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An adult patient with a mixed germ cell tumor of the spermatic cord
CASE REPORT
AN ADULT PATIENT WITH A MIXED GERM CELL TUMOR OF THE SPERMATIC CORD GEORGE ZOGRAFOS, CONSTADINA PAPADIMITRIOU, IIAS KOUERINIS, EVANGELOS MESSARIS, KALLIOPI PETRAKI, AND GEORGE ANDROULAKIS
ABSTRACT Tumors of the spermatic cord are very rare, and approximately one half of all primary spermatic cord tumors are malignant. We report the presentation and treatment of an adult (36-year-old) patient with a mixed germ cell tumor that originated in the spermatic cord. No similar cases of mixed tumors of the spermatic cord in adults have been reported. UROLOGY 55: 436xiv–436xvi, 2000. © 2000, Elsevier Science Inc.
T
he study of extragonadal germ cell tumors has aroused considerable interest in recent decades. Tumors of the spermatic cord are very rare. Approximately one half of all primary spermatic cord tumors are malignant. We report a case of mixed germ cell tumor originating in the spermatic cord in an adult patient. In 1974, Leaf et al.1 reported the first case in English published reports of an embryonal cell carcinoma in the spermatic cord in an infant. To our knowledge, our case is the first to be reported in an adult patient. CASE REPORT A 36-year-old male patient presented with a 3-month history of diffuse discomfort in the left iliac fossa. He had a palpable painful mass in the left groin, which had grown up to the previous 2 days, with a temperature up to 38°C. His past history, including an undescended testis, was negative. On physical examination, no evidence of an acute abdomen was found, except for the painful hard mass that did not move when the patient was coughing. Both testes were normal to palpation, except for a small hydrocele in the left scrotum. Laboratory tests were normal, except for the white blood cell count, which was 15,000/dL, with From the First Department of Propaedeutic Surgery, University of Athens, Hippocration Hospital, and Department of Pathology, Hippocration Hospital, Athens, Greece Address for correspondence: George Zografos, M.D., Alopekis 22, Athens 106 75, Greece Submitted: June 9, 1999, accepted (with revisions): October 18, 1999
436xiv
© 2000, ELSEVIER SCIENCE INC. ALL RIGHTS RESERVED
a neutrophilic type. The chest x-ray and the abdominal x-ray in the erect position did not show any specific sign. Both testes were normal by ultrasound. The computed tomography scan revealed a solid mass, 3 ⫻ 4 cm in diameter, located at the left deep inguinal ring (Fig. 1). In addition, lymph node masses, 3 ⫻ 3 cm in size, were found at the paraaortic region, below the origin of the renal vein, laterally (Fig. 2). Fine needle aspiration biopsy of the groin mass was performed twice, and histologic evaluation was made by two independent pathologists. Both evaluations revealed a malignant tumor of mesenchymal origin. Human chorionic gonadotropin, embryonic globulin, alpha-1-fetoprotein, and carcinoembryonic antigen levels were within normal limits. At surgery, a tumor of the spermatic cord at the deep inguinal ring was found. The testis was mobilized and was normal, with a small hydrocele. A left orchiectomy with very high ligation and removal of the spermatic cord en bloc was done, and a well-circumscribed tumor (3.5 ⫻ 1.2 cm) of the distal spermatic cord was found. The tumor was located approximately 20 cm from the superior pole of the testis. It was not encapsulated and had a uniform, yellowish, solid, and partially nodular appearance on the cut surface. The tumor was mostly necrotic. The histologic picture was of a mixed germ cell tumor that consisted of an anaplastic seminoma coexisting with large areas of embryonal cell carcinoma (approximately 50% of the tumor). The seminomatous cells were large and irregular, with hyperchromatic nuclei, considerable mitotic activity (four or more mitoses per high-power field) and showed positive immunoreactivity for placental alkaline phospha0090-4295/00/$20.00 PII S0090-4295(99)00545-2
FIGURE 1. Computed tomography scan revealing a solid mass, 3 ⫻ 4 cm in diameter, located at the left deep inguinal ring.
