A case of metastatic paratesticular rhabdomyosarcoma in an adult successfully treated with multidrug combination chemotherapy

A 20-year-old man was referred to our hospital with the complaint of a mass in the left scrotum. Computed tomography showed retroperitoneal lymph node metastases and multiple lung metastases. He underwent left high orchiectomy and was diagnosed with paratesticular embryonal rhabdomyosarcoma. He received combination chemotherapy consisting of cyclophosphamide, vincristine, doxorubicin, and dacarbazine. After four cycles of chemotherapy, lung metastases disappeared and lymph nodes decreased dramatically. He was alive without recurrence 31 months after the operation.     

A case of paratesticular rhabdomyosarcoma

A case of paratesticular rhabdomyosarcoma in an 18-year-old male is reported. The patient was admitted for swelling of the right scrotum and right inguinal pain. Malignant tumor of the right testis was suspected and a high inguinal orchiectomy was performed. The pathological diagnosis was paratesticular rhabdomyosarcoma. Following retroperitoneal lymph node extirpation, chemotherapy, so-called CYVADIC therapy, consisting of cyclophosphamide, vincristine, adriamycin and dacarbazine was employed in 3 regions. The patient is in good health without recurrence 16 months after the surgery.     

Paratesticular rhabdomyosarcoma: results of therapy in 18 cases

The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.     

睾旁横纹肌肉瘤（附三例报告）

目的 提高对睾旁横纹肌肉瘤的认识。方法 对3例睾旁横纹肌肉瘤患者的发病、诊断及治疗情况进行总结。结果 3例均行根治性睾丸切除＋同侧阴囊切除＋腹膜后淋巴清扫术。Ⅰ期2例,Ⅱ期1例。1例Ⅰ期患者术后采用足叶乙甙、丝裂霉素、顺铂联系化疗,余2例未行进一步治疗。3例分别随访28、18及6个月,均未见肿瘤复发和转移。结论 手术辅以化疗、放疗是治疗睾旁横纹肌肉瘤的有效手段。     

A case of paratesticular rhabdomyosarcoma

We report a case of paratesticular rhabdomyosarcoma in a 16-year-old male. The patient with a mass in the right scrotum was at first diagnosed with testicular tumor. Right orchiectomy revealed paratesticular rhabdomyosarcoma. Para-aortic lymph node metastasis was pointed out by abdominal computed tomography. Although he received multidrug chemotherapy consisting of vincristine, adriamycin, cyclophosphamide, cisplatin, and actinomycin-D (IRS-III regimen 35 and IRS-IV regimen) without radiotherapy, he died twenty-one months after the operation.     

Two cases of paratesticular rhabdomyosarcoma

Two cases of paratesticular rhabdomyosarcoma are reported. A 15-year-old boy was admitted in July, 1983, with a 3-week history of gradually increasing painless scrotal swelling on the right side. With the suspicion of testicular cancer, right radical orchiectomy was performed and pathological examination revealed an embryonal rhabdomyosarcoma of the paratesticular region. Chemotherapy and retroperitoneal lymphadenectomy were performed, and the latter revealed lymph node metastasis at the bifurcation of the aorta. Radiotherapy was not done because of adherent ileus. About 8 months after the orchiectomy, he died of recurrence at pelvic cavity and brain metastasis. A 6-year-old boy presented in March, 1985, with a complaint of right scrotal swelling that was painless and gradually increasing for about a month. Transinguinal exploration revealed a paratesticular neoplastic lesion and right radical orchiectomy was performed. Pathological diagnosis was paratesticular embryonal rhabdomyosarcoma. No evidence of metastasis was found by chest X-ray, excretory urogram, abdominal CT-scan or lymphangiography. As post-operative treatment, only chemotherapy was performed, and 9 months after the orchiectomy, the patient was asymptomatic. Ninety four cases of paratesticular rhabdomyosarcoma found in the Japanese literature are reviewed and mainly the policy of treatment is discussed.     

