Late relapse of a testicular germ cell tumour is an uncommon occurrence. We report a case of osteolytic bone metastasis appearing 23 years after the initial treatment of a metastatic testicular mixed tumour (choriocarcinoma and embryonal carcinoma). This is one of the longest periods of recurrence reported for testicular germ cell tumours.
A 52-year-old Caucasian man who underwent a right inguinal orchiectomy due to testicular tumour in 1984 presented to our outpatient clinic in a generally bad condition of health and with severe pain of his right hip joint and os ischii caused by osteolytic metastasis.
This case emphasizes the need for a life-long follow-up of patients with primary metastatic testicular cancer.
A twenty-six year old male with decreased vision in right eye was diagnosed with non-rhegmatogenous retinal detachment. Further evaluation revealed a yet undiagnosed left testicular mass found to be choriocarcinoma on histopathology. Chemotherapy resulted in complete remission; however, there was no improvement in vision. There is a substantial risk of visual loss in the presence of ocular metastatic lesions. Early detection and treatment is highly effective in terms of tumor control and a more favorable survival and visual outcome. The onus of the ocular diagnosis lies on the ophthalmologist. The presence of a testicular origin tumor must be considered in a young male with intraocular metastasis.
Choroidal metastasis; Retinal detachment; Choriocarcinoma of testis
Choriocarcinomas generally develop in females. Non-gestational choriocarcinoma in males is extremely rare. The present study describes two cases of young males who were diagnosed with intracranial choriocarcinoma. One case was of an aggressive choriocarcinoma with multiple metastases to the brain, but with an unidentified origin. The patient was admitted in the terminal stage of the cancer. Although a tumor resection was performed, the condition of the patient rapidly deteriorated and chemotherapy was not recommended. The patient succumbed nine days after the surgery. The second case was of a primary ventricular choriocarcinoma. The patient was hospitalized for acute hydrocephalus caused by a mass that was located in the ventricle. Following a tumor resection, the patient underwent a course of whole-brain and spinal radiotherapy. The patient was followed up for more than half a year and remained in a good condition. The present study describes the two cases and a comprehensive review of the literature that was performed to identify similar studies that document choriocarcinomas in males.
intracranial choriocarcinomas; male; tumorigenesis; prognosis
A report of choroidal neovascularisation (CNV) associated with radiation retinopathy. A 43-year-old Caucasian man presented with a 4-week history of sudden loss of central vision in the left eye, 8.5 years following radical radiotherapy for left tempero-parietal anaplastic astrocytoma. His visual acuity was 6/6 in the right eye and 6/60 in the left eye. Ophthalmoscopy of the left eye showed central macular pigmented area surrounded by subretinal fluid, haemorrhage and exudates. Fundus fluorescein angiography revealed left subfoveal CNV with surrounding macular oedema and peripheral retinal ischaemia. Radiation retinopathy associated CNV is a late ocular complication of cerebral radiotherapy. It should be investigated as a possible aetiology of severe visual loss in long-term survivors of cerebral tumours who have previously received radiotherapy.
The role of surgery in the treatment of patients with metastatic choriocarcinoma has diminished. We present a case of chemo-resistant metastatic choriocarcinoma salvaged by surgery. A 48-year-old patient presented with uterine perforation and severe intractable hemorrhage, and histological examination revealed a choriocarcinoma. After 6 years of disease-free state, recurrence occurred in the rectosigmoid colon. Seven cycles of EMACO chemotherapy was administered, and the human chorionic gonadotropin level was normalized. Three months after the chemotherapy, the rectosigmoid colon metastasis progressed. Low anterior resection with lymphadenectomy up to the level of the inferior mesenteric artery was conducted. After the operation, the human chorionic gonadotropin level decreased to within the normal range. There has been no evidence of disease for 13 months since the operation. Local resection of metastases seems to play a significant role in curing the disease in a small subset of patients.