FIGURE 3. Area of embryonal cell carcinoma with solid growth pattern and some embryoid-like bodies. Hematoxylin-eosin stain, original magnification ⫻100.
lymphadenectomy, from the bifurcation of the aorta to the common iliac arteries to the origin of the renal vein, including the residual mass, was done.2 A necrotic mass lesion was removed, and pathologic evaluation revealed a completely necrotic tumor with no active malignant tissue in the mass or in the excised nodes. One month later, the patient underwent radiotherapy at a dose of 3000 cGy directed to the area previously occupied by the residual mass. FIGURE 2. Para-aortic lymph node mass below the origin of the renal vein, laterally.
tase (PLAP). The embryonal cell carcinoma exhibited a solid growth pattern, contained some embryoid-like bodies, and was immunoreactive for CAM 5.2, CD30, and MNF 116 (Fig. 3). Additional features were the presence of a small number of syncytiotrophoblastic cells and a granulomatous reaction containing epithelioid cells, which was extensive and obscured the neoplastic invasion in some areas. The testis parenchyma did not demonstrate any macroscopic abnormality. Multiple paraffin-embedded sections stained with hematoxylin-eosin and with immunohistochemical stains for PLAP, CAM 5.2, MNF 116, and CD30 were negative for neoplasia. Marked atrophy of the spermatic epithelium was observed. The epididymis was free of neoplastic lesions. The patient received four cycles of chemotherapy (bleomycin, cis-platinum, and etoposide); each cycle lasted 3 days and was repeated every 20 days. The para-aortic mass regressed markedly to 1.5 ⫻ 1.5 cm, and there was no evidence of any other metastatic lesion. A complete retroperitoneal UROLOGY 55 (3), 2000
COMMENT Lesauvage recorded the first documented case of spermatic cord tumor in 1845. Since then, several reports have demonstrated a variety of tumors arising in the spermatic cord,3–7 such as adenocarcinomas, teratomas, seminomas, fibrosarcomas, paragangliomas, and rhabdomyosarcomas. In 1974, Leaf et al.1 reported the first case in English published reports of an embryonal cell carcinoma originating in the spermatic cord in a 6-month-old male infant. Germ cells during fetal development may deviate from their gonadal course and become sequestered in several areas of the body. Extragonadal germ cell tumors may be located in several sites of the human body, and they usually develop as the infant becomes older. Until now, they have been found in the sacrococcygeal area, retroperitoneum, anterior mediastinum, vagina, pineal gland, and other unusual sites.8 No report of an anaplastic seminoma coexisting with an embryonal carcinoma in a mixed germ cell tumor in an adult has been made. Our patient was 36 years old, and the first clinical signs of the tumor presented 3 months before the definitive diagnosis. Most patients present with secondary metastatic lesions. Thus, the diagnosis of an extragonadal 436xv
neoplasm of germ cell origin should only be made after a careful and persistent search for an occult gonadal primary tumor. In our patient, a meticulous histologic examination of the ipsilateral testis did not reveal any primary neoplasm. The utility of the fine needle aspiration biopsy in the diagnosis of spermatic cord tumors is not well defined in published reports because of the small number of cases.9 Fine needle aspiration of masses of the spermatic cord has been increasingly used as an alternative to surgical exploration to establish a tissue diagnosis.10 Radical orchiectomy with high ligation of the spermatic cord is the optimal initial treatment for a mass of the spermatic cord. There is no precise knowledge of the benefits of either chemotherapy11,12 or radiotherapy13,14 in patients with tumors of the spermatic cord. The role of chemotherapy in the treatment of spermatic cord tumors is controversial. The main question is whether chemotherapy should be performed before or after the retroperitoneal lymph node dissection. We decided to perform the surgery after the chemotherapy to provide the oncologist with vital information to determine whether the tumor is reduced. REFERENCES 1. Leaf D, Tucker G, and Harrison L: Embryonal cell carcinoma originating in the spermatic cord: case report. J Urol 112: 285–286, 1974. 2. Bracken B, Johnson D, Frazier H, et al: The role of