A case of intrascrotal rhabdomyosarcoma

We report a case of intrascrotal rhabdomyosarcoma. A 15-year-old boy visited our clinic with the chief complaint of swelling of the right hemiscrotum. He had a history of fever and right hemiscrotal pain. With the initial diagnosis of acute epididymitis, the patient underwent medical therapy with antibiotics. One month later, because the mass had not responded to medical therapy, a right inguinal orchiectomy was done. Histologic examination revealed rhabdomyosarcoma. The patient received retroperitoneal lymph node dissection and combined chemotherapy with cyclophosphamide, bleomycin, actinomycin-D, adriamycin and vinblastine. He was well 5 years and 10 months postoperatively with no evidence of tumor recurrence or metastasis.     

Successful salvage chemotherapy with gemcitabine,etoposide and cisplatin for metastatic ureteral cancer: a case report

A 35-year-old man who had undergone nephroureterectomy and a single cycle of adjuvant MVAC chemotherapy for the left ureteral cancer was referred our clinic for the treatment of paraaortic lymph node metastases. Following histologic confirmation of transitional cell carcinoma by computed tomography (CT) guided biopsy, we treated him with combination chemotherapy consisting of ifosfamide, 5-fluorouracil, etoposide and cisplatin. After 5 cycles of chemotherapy complete remission was obtained. Six months later, however, metastases recurred in the left supraclavicular and paraaortic lymph nodes. Thus, we treated him with a new combination chemotherapy comprising gemcitabine, etoposide and cisplatin which was approved as a phase I study by the institutional review board. Although he was the first patient enrolled in the study and received the minimum dose of gemcitabine (level 1), complete remission was again achieved. Adjuvant radiotherapy of 40 Gy was given to the metastatic sites. He has been well without evidence of disease for 12 months.     

A case of intrascrotal rhabdomyosarcoma with para-aortic lymph node metastasis--complete response after multidrug chemotherapy

We report a case of intrascrotal rhabdomyosarcoma in a 59-year-old-male. The patient with a mass in the right scrotum was at first diagnosed with epididymitis and treated with antibiotics. The mass however, grew gradually and right orchiectomy revealed intrascrotal alveolar rhabdomyosarcoma. Para-aortic lymph node metastasis was pointed out by abdominal computed tomography. He received multidrug chemotherapy consisting of vincristine, actinomycin-D, cyclophosphamide, adriamycin, and cisplatin (IRS-III regimen 35). A complete response was obtained 3 months after the start of the chemotherapy. The patient is alive without recurrence 2 years after the surgery.     

Paratesticular rhabdomyosarcomas and leiomyosarcomas: a clinicopathological review.

One case of embryonal paratesticular sarcoma and 2 cases of leiomyosarcomas are reported. The 13-year-old boy with embryonal sarcoma is well after an orchiectomy and high ligation of the spermatic cord followed by radiotherapy and chemotherapy. A 66-year-old man has been doing well after orchiectomy and hemiscrotectomy for a paratesticular leiomyosarcoma. The third patient had a highly pleomorphic leiomyosarcoma and died 2 months postoperatively. The clinical and histological diagnosis of paratesticular rhabdomyosarcomas and leiomyosarcomas is reviewed and the various therapeutic approaches to these neoplasms are discussed. Retroperitoneal lymph node dissection, radiotherapy and chemotherapy are important adjuncts to orchiectomy in the management of rhabdomyosarcomas. On the contrary, retroperitoneal lymph node dissection and radiotherapy are not indicated in leiomyosarcomas, since these neoplasms tend to metastasize by the hematogenous route and are radioresistant. At the present time we are unable to evaluate chemotherapy in the management of paratesticular leiomyosarcomas.     