Choriocarcinoma; Colon; Low anterior resection
Choriocarcinoma in the testis is very rare, and it represents less than 1% (0.3%) of all the testicular germ cell tumors. It is a particularly aggressive variant of non-seminoma tumor, which is characterized by a high serum β-HCG level and multiple lung metastases. The optimal management for this disease remains undefined. We report here on a case of choriocarcinoma with multiple lung metastases, and the patient has achieved continuous remission for 2 years after combination chemotherapy of BEP (bleomycin, etoposide and cisplatin) and sequential high-dose chemotherapy with autologous peripheral stem cell rescue.
Neoplasms; Germ cell and embryonal; Testicular choriocarcinoma; High-dose chemotherapy
Malignant transformation describes the phenomenon in which a somatic component of a germ cell teratoma undergoes malignant differentiation. A variety of different types of sarcoma and carcinoma, all non-germ cell, have been described as a result of malignant transformation.
A 33-year-old man presented with a left testicular mass and elevated tumour markers. Staging investigations revealed retroperitoneal lymphadenopathy with obstruction of the left ureter and distant metastases. Histopathology from the left radical orchiectomy showed a mixed germ cell tumour (Stage III, poor prognosis). The ureter was stented and four cycles of cisplatin, etoposide and bleomycin chemotherapy administered. After initial remission, the patient recurred four years later with a large retroperitoneal mass involving the renal vessels and the left ureter. Left retroperitoneal lymph node dissection with en-bloc resection of the left kidney was performed.
Histopathology revealed a germ cell tumour metastasis consisting mainly of mature teratoma. Additionally, within the teratoma a papillary renal cell carcinoma was found. The diagnosis was supported by immunohistochemistry showing positivity for AMACR, CD10 and focal expression of RCC and CK7. There was no radiological or histo-pathological evidence of a primary renal cell cancer.
To the best of our knowledge, malignant transformation into a papillary renal cell carcinoma has not been reported in a testicular germ cell tumour metastasis following platinum-based chemotherapy. This histological diagnosis might have implications for potential future therapies. In the case of disease recurrence, renal cell cancer as origin of the recurrent tumour has to be excluded because renal cell carcinoma metastases would not respond well to the classical germ cell tumour chemotherapy regimens.
Retroperitoneal teratoma; Malignant transformation; Germ cell tumour metastasis; Renal cell cancer
The recurrence or metastasis of intracranial pure germinomas as germ cell tumours of different histological types has rarely been reported. The present report concerns the first case in the literature of intracranial recurrence of a germinoma transformed into a choriocarcinoma. A 17-year-old man presented with a 1 month history of headache. MRI revealed a homogeneously enhanced mass within the pineal and suprasellar regions. The tumour was resected, and histological examination identified it as a pure germinoma. After resection, the patient underwent chemotherapy and radiotherapy and the mass disappeared. At 5 years and 5 months later, the patient presented with nausea and headache, and recurrence of the tumour was revealed in the left lateral ventricle. The tumour was resected and histological diagnosis identified it as a choriocarcinoma. The patient was treated with chemotherapy and radiotherapy, but died due to dissemination 1 month later. An autopsy was not performed.
Testicular cancer is the most common cancer for males aged 15~35 years old. The initial presentation is typically an asymptomatic enlarged testicle. The retroperitoneum is the most common metastatic area. Other metastatic sites include the lung, liver, brain, adrenal glands, gastrointestinal tract and spleen. Skin metastasis is a rare event and frequently associated with poor prognosis.
A 19-year old male was diagnosed testicular mixed germ cell tumor with initial presentation of cutaneous metastasis at scalp and upper abdomen. After radical orchiectomy and four courses of cisplatin-based chemotherapy, the scalp and upper abdominal lesions regressed completely. The size of lung metastases remained unchanged.
For advanced stage testicular cancer, cisplatin-based chemotherapy is still effective to achieve partial response.
