nuclear lobulations, and small pleomorphic electron-dense membrane-bound granules in the basal cytoplasm, and lipid droplets in the apical cytoplasm and bulbous cytoplasmic projections along the apical surface cells. The glandular pattern consists of moderately- to poorly-differentiated glands. Solid pattern shows irregular arrangement of cells along with greater cellular and nuclear pleomorphism.
The differential diagnoses include malignant mesothelioma of the tunica, serous Mullerian tumours of the testis, and Sertoli cell tumours [7]. Adenocarcinoma of the rete testis is an aggressive malignant neoplasm.

The treatment is primarily surgery (high inguinal orchidectomy)[8]. Radical orchidectomy, retroperitoneal lymph node dissection, radiotherapy and chemotherapy have been tried alone or in combination, with no fruitful results.

The stage at diagnosis or any form of therapy has no influence on final outcome, and prognosis remains poor. Patients seldom survive longer than one year, although survival rates up to five years without metastasis have been reported.

Careful follow-up is advisable.

References

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1.Menon PK, Vasudevarao, Sabhiki A, et al. (2002) A case of carcinoma rete testis. Indian J Cancer; 39:106-11.
2.Nochomovitz LE, Orenstein JM. (1984) Adenocarcinoma of the rete testis. Case report, ultrastructural observations, and clinicopathologic correlates. Am J Surg Pathol; 8:625-34.
3.Young RH, Scully RE.( 1986) Miscellaneous neoplasm and nonneoplastic lesions. In: Talerman A, Roth LM, eds. Pathology of the testis and its adenexa. New York: Churchill Livingstone: 93-129.
4.Dundon C. (1952) Carcinoma of the rete testis occurring ten years after orchidopexy. Br J Urol; 24:58-63.
5.Shillitoe AJ. (1952) Carcinoma of the rete testis. J Pathol Bacteriol; 64:650-1.
6.Jacobetlis U, Ricco R, Ruotolo G. (1981) Adenocarcinoma of the rete testis 21 years after orchiopexy: case report and review of literature. J Urol;125:429-31,
7.Young RH, Scully RE. (1986) Testicular and paratesticular tumors and tumor-like lesions of ovarian common epithelial and müllerian types. A report of four cases and review of the literature. Am J Clin Pathol; 86:146-52.
8.Fukunaga M, Aizawa S, Furusato M, Akasaka Y, Machida T. (1982) Papillary adenocarcinoma of the rete testis: a case report. Cancer; 50:134-8.

Source(s) of Funding

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government hospital

Competing Interests

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none

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