Primary adult intratesticular rhabdomyosarcoma: Results of the treatment of six cases

Abstract: The aim of the present study was to assess the treatment outcomes in a cohort of adult patients with intratesticular rhabdomyosarcoma. Between 1999 and 2008, 296 patients underwent radical orchiectomy for intrascrotal mass. A retrospective chart review was carried out for adult patients diagnosed with intratesticular rhabdomyosarcoma. Overall, six patients (mean age 21 years, range 17–23) were included: five had embryonic rhabdomyosarcoma and one had pleomorphic rhabdomyosarcoma. Four patients underwent retroperitoneal lymph node dissection and five patients had postoperative chemotherapy. The mean length of follow up was 28 months (range 12–51 months). One patient was lost to follow up after 26 months, five cases remained alive at the end of the study. Surgical resection with chemotherapy was an effective way to achieve favorable outcomes and long‐term survival in adult patients with metastases from intratesticular rhabdomyosarcoma.     

Intra-arterial infusion chemotherapy for liver metastases of testicular tumors: report of two cases

Two cases of testicular tumors with lymph node involvement and multiple lung and liver metastases were treated successfully with intra-arterial infusion chemotherapy. Case 1: A 30-year-old man presented with right scrotal swelling and an abdominal mass. He had a large retroperitoneal mass and multiple lung and liver metastases on computed tomographic (CT) scan and chest X-ray. Right inguinal orchiectomy was performed. Histopathological diagnosis revealed embryonal cell carcinoma and choriocarcinoma. Cisplatin, vinblastine, VP-16 and pepleomycin combination chemotherapy (PVeBV) was started and repeated for 2 courses. The retroperitoneal mass and lung tumors decreased in size, but liver tumors enlarged. Systemic and intrahepatic arterial infusion combined with chemotherapy was administered, and intra-arterial chemotherapy (cisplatin, VP-16) was added. The patient also received systemic chemotherapy (carboplatin, VP-16, ifosfamide). After chemotherapy, retroperitoneal lymph node dissection was performed. Microscopic examination revealed no viable cancer cells. On CT scan, no retroperitoneal, liver, or lung tumor was detected. Case 2: A 43-year-old man presented with right scrotal swelling and an abdominal mass. CT scan revealed a large retroperitoneal mass as well as lung and multiple liver metastases. Right inguinal orchiectomy was performed. Histopathological diagnosis revealed seminoma. Cisplatin, vinblastine, VP-16 and pepleomycin combination chemotherapy (PVeBV) was administered, but the liver tumors ware enlarged on CT scan. Intrahepatic arterial infusion chemotherapy (cisplatin, VP-16) was started and repeated for 4 courses. On CT scan, the lung metastasis seemed to have disappeared, and the retroperitoneal mass and liver metastases were decreased in size.     

Complete Response of Highly Advanced Colon Cancer with Multiple Lymph Node Metastases to Irinotecan Combined with UFT: Report of a Case

Massive lymph node metastasis of the para-aortic region and supraclavicular lymph nodes, Virchow's lymph node metastasis due to colon cancer, is extremely rare. We herein report a case of such systemic lymph node metastasis that was successfully treated with a combination of irinotecan (CPT-11) and UFT, a combination drug of tegafur and uracil. The patient was a 57-year-old woman who had a tumor in the ascending colon, and massively swollen para-aortic and supraclavicular lymph node metastasis. She was treated with combination chemotherapy of CPT-11 and UFT. The main tumor was detected as a decompressed scar, and the supraclavicular and para-aortic lymph nodes had completely disappeared after the second cycle of treatment. A histopathological examination and immunohistochemistry with cytokeratin showed complete remission of adenocarcinoma in the tumor and para-aortic lymph nodes. She remains alive without recurrence 52 months after chemotherapy. Combination chemotherapy of CPT-11 and UFT may be of potential value in the treatment of advanced colorectal carcinoma, and both histopathological and immunohistochemical confirmation of a complete remission may indicate prolonged disease-free survival.     