A 43-year-old male presented with a painless left testicular mass. The pathologic diagnosis of the radical orchiectomy specimen was peripheral T-cell lymphoma, unspecified (PTCL-u). According to the Ann Arbor staging system, his initial stage was III because of the right nasopharyngeal involvement. After first-line chemotherapy with four courses of the CHOP regimen and this was followed by involved-field radiotherapy, he achieved complete remission. Two months later, disease recurred to the left ciliary body of the left eye without evidence of involvement at other sites. Although the patient received intensive chemotherapy with autologous hematopoietic stem cell transplantation, he ultimately died of leptomeningeal seeding. Because both the central nervous system (CNS) and the orbit are sanctuary sites for chemotherapy, orbital infiltration of lymphoma should prompt physicians to evaluate involvement of the CNS and to consider performing prophylactic intrathecal chemotherapy as a treatment option.
Non-Hodgkin's lymphoma; T cell lymphoma; Testes; Eye neoplasm
Most malignant testicular neoplasms are of germ cell origin. They are divided into five basic types: seminomas, embryonal carcinomas, teratocarcinomas, adult teratomas and choriocarcinomas. Clinically they may present as an enlarging testicular mass, or with symptoms resulting from metastases or hormonal secretions. The treatment of choice for patients with seminomas is orchiectomy, followed by radiation therapy. This combination results in an 80 to 100 percent five-year survival rate in patients with nonmetastatic or locally metastatic disease. The treatment of nonseminomatous germ cell tumors is more controversial. An aggressive approach, however, with retroperitoneal lymph node dissection and adjuvant chemotherapy has resulted in an overall 78 percent survival rate. Several placental and fetal proteins are secreted by these tumors. Two of these, human chorionic gonadotropin and alpha-fetoprotein, have been shown to be useful for the diagnosis of these neoplasms, for following the disease activity during therapy and for detection of recurrences.
The development of sarcomatous component (SC) in testicular germ cell tumor (GCT) is an uncommon phenomenon. We searched our surgical pathology files from 1985 to 2007 and identified 33 cases of testicular GCTs with SC. The average age of patients was 31 years. All patients underwent radical orchiectomy, which demonstrated a GCT in all patients except for 3 patients who had received neoadjuvant chemotherapy. All testicular GCTs contained a teratomatous component. The GCTs were pure teratomas in 3 cases, and were mixed GCTs in the other cases. The SC was observed in primary testicular tumor (n = 19), in metastasis (n = 11), or in both primary testicular tumor and metastasis (n=3). The average percentage of the SC in the primary testicular GCT was 32% (range, 5% to 99%). The most common histologic type of SC was rhabdomyosarcoma (n = 24), followed by high-grade unclassified sarcoma (n = 5), rhabdomyosarcoma admixed with high-grade unclassified sarcoma (n = 2), angiosarcoma (n = 1), and low-grade myxoid sarcoma (n = 1). Clinical follow-up information was available for 27 patients. Of the 13 patients whose SC was limited to the testicular GCT, 2 died of GCT not otherwise specified (NOS) at 37 and 68 months, respectively; and 11 patients were free of disease at a mean of 46 months. Of the 14 patients with a SC in the metastasis, 7 patients died of GCT NOS at a mean of 95 months, and 7 patients were free of disease at a mean of 104 months. These results suggest that patients with a SC confined to the primary testicular GCT may not have a higher risk of mortality than those at a comparable stage without a SC. However, patients with a SC in the metastasis have an increased risk of mortality.
testicular germ cell tumor; sarcomatous component; rhabdomyosarcoma
Polyarteritis nodosa (PAN) is a systemic vasculitis which may result in thrombosis or aneurysm formation in any organ of the body. We report a case polyarteritis nodosa (PAN) resulting in bilateral asynchronous testicular necrosis. A 55-year-old male developed acute onset of left testicular pain resulting in a left orchiectomy and right orchidopexy for an ischemic left testicle without evidence of torsion. Three weeks later, the patient developed acute right-sided scrotal pain, and surgical exploration revealed a right necrotic testicle resulting in a right orchiectomy. Pathologic evaluations demonstrated benign testes with acute interstitial hemorrhage and focal atrophy. The patient also experienced abdominal skin necrosis, penile pain and swelling, and temporary loss of vision. This is a unique case of PAN and the only case of asynchronous testicular necrosis in the medical literature.
