Vaginal endodermal sinus tumor
http://www.100md.com
《美国医学杂志》
1 Departments of Pediatric Surgery, Kasturba Hospital, Kasturba Medical College Manipal, Karnataka, India
2 Departments of Pediatrics, Kasturba Hospital, Kasturba Medical College Manipal, Karnataka, India
Abstract
Malignant germ cell tumors are rare tumors of childhood accounting less than 3% of pediatric malignancies, and endodermal sinus tumor (EST) is the most common histological subtype. The vagina is an extremely rare site for germ cell tumors (GCT). A one-year female was admitted with history of bleeding per vagina. She had pallor and a mass was palpable anteriorly on rectal examination. Magnetic Resonance Imaging (MRI) showed a tumor mass at the junction of cervix and vagina and biopsy was