Different transformation of mature teratoma in a patient with mixed germ cell tumor of the testis

A 44-year-old male was referred with a left supraclavicular lymphadenopathy. A biopsy of the lymph node showed metastatic embryonal carcinoma. Tumor markers were present at high levels: alpha-fetoprotein 253.9 ng/mL, beta-human chorionic gonadotrophin 62 ng/mL. Computed tomography (CT) showed retroperitoneal adenopathy. High orchiectomy was done. The patient was treated with three cycles of etoposide plus cisplatin, achieved normalization of the serum tumor markers and underwent retroperitoneal lymph node dissection. Pathological findings of multiple lymph nodes showed teratomatous glands without viable cells. At follow-ups performed every 3 months, tumor markers remained within normal limits and no evidence of recurrence was observed. Eight years after first admission a CT scan revealed a cystic tumor 1 cm in diameter in the para-aortic region. The cystic tumor continued to slowly grow, expanding by 1 cm in diameter per year without elevation of tumor markers. The para-aortic tumor had grown to 4 cm in diameter and a left supraclavicular lymphadennopathy recurred. A resection of the supraclavicular cystic tumor showed mucinous cystadenocarcinoma, but a cystic tumor in the para-aortic region revealed mature teratoma. Here we report a case of mature teratoma with metastases at supraclavicular and para-aortic lymph nodes which had different transformations in spite of both regions consisting of cystic tumors.     

Aortic replacement during post chemotherapy retroperitoneal residual tumor resection for nonseminomatous germ cell tumor: a case report

A 59-year-old man visited our hospital complaining of epigastralgia. A large hard mass was palpable in the abdominal cavity. Abdominal computed tomography revealed large retroperitoneal cystic tumors. His left testis was hard and swollen. Under the diagnosis of testicular tumor and retroperitoneal lymph node metastasis, left radical orchiectomy was performed and the histopathological examination showed mature teratoma. He was diagnosed with nonseminomatous germ cell tumor and retroperitoneal lymph node metastasis (TNM classification stage IIC). He received three cycles of chemotherapy with bleomycin, etoposide, and cisplatin and we performed retroperitoneal residual tumor resection. Because the tumor tightly adhered to the aortic wall, abdominal aorta was resected and replaced by an artificial vessel. The post-operative course was uneventful. Histopathological diagnosis was cystopapillary adenocarcinoma and mature teratoma. The patient is well 1 and a half years after the operation without recurrence.     

Paratesticular rhabdomyosarcoma in children.

The clinical characteristics, surgical treatment, clinical grouping, chemotherapy, radiotherapy, complications and survival of paratesticular rhabdomyosarcoma are discussed. When the combined approach of radical inguinal orchiectomy, retroperitoneal node dissection, chemotherapy and radiotherapy when indicated is used a survivorship in excess of 80 per cent can be anticipated for all stages of disease. A combined multidisciplinary approach to this lesion is stressed to achieve improved survivorship in this previously lethal lesion.     

Adult paratesticular sarcomas: a review of 21 cases

We reviewed 21 patients more than 16 years old who were seen with a diagnosis of paratesticular sarcoma from 1958 to 1987. Of the patients 14 presented with primary disease and 7 with recurrent disease. The survival of the primarily treated patients was 58% at 5 years, calculated by the product limit method. Of the 14 patients with primary disease 13 had grade 3/4 or 4/4 sarcoma and 13 of 14 underwent initial radical orchiectomy. In addition, 6 of the 14 patients underwent an adjuvant operation or radiotherapy to the groin, or groin and scrotum, and none had local relapse. Some patients also had chemotherapy. Three patients underwent adjuvant retroperitoneal node dissection and 2 had microscopically positive nodes. All 3 patients remain without relapse. Six patients had relapse: 2 locally, 2 in the retroperitoneal nodes and 2 with distant metastases. Only 1 patient (with scrotal recurrence) was salvaged. Of the 7 patients referred with recurrent disease none was salvaged. In this series there is a 2 of 14 (14%) risk of local failure and a 4 of 14 (28%) risk of retroperitoneal relapse after radical orchiectomy. Since salvage has not proved successful, patients with rhabdomyosarcoma, intermediate or high grade malignant fibrous histiocytoma, or fibrosarcoma should be considered for adjuvant retroperitoneal node dissection. All patients should undergo adjuvant dissection or irradiation of the ipsilateral pelvic and groin nodes, and scrotum.     