We report the case of a true hermaphrodite with testicular seminoma with resulting metastases to the inguinal lymph nodes eight months after radical orchidectomy. This is an unusual presentation of testicular cancer and, to the best of our knowledge, the first report of this kind in the literature.
A 45-year-old Caucasian true hermaphrodite, raised as a male, developed a testicular seminoma. He had undergone a left orchidopexy at the age of 10 for undescended testes. Metastases from testicular tumors to inguinal lymph nodes are a rare occurrence. It has been suggested that previous inguinal or scrotal surgery may alter the pattern of nodal metastasis of testicular cancer. We review the literature to evaluate the incidence of inguinal lymph node involvement in early stage testicular cancer and discuss possible routes of metastases to this unusual site. We also discuss the management of the inguinal lymph nodes in patients with testicular tumors and a previous history of inguinal or scrotal surgery, as this remains controversial.
Inguinal lymph node metastases from testicular cancer are rare. A history of inguinal or scrotal surgery may predispose involvement of the inguinal nodes. During radical inguinal orchidectomy, the surgeon should be careful to minimize the handling of the testis and ensure high ligation of the spermatic cord up to the internal inguinal ring to reduce the risk of inguinal lymph node metastasis.
This report presents a case of primary intratesticular leiomyosarcoma. A 73-year-old male presented with a 6-year history of left scrotal swelling. A radiological examination revealed a left testicular tumour with multiple metastases in the lung, para-aortic lymph node and other organs. A radical orchiectomy was carried out and the pathology revealed an intratesticular leiomyosarcoma. The patient received additional chemotherapy. Cases of primary intratesticular leiomyosarcoma are rare. This is, to the best of our knowledge, only the tenth case of leiomyisaocoma in an adult reported in the literature, and the first case involving multiple metastases.
We experienced a rare case of primary intracranial choriocarcinoma treated successfully with synchronous chemotherapy and radiotherapy followed by three consecutive courses of chemotherapy without surgery. A 19-year-old male patient presented with a two-week history of diplopia, headache, nausea and vomiting. Neurological examination revealed visual disturbance, bilateral hearing loss, bilateral sixth nerve palsy, left skew deviation, downgaze limitation and clockwise torsional nystagmus on the left upgaze. During image evaluation, the patient suddenly presented with a confused mental status, bradycardia and decreased respiration. An emergency third ventriculostomy was conducted, followed by synchronous ifosfamide, cisplatin and etoposide chemotherapy (ICE; I, 900 mg/m2; C, 20 mg/m2; and E, 60 mg/m2) on days 1–5, and external radiotherapy (whole brain, 30 Gy/15 Fxs; local boost, 30 Gy/15 Fxs) followed again by three consecutive courses of chemotherapy. This therapy resulted in tumor regression by 63% and full improvement in the patient’s neurological condition. However, it was difficult to remove the remaining tumor using a surgical approach due to the risk of postoperative hemorrhage and limited access. We planned to observe the remaining mass without surgery. This patient is now alive and the mass size has not changed for 18 months since treatment. This is the first report of the effectiveness of synchronous chemotherapy and radiotherapy followed by consecutive chemotherapy without surgery in a patient with primary intracranial choriocarcinoma.
synchronous chemotherapy and radiotherapy; consecutive chemotherapies; primary intracranial choriocarcinoma
Gastric choriocarcinoma is a rare neoplasm and usually accompanies gastric adenocarcinoma. The prognosis is poor due to the aggressive course of the disease. A 57-year-old female patient with weight loss and abdominal pain was examined. The patient was operated following the examination, and pathological analysis revealed the presence of a gastric adenocarcinoma associated with choriocarcinoma. Immunohistochemical analysis showed a positive reaction with antibodies to beta-human chorionic gonadotropin and overexpression of the cErbB2 proto-oncogene. Staging revealed multiple metastases in the liver. A complete response was obtained with a combination of trastuzumab and chemotherapy. The diagnosis of gastric choriocarcinomas without pathological examination is difficult due to their rare occurrence. A complete response can be obtained with trastuzumab in the treatment of cases with overexpression of the cErbB2 protein.