436xvi
surgery following chemotherapy in stage III germ cell neoplasms. J Urol 129: 39 – 43, 1983. 3. Hinman F, and Gibson E: Tumors of the epididymis, spermatic cord and testicular tunics: a review of the literature and report of three new cases. Arch Surg 8: 100 –105, 1924. 4. Arlen M, Grabstald H, and Whitemore F: Malignant tumors of the spermatic cord. Cancer 23: 525–532, 1969. 5. Young T: Malignant tumors of the spermatic cord. Br J Surg 56: 260 –262, 1969. 6. Bacchi C, Schmidt R, Brandao M, et al: Paraganglioma of the spermatic cord. Report of a case with immunohistochemical and ultrastructural studies. Arch Pathol Lab Med 114: 899 –901, 1990. 7. Nistal M, Fachal C, and Paniagua R: Testicular carcinoma in situ associated with rhabdomyosarcoma of the spermatic cord. J Urol 142: 358 –362, 1989. 8. Meares E, and Briggs E: Occult seminoma of the testis masquerading as primary extragonadal germinal neoplasms. Cancer 30: 300 –306, 1972. 9. Dalla Palma P, and Barbazza R: Well-differentiated liposarcoma of the paratesticular area: report of a case with fine needle aspiration preoperative diagnosis and review of the literature. Diagn Cytopathol 6: 421– 426, 1990. 10. Perez Guillermo M, Thor A, and Lowhagen T: Paratesticular adenomatoid tumors. The cytologic presentation in fine needle aspiration biopsies. Acta Cytol 33: 6 –10, 1989. 11. Lioe TF, and Biggart JD: Tumors of the spermatic cord and paratesticular tissue. A clinicopathological study. Br J Urol 71: 600 – 606, 1993. 12. Merimsky O, Terrier P, Bonvalot S, et al: Spermatic cord sarcoma in adults. Acta Oncol 38: 635– 638, 1999. 13. Fangundes MA, Zietman AL, Althausen AF, et al: The management of spermatic cord sarcoma. Cancer 77: 1873– 1876, 1996. 14. Warde P, Gospodarowicz MK, Panzarella T, et al: Stage I testicular seminoma: results of adjuvant irradiation and surveillance. J Clin Oncol 13: 2255–2262, 1995.
UROLOGY 55 (3), 2000
AN ADULT PATIENT WITH A MIXED GERM CELL TUMOR OF THE SPERMATIC CORD GEORGE ZOGRAFOS, CONSTADINA PAPADIMITRIOU, IIAS KOUERINIS, EVANGELOS MESSARIS, KALLIOPI PETRAKI, AND GEORGE ANDROULAKIS
ABSTRACT Tumors of the spermatic cord are very rare, and approximately one half of all primary spermatic cord tumors are malignant. We report the presentation and treatment of an adult (36-year-old) patient with a mixed germ cell tumor that originated in the spermatic cord. No similar cases of mixed tumors of the spermatic cord in adults have been reported. UROLOGY 55: 436xiv–436xvi, 2000. © 2000, Elsevier Science Inc.
T
he study of extragonadal germ cell tumors has aroused considerable interest in recent decades. Tumors of the spermatic cord are very rare. Approximately one half of all primary spermatic cord tumors are malignant. We report a case of mixed germ cell tumor originating in the spermatic cord in an adult patient. In 1974, Leaf et al.1 reported the first case in English published reports of an embryonal cell carcinoma in the spermatic cord in an infant. To our knowledge, our case is the first to be reported in an adult patient. CASE REPORT A 36-year-old male patient presented with a 3-month history of diffuse discomfort in the left iliac fossa. He had a palpable painful mass in the left groin, which had grown up to the previous 2 days, with a temperature up to 38°C. His past history, including an undescended testis, was negative. On physical examination, no evidence of an acute abdomen was found, except for the painful hard mass that did not move when the patient was coughing. Both testes were normal to palpation, except for a small hydrocele in the left scrotum. Laboratory tests were normal, except for the white blood cell count, which was 15,000/dL, with From the First Department of Propaedeutic Surgery, University of Athens, Hippocration Hospital, and Department of Pathology, Hippocration Hospital, Athens, Greece Address for correspondence: George Zografos, M.D., Alopekis 22, Athens 106 75, Greece Submitted: June 9, 1999, accepted (with revisions): October 18, 1999