High-dose chemotherapy followed by peripheral blood stem cell transplantation in advanced extragonadal germ cell tumor--a case report]

We reported the experience of high-dose chemotherapy (HDC) combined with peripheral stem cell transplantation (PBSCT) in 29 years-old man with advanced retroperitoneal germ cell tumor accompanied with left supraclavicular lymph node metastases, who obtained complete remission after comprehensive treatment. The initial levels of serum AFP, hCG and beta-hCG were high at 30.2 ng/ml, 14,000 mIU/ml and 66 ng/ml, respectively. After 3 courses of chemotherapy (BEP regimen), while left supraclavicular lymph node swelling was disappeared, the retroperitoneal mass lesion persisted on CT scan. Not all of 3 markers fell to the normal range. After myelosuppressive chemotherapy (etoposide 500 mg/m2 Day 1-3), PBSCs were collected by two consecutive apheresises on Day 17 and 18. In total, 19.5 x 10(6)/kg CD 34 positive cells were obtained. The patient underwent PBSCT (all CD 34 positive cells were infused) on Day 0 following HDC (CBDCA 250 mg/m2/day, etoposide 300 mg/m2/day, IFM 1.5 g/m2/day, Day-7(-)-3, respectively). He became severely leukopenic and thrombopenic with nadir of 200/microliter on Day 6 and 2 x 10(4)/microliter on Day 2, respectively. By administration of platelet transfusion and G-CSF, the white blood cell counts and thrombocyte counts recovered to 6,400/microliter and 4.1 x 10(4)/microliter on Day 10, respectively. Microbiologically enterocolic and respiratory tract infections occurred with elevated body temperature (> 40 degrees C). Antibiotic and antimycotic treatments were continued until disappearance of all clinical and microbiological evidence. He was kept for 10 days in clean room. After HDC, all markers fell to the normal range, but the retroperitoneal residual mass still persisted. Resection of the residual mass and retroperitoneal lymph node dissection were performed with pathological examination revealing tissue necrosis without viable cell. The patient has survived with no sign of the disease for 9 months.     

Stage II nonseminomatous germ cell tumors of the testis: an analysis of treatment options in patients with low volume retroperitoneal disease

The management of patients with nonseminomatous germ cell tumors of the testis and low volume retroperitoneal disease remains controversial. We analyzed the treatment modalities of 56 patients divided into 3 groups: group I--18 clinical stage I cancer patients who had pathological stage II disease after retroperitoneal lymph node dissection, group II--31 patients with abdominopelvic computerized tomography abnormalities of 5 cm. or less with or without positive biological markers and group III--7 patients with persistently positive biological markers after orchiectomy with normal abdominopelvic computerized tomography scans. In group I 4 of 18 patients received chemotherapy after retroperitoneal lymph node dissection. Two patients met criteria for post-dissection chemotherapy, but they did not receive it and subsequently had relapse. Of the remaining 12 patients who were observed after dissection only 1 (8 per cent) had relapse. In group II 19 of 31 patients were treated with initial chemotherapy: 13 (68 per cent) achieved a complete response, while 6 required retroperitoneal lymph node dissection after chemotherapy. Of 31 patients 12 were treated with initial retroperitoneal lymph node dissection and 9 (75 per cent) received post-dissection chemotherapy. In group III 5 of 7 patients were treated with initial retroperitoneal lymph node dissection and 3 of the 5 subsequently required chemotherapy. Of the 7 patients 2 received initial chemotherapy and achieved a complete response. All 56 patients are without disease at 4+ to 106+ months (median 28 months). We conclude that patients with the characteristics of groups II and III should be managed initially with chemotherapy, with retroperitoneal lymph node dissection reserved for patients who fail to achieve a complete response. In group I retroperitoneal lymph node dissection is sufficient initial treatment if pathological evaluation of the retroperitoneal lymph nodes does not meet our criteria for post-dissection adjuvant chemotherapy.     