Gastric choriocarcinoma; cErbB2; Trastuzumab
Diverse sequelae of central nervous system metastasis of choriocarcinoma have been reported, including infarction, intra or extra axial hemorrhages, aneurysm formation and carotid-cavernous fistula. Here we report a case of subdural hematoma as the first presentation of choriocarcinoma.
The patient is a 34-year-old woman whose initial presentation of widely metastatic choriocarcinoma was an acute subdural hematoma, requiring decompressive craniectomy. Histopathologic examination of the tissue showed no evidence of choriocarcinoma, but the patient was found to have diffuse metastatic disease and cerebrospinal fluid indices highly suggestive of intracranial metastasis.
Choriocarcinoma frequently metastasizes intracranially. We review the diverse possible manifestations of this process. In addition, the cerebrospinal fluid:serum beta-human chorionic gonadotropin ratio is an important factor in diagnosing these cases. Finally, the role of the neurosurgeon is discussed.
We report a case of a 55-year-old male patient with breast carcinoma, who developed choroidal metastasis. The patient had undergone mastectomy for carcinoma of right breast, five years ago. The patient was advised close follow-up for the left eye, as he was already on tamoxifen therapy (started a month ago) for spinal metastasis. On last follow-up, a year later, the choroidal lesion had completely scarred, with no recurrences. Systemic hormonal therapy like tamoxifen given for the breast primary and other systemic metastases may cause regression of the choroidal metastasis, thereby avoiding ocular radiotherapy. Medline search revealed only one published case of regression of choroidal metastasis from a male breast primary, on tamoxifen therapy.
Choroidal metastasis; male breast carcinoma; tamoxifen
Choroidal metastases secondary to urothelial carcinoma are extremely rare and are usually associated with an extremely poor prognosis. We present a case of an 88-year-old man with newly diagnosed urothelial carcinoma of the bladder who presented with acute loss of vision before commencing definitive concurrent chemoradiotherapy to the bladder. Ophthalmological examination demonstrated bilateral choroidal metastases. He received palliative radiotherapy to the orbits and completed his planned radiotherapy to the bladder. He remained disease-free at last follow-up 4 years after the completion of treatment. We review the literature particularly with regard to diagnosis and management of choroidal metastases. Choroidal metastases should be considered in a patient with a history of urothelial cancer presenting with new onset of eye symptoms.
Late relapses of testicular germ cell tumor are uncommon. We report a case of cervical mature teratoma appeared 17 years after treatment of testicular teratocarcinoma.
A 20- year- old patient underwent left sided orchiectomy followed by systemic therapy and retroperitoneal residual mass resection in 1989. He remained in complete remission for 200 months. In 2005 a huge left supraclavicular neck mass with extension to anterior mediastinum appeared. Radical surgical resection of the mass was performed and pathologic examination revealed mature teratoma.
This is one of the longest long-term reported intervals of a mature teratoma after treatment of a testicular nonseminoma germ cell tumor. This case emphasizes the necessity for follow up of testicular cancer throughout the patient's life.
A 23-year-old man with a right scrotal mass and back pain was referred for further treatment after right radical orchiectomy for testicular cancer. CT scans brought by the patient showed extensive metastasis to the retroperitoneal lymph nodes with no lung involvement. α-Fetoprotein and human chorionic gonadotropin were elevated preoperatively (384 ng/ml and 112 mIU/ml, respectively). Confirmation of the histopathologic examination revealed a mixed germ cell tumor (95% immature teratoma and 5% embryonal carcinoma). We started the patient on chemotherapy with bleomycin, etoposide, and cisplatin (BEP). After a single course, tumor markers began to normalize, but there was radiologic evidence of continued growth of the retroperitoneal mass and new metastases in the lung. The patient was given 2 courses of salvage chemotherapy with etoposide, ifosfamide, and cisplatin (VIP). However, the mass and lung metastases continued to progress, and the patient was growing rapidly intolerant of the side effects of treatment (i.e., nausea, appetite loss, and pancytopenia). After thorough discussion with the patient and his family, we decided to start the patient on interferon (IFN)-α therapy. Natural, nonrecombinant IFN-α (OIF, Otsuka, Japan) 5,000,000 IU was administered twice weekly with approval of the ethics committee of our institution. The patient responded moderately with marked deceleration of tumor growth and stabilization of the lung metastases. He is alive and well at 16 months on IFN-α therapy.
Testicular tumor; Immature teratoma; Growing teratoma syndrome; Interferon-α
Synchronous presentation of more than one germ cell tumours of different histology in the same patient is considered to be very rare. In these cases of multiple germ cell tumours, strong theoretical and clinical data suggest an underlying common pathogenetic mechanism concerning genetic instability or abnormalities during the pluripotent embryonic differentiation and maturation of the germ cell.
A 25 year-old young man presented with an enlarging, slightly painful left cervical mass. Despite the initial disorientation of the diagnosis to a possible thyroid disorder, the patient underwent complete surgical resection of the mass revealing mediastinal choriocarcinoma. Subsequent ultrasound of the scrotum indicated the presence of a small lobular node in the upper pole of the left testicle and the patient underwent radical left inguinal orchiectomy disclosing a typical seminoma. Based on these results, the patient received 4 cycles of Bleomycin, Etoposide and Platinum chemotherapy experiencing only mild toxicity and resulting in complete ongoing clinical and biochemical remission.
The pathogenesis of concurrent germ cell tumours in the same patient remains an area of controversy. Although the genetic instability of the pluripotent germ cell offers an adequate explanation, the possibility of metastasis from the primary, less differentiated tumour to a distant location as a more mature subtype cannot be excluded. Possible development of a metastatic site of different histology and thus biological behaviour (e.g choriocarcinoma) should be anticipated. Furthermore, urologists, pathologists and medical oncologists should be meticulous in the original pathological diagnosis in these patients, since there is a significant frequency of germ cell tumours with mixed or overlapping histological elements with diverse potential of evolution and differentiation.
We report a case of gastric choriocarcinoma admixed with an α-fetoprotein (AFP)-producing adenocarcinoma. A 70-year-old man was hospitalized for gastric cancer that was detected during screening by esophagogastroduodenoscopy (EGD). Initial laboratory data showed the increased serum level of AFP and EGD revealed a 5-cm ulcerofungating mass in the greater curvature of the gastric antrum. The patient underwent radical subtotal gastrectomy with D2 lymph node dissection and Billroth II gastrojejunostomy. Histopathological evaluation confirmed double primary gastric cancer: gastric choriocarcinoma admixed with an AFP-producing adenocarcinoma and separated adenocarcinoma. At 2 wk postoperatively, his human chorionic gonadotropin and AFP levels had reduced and six cycles of adjuvant chemotherapy were initiated. No recurrence or distant metastasis was observed at 4 years postoperatively.
α-fetoproteins; Adenocarcinoma; Choriocarcinoma; Stomach neoplasms
Here, we present the case of a patient with bilateral choroidal metastases with extraocular extension in one eye. Metastasis of papillary thyroid carcinoma to the uvea is extremely rare, with only 6 patients reported in the literature. A 62-year-old man with a prior history of papillary thyroid carcinoma suffered the rapid loss of vision in his right eye. He had neovascular glaucoma, total retinal detachment, and a solitary choroidal mass. A month later, his left visual acuity also decreased because of a small macular choroidal mass. The right eye was enucleated and a nodular lesion over the sclera representing extraocular extension was observed. This tumor and the intraocular lesion were composed of papillary excrescences and cystic spaces and stained positively for thyroid transcription factor 1 and thyroglobulin, all confirming the diagnosis of metastatic papillary thyroid carcinoma. The tumor in the left eye was successfully treated with diode laser transpupillary thermotherapy. The patient expired within a month as a result of widespread pulmonary metastases. Papillary thyroid carcinoma may metastasize to the uvea bilaterally, cause rapid visual function loss, erode the sclera, and may extend outside the globe similar to choroidal melanoma. This aggressive ocular involvement was associated with a dismal prognosis in our patient.
Choroid; Neoplasm metastasis; Papillary carcinoma; Thyroid gland; Transpupillary thermotherapy