Testicular germ cell tumor with rhabdomyosarcoma successfully treated by disease-adapted chemotherapy including high-dose chemotherapy: case report and review of the literature

Abstract

A teratoma is associated with malignant nongerm cell tumors in 3 to 6% of patients with metastatic GCT who are treated with platinum-based CHT. 1-3 A derivation from preexistent mesenchymal elements of the teratomatous tissue is assumed. 4 Alternatively, the nongerm tissue may arise from the malignant transformation of totipotential germ cell subpopulations showing partial differentiation into somatic mesenchymal tissue. 5 Up to 80% of patients present with metastases. 4 Most nongerm cell malignancies associated with GCT are sarcomas, rhabdomyosarcoma being the most common subtype. 6 Sarcomas can be present in the primary tumor or appear in metastases. 1,7 Teratomas with rhabdomyosarcomatous differentiation have a poor prognosis. 4 Here, we report a rare case of a combination of seminoma, teratoma and rhabdomyosarcoma. To our knowledge, this is the first report of successful treatment of a metastasized teratoma with rhabdomyosarcomatous differentiation, with HD-CHT and APBSCT. Case reportA 51-year-old man with a left testicular mass underwent inguinal orchidectomy in November 1996. Histologically, the tumor was a combined seminoma, partially immature teratoma and rhabdomyosarcoma. Biopsy of the right testis revealed no malignancy. AFP and ␤HCG serum levels were normal. Retroperitoneal lymph node dissection was performed 3 weeks later due to the unfavorable histology and revealed no evidence of malignant spread.Three months later, the patient was admitted to our hospital because of generalized seizures. There was a 4-week history of back pain, appetite loss, nausea, vomiting, and lethargy. On physical examination, the patient was somnolent but there were no other significant abnormalities. CT scan of the brain and lumbar puncture yielded normal findings. Hematologic and blood chemical tests showed an elevated creatinine (489 mol/l; normal 58-110 mol/l), lactate dehydrogenase (1099 U/l; normal 120-240 U/l), and calcium (4.0 mmol/l; normal 2.24-2.78 mmol/l). A bone scan revealed no radiolabeled technetium uptake. Cytologic and histologic BM examination showed massive rhabdomyosarcoma involvement (Figure 1a), which was confirmed by histochemical stains positive for muscle-specific antigen and desmin (Figure 1b). MRI of the vertebral column revealed multiple spots with low signal intensity in T1-and high signal intensity in T2-weighted images typical of tumor infiltration (Figure 2a). A solitary metastasis was found in the liver.Infusions of normal saline, furosemide, steroids, and pamidronate were given. CHT with 50 mg/m 2 epirubicin for 1 day, etoposide 250 mg/m 2 , 2000 mg/m 2 ifosfamide, and 25 mg/m 2 cisplatin for 2 days each was initiated. The patient regained normal conciousness, and calcium and creatinine levels returned to normal. Four CHT courses were given (total epirubicin dose 200 mg/m 2 ) and 90 mg pami-

Retroperitoneal teratoma with somatic malignant transformation: A papillary renal cell carcinoma in a testicular germ cell tumour metastasis following platinum-based chemotherapy

“…Somatic MT develops in 3-6% of chemotherapy- naïve patients with metastatic GCT containing teratomous components [4,9]. After platinum-based chemotherapy the incidence of MT increases up to 14% [4].…”

Teratoma with Malignant Transformation: A Case Report with Pathological, Cytogenetic, and Immunohistochemistry Analysis

“…Patients who had complete resection of TMTs had significantly better overall survival, and the prognosis for patients with metastatic disease was very poor [3]. Recent reports indicate that chemotherapy directed against transformed component may achieve better results [11]. There have been only a few reports of successful treatment of metastatic TMTs with chemotherapy with long-term survival to doxorubicin-based chemotherapy [10, 11].…”

“…Recent reports indicate that chemotherapy directed against transformed component may achieve better results [11]. There have been only a few reports of successful treatment of metastatic TMTs with chemotherapy with long-term survival to doxorubicin-based chemotherapy [10, 11]. …”

“…The prognosis is especially poor for mediastinal TMTs and for those with neural or rhabdomyosarcomatous differentiation [1, 2, 7]. Surgical resection is the mainstay of therapy for localized disease, because TMTs are considered to be resistant to radiation and systemic chemotherapy [8–11]. Effective therapeutic strategies targeted to TMT are needed.…”

A Review of Second-line Chemotherapy and Prognostic Models for Disseminated Germ Cell Tumors

“…The rate of malignant transformation increased in this group. 98, 99 Malignant transformation should be suspected if the disease does not respond to cisplatin-based chemotherapy or growth is seen on imaging without rise of disease tumor markers despite cisplatin-based chemotherapy. Isochromosome of chromosome 12, i(12p), is a specific marker of GCT.…”