Testicular tumors in children

Purpose: The aim of this study was to present an updated picture of surgical management of pediatric testicular tumors based on our 30 years' experience, which consisted of one of the largest noncollected series treated in a single medical center. Methods: Records of children who were treated for testicular tumor in our unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures, treatment methods, histopathologic findings, and outcome. Results: Fifty-one patients with a mean age of 3.8 ± 0.5 years were treated for testicular tumors. Of these, 35 (69%) had germ cell testis tumor (GCT) and 16 (31%) had non–germ cell testis tumor (NGCT). Endodermal sinus tumor and paratesticular rhabdomyosarcoma were the dominant histologic subtypes in each group, respectively. The most common mode of presentation was painless scrotal mass. At initial presentation, retroperitoneal (n = 5), both retroperitoneal and lung (n = 2), and retroperitoneal and liver (n = 3) metastases were recorded in 10 (19%) patients. Initial operative procedures were radical inguinal orchiectomy (RIO) (n = 29), scrotal orchiectomy (SO; n = 9), bilateral RIO (n = 2), both RIO and unilateral retroperitoneal lymph node (RPLN) excision (n = 6), testis-sparing enucleation of the tumor (n = 5). SOs were performed elsewhere, and these patients underwent high ligation (n = 4) and both high ligation plus RPLN excision (n = 5) in our unit. Histopathologically, spermatic cord invasion and RPLN involvement were present in 10 patients. Scrotal recurrences were encountered in 2 patients who had scrotal orchiectomy initially. Retroperitoneal recurrences were noted in a patient presenting with stage I embryonal carcinoma and in 2 patients presenting with group IV paratesticular rhabdomyosarcoma. The mean follow-up period was 89 ± 10 months. Four patients with stage IV embryonal carcinoma (n = 2) and group IV paratesticular rhabdomyosarcoma (n = 2) died of progression of the disease. All remaining patients were alive and disease free at their last outpatient appointment. No significant difference was noted with regard to 5-year survival rates between (1) malignant GCT and paratesticular rhabdomyosarcoma patients (91% v 80%) and (2) patients treated by RIO (88%), SO plus high ligation (87%), and RIO plus RPLN excision (80%). Five-year survival rates were 100% for stage I, II, III patients and 33.3% for stage IV and group IV patients presenting with malignant testicular tumors (P < .05). Conclusions: Childhood testicular tumors deserve special attention from the therapeutic point of the view. A solid scrotal mass should be considered malignant until proved otherwise. Any suspicion of the testicular tumor warrants an inguinal approach to prevent scrotal violation by the tumor. Current trends emphasize that testis-sparing surgery should be performed for benign lesions such as teratoma, leydig cell tumor, and epidermoid cyst based on frozen biopsy findings. Literature findings and our experience suggest that RIO is the accurate treatment for stage I malignant GCT and group I and IIa paratesticular rhabdomyosarcoma. RPLN excision is not of benefit either as a staging or therapeutic procedure in stage I and group I and IIa diseases of these tumors. RPLN excision should be reserved for (1) malignant GCT patients who have persistent elevation of alpha-fetoprotein after orchiectomy in the presence of normal total body CT scan, and for patients presenting with stage II and III disease with definitive abnormality on CT scans, and (2) group IIb, IIc, and III paratesticular rhabdomyosarcoma patients with radiologic evidence of retroperitoneal involvement on CT scans. High ligation should be done as a complementary procedure after SO to increase the survival rates. J Pediatr Surg 36:1796-1801. Copyright © 2001 by W.B. Saunders Company.