436xiv
© 2000, ELSEVIER SCIENCE INC. ALL RIGHTS RESERVED
a neutrophilic type. The chest x-ray and the abdominal x-ray in the erect position did not show any specific sign. Both testes were normal by ultrasound. The computed tomography scan revealed a solid mass, 3 ⫻ 4 cm in diameter, located at the left deep inguinal ring (Fig. 1). In addition, lymph node masses, 3 ⫻ 3 cm in size, were found at the paraaortic region, below the origin of the renal vein, laterally (Fig. 2). Fine needle aspiration biopsy of the groin mass was performed twice, and histologic evaluation was made by two independent pathologists. Both evaluations revealed a malignant tumor of mesenchymal origin. Human chorionic gonadotropin, embryonic globulin, alpha-1-fetoprotein, and carcinoembryonic antigen levels were within normal limits. At surgery, a tumor of the spermatic cord at the deep inguinal ring was found. The testis was mobilized and was normal, with a small hydrocele. A left orchiectomy with very high ligation and removal of the spermatic cord en bloc was done, and a well-circumscribed tumor (3.5 ⫻ 1.2 cm) of the distal spermatic cord was found. The tumor was located approximately 20 cm from the superior pole of the testis. It was not encapsulated and had a uniform, yellowish, solid, and partially nodular appearance on the cut surface. The tumor was mostly necrotic. The histologic picture was of a mixed germ cell tumor that consisted of an anaplastic seminoma coexisting with large areas of embryonal cell carcinoma (approximately 50% of the tumor). The seminomatous cells were large and irregular, with hyperchromatic nuclei, considerable mitotic activity (four or more mitoses per high-power field) and showed positive immunoreactivity for placental alkaline phospha0090-4295/00/$20.00 PII S0090-4295(99)00545-2
FIGURE 1. Computed tomography scan revealing a solid mass, 3 ⫻ 4 cm in diameter, located at the left deep inguinal ring.
FIGURE 3. Area of embryonal cell carcinoma with solid growth pattern and some embryoid-like bodies. Hematoxylin-eosin stain, original magnification ⫻100.
lymphadenectomy, from the bifurcation of the aorta to the common iliac arteries to the origin of the renal vein, including the residual mass, was done.2 A necrotic mass lesion was removed, and pathologic evaluation revealed a completely necrotic tumor with no active malignant tissue in the mass or in the excised nodes. One month later, the patient underwent radiotherapy at a dose of 3000 cGy directed to the area previously occupied by the residual mass. FIGURE 2. Para-aortic lymph node mass below the origin of the renal vein, laterally.
tase (PLAP). The embryonal cell carcinoma exhibited a solid growth pattern, contained some embryoid-like bodies, and was immunoreactive for CAM 5.2, CD30, and MNF 116 (Fig. 3). Additional features were the presence of a small number of syncytiotrophoblastic cells and a granulomatous reaction containing epithelioid cells, which was extensive and obscured the neoplastic invasion in some areas. The testis parenchyma did not demonstrate any macroscopic abnormality. Multiple paraffin-embedded sections stained with hematoxylin-eosin and with immunohistochemical stains for PLAP, CAM 5.2, MNF 116, and CD30 were negative for neoplasia. Marked atrophy of the spermatic epithelium was observed. The epididymis was free of neoplastic lesions. The patient received four cycles of chemotherapy (bleomycin, cis-platinum, and etoposide); each cycle lasted 3 days and was repeated every 20 days. The para-aortic mass regressed markedly to 1.5 ⫻ 1.5 cm, and there was no evidence of any other metastatic lesion. A complete retroperitoneal UROLOGY 55 (3), 2000
