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Q1: What is the probable diagnosis of this condition?
The diagnosis was malignant transformation of the cryptorchid testis. A mass in the abdomen in a sexually active man with a cryptorchid testis strongly points towards the diagnosis of malignancy in the abnormal testis. The characteristic features of malignancy are a homogenous tumour with grey-white colour on cut section and vascular markings over the tumour. The histological features of monotonous tumour cells in lobules with clear cytoplasm and a centrally located nucleus clinches the diagnosis of seminoma; this is the commonest histology in a cryptorchid testis. Moreover the associated computed tomogram evidence of lymphadenopathy supports the above diagnosis.
Q2: Which is the best treatment in this situation?
In case of locally advanced seminoma with surgical spill, associated mesenteric lymphadenopathy and visceral infiltration, the best initial treatment is combination chemotherapy. The preferred combination chemotherapy regimens are bleomycin, etoposide, and cis-platin (BEP) or cis-platin, vinblastine, and bleomycin. In case of residual disease after a full course of chemotherapy, the above area is supplemented with localised radiotherapy.
Q3: What is the chance of second cancer in the opposite testis?
The chance of developing malignancy on the opposite testis is extremely high among adults. Even after reposition of the cryptorchid testis, there is very minimal decrease in the incidence of a second malignancy. The usual incidence of developing malignancy in the opposite testis is around 20%. Hence where stringent follow up is a problem some surgeons prefer to advocate prophylactic orchiectomy of the normal looking testis. The testis histologically demonstrates the features of hypoplastic or atrophic seminiferous tubules, thus supports infertility in cryptorchidism.
Due to the risk of second malignancy in the opposite testis, an elective orchiectomy was done and on pathological evaluation found to have features of hypoplastic seminiferous tubules. He was then treated with the standard BEP chemotherapy regimen (that is, bleomycin 30 units intravenously on days 1, 8, and 15; etoposide 120 mg/m2 on days 1–3, and cis-platin 100 mg/m2 (total) as an intravenous infusion over three days. After three courses of the BEP regimen, the patient developed deterioration of creatinine clearance for which his chemotherapy was changed to the vincristine, actinomycin-D, and cyclophosphamide (VAC) regimen. Three more courses of the VAC (vincristine 1.4 mg/m2 on days 1 and 8, actinomycin-D 500 μg on days 1–5, and cyclophosphamide 500 mg/m2 on day 1 repeated every four weeks) regimen were given. The patient tolerated the above regimen well and was found to have clinical and radiological evidence of resolution of the disease nine months after treatment.
Cryptorchidism is a known cause of testicular tumour. Genetic and hormonal influences affect the migration of testis from the abdomen to the scrotal sac through the inguinal canal. Moreover the availability of free oestrogen in the first trimester of pregnancy also influences the migration of the testis.1 A high intra-abdominal temperature has been incriminated as the cause of carcinogenesis in the testis.
Cryptorchid testis is an aetiological factor for testicular tumour especially if it is abdominal
Seminoma is the commonest histology in cryptorchid related malignancy
Sometimes an undescended testis can manifest as a massive tumour with visceral invasion of acute abdomen
Multiagent chemotherapy is very useful in abdominal testes complicated by seminoma
A suspicion of intra-abdominal testicular tumour should be made while investigating a case of an infertile man with an abdominal mass
The risk of developing testicular tumour in cryptorchid testis is 10–40 times higher than the normal testis. About 1%–5% of boys with undescended testes develop germ cell tumours.2 The position of the undescended testis is related to the likelihood of carcinogenesis with the intra-abdominal location having the highest risk for malignancy.3
The normal testis of the cryptorchid patient carries a higher risk for malignant transformation. The cause of carcinogenesis is still an enigma. About 5%–20% of patients with a cryptorchid testis develop a testicular tumour on the opposite, normally descended, testis.4 The incidence of malignancy is about 25% if the opposite testis has features of testicular atrophy.2 5
The histological transformation is a gradual process, which takes a long time to develop. Persistence of abnormal testis is associated with other structural abnormalities. There may be a decrease in the spermatogenesis, Leidig cell abnormality, and delay in the development of the Sertoli cells in the testis.6 7 In our case too, there was evidence of sterility due to the testicular malfunction. Approximately all the malignancies in cryptorchid testis are seminomas.8 But the spermatocytic seminoma9and teratomas10 can very rarely occur in cryptorchid testis.
Painless enlargement of the testis, or abdominal mass, is the common mode of presentation in a cryptorchid testis.11 But very rarely, an abdominal testicular tumour can cause acute abdomen, massive abdominal mass, pain, and haematuria because of adjacent visceral infiltration.12 13 There are very few reports of malignant testicular tumour in cryptorchid testis in the pelvis, which mimicked acute appendicitis due to local infiltration.3This case also presented with bladder and sigmoid colon infiltration.
The management of the contralateral testis in cryptorchid patients is controversial and there are no firm guidelines for their management. Some authors suggest prophylactic orchiectomy of the uninvolved testis as the preferred option rather than stringent follow up of unreliable patients.11 In our case we did an elective orchiectomy on the opposite testis in order to avoid the risk of carcinogenesis. The histopathology of the opposite testis revealed hypoplastic tubules. The management of an abdominal testis is very difficult. However we achieved complete response at nine months after treatment with combination chemotherapy.
In conclusion, the abdominal variant of cryptorchid testis is very rare and carries a high risk of malignant transformation to seminoma. Very rarely they can affect the nearby viscera presenting as acute abdomen. Early diagnosis with detection of an undescended testis and proper management can result in good long term cure.
Malignant transformation of the cryptorchid testis