COMMENT Lesauvage recorded the first documented case of spermatic cord tumor in 1845. Since then, several reports have demonstrated a variety of tumors arising in the spermatic cord,3–7 such as adenocarcinomas, teratomas, seminomas, fibrosarcomas, paragangliomas, and rhabdomyosarcomas. In 1974, Leaf et al.1 reported the first case in English published reports of an embryonal cell carcinoma originating in the spermatic cord in a 6-month-old male infant. Germ cells during fetal development may deviate from their gonadal course and become sequestered in several areas of the body. Extragonadal germ cell tumors may be located in several sites of the human body, and they usually develop as the infant becomes older. Until now, they have been found in the sacrococcygeal area, retroperitoneum, anterior mediastinum, vagina, pineal gland, and other unusual sites.8 No report of an anaplastic seminoma coexisting with an embryonal carcinoma in a mixed germ cell tumor in an adult has been made. Our patient was 36 years old, and the first clinical signs of the tumor presented 3 months before the definitive diagnosis. Most patients present with secondary metastatic lesions. Thus, the diagnosis of an extragonadal 436xv
neoplasm of germ cell origin should only be made after a careful and persistent search for an occult gonadal primary tumor. In our patient, a meticulous histologic examination of the ipsilateral testis did not reveal any primary neoplasm. The utility of the fine needle aspiration biopsy in the diagnosis of spermatic cord tumors is not well defined in published reports because of the small number of cases.9 Fine needle aspiration of masses of the spermatic cord has been increasingly used as an alternative to surgical exploration to establish a tissue diagnosis.10 Radical orchiectomy with high ligation of the spermatic cord is the optimal initial treatment for a mass of the spermatic cord. There is no precise knowledge of the benefits of either chemotherapy11,12 or radiotherapy13,14 in patients with tumors of the spermatic cord. The role of chemotherapy in the treatment of spermatic cord tumors is controversial. The main question is whether chemotherapy should be performed before or after the retroperitoneal lymph node dissection. We decided to perform the surgery after the chemotherapy to provide the oncologist with vital information to determine whether the tumor is reduced. REFERENCES 1. Leaf D, Tucker G, and Harrison L: Embryonal cell carcinoma originating in the spermatic cord: case report. J Urol 112: 285–286, 1974. 2. Bracken B, Johnson D, Frazier H, et al: The role of
436xvi
surgery following chemotherapy in stage III germ cell neoplasms. J Urol 129: 39 – 43, 1983. 3. Hinman F, and Gibson E: Tumors of the epididymis, spermatic cord and testicular tunics: a review of the literature and report of three new cases. Arch Surg 8: 100 –105, 1924. 4. Arlen M, Grabstald H, and Whitemore F: Malignant tumors of the spermatic cord. Cancer 23: 525–532, 1969. 5. Young T: Malignant tumors of the spermatic cord. Br J Surg 56: 260 –262, 1969. 6. Bacchi C, Schmidt R, Brandao M, et al: Paraganglioma of the spermatic cord. Report of a case with immunohistochemical and ultrastructural studies. Arch Pathol Lab Med 114: 899 –901, 1990. 7. Nistal M, Fachal C, and Paniagua R: Testicular carcinoma in situ associated with rhabdomyosarcoma of the spermatic cord. J Urol 142: 358 –362, 1989. 8. Meares E, and Briggs E: Occult seminoma of the testis masquerading as primary extragonadal germinal neoplasms. Cancer 30: 300 –306, 1972. 9. Dalla Palma P, and Barbazza R: Well-differentiated liposarcoma of the paratesticular area: report of a case with fine needle aspiration preoperative diagnosis and review of the literature. Diagn Cytopathol 6: 421– 426, 1990. 10. Perez Guillermo M, Thor A, and Lowhagen T: Paratesticular adenomatoid tumors. The cytologic presentation in fine needle aspiration biopsies. Acta Cytol 33: 6 –10, 1989. 11. Lioe TF, and Biggart JD: Tumors of the spermatic cord and paratesticular tissue. A clinicopathological study. Br J Urol 71: 600 – 606, 1993. 12. Merimsky O, Terrier P, Bonvalot S, et al: Spermatic cord sarcoma in adults. Acta Oncol 38: 635– 638, 1999. 13. Fangundes MA, Zietman AL, Althausen AF, et al: The management of spermatic cord sarcoma. Cancer 77: 1873– 1876, 1996. 14. Warde P, Gospodarowicz MK, Panzarella T, et al: Stage I testicular seminoma: results of adjuvant irradiation and surveillance. J Clin Oncol 13: 2255–2262, 1995.
UROLOGY 55 (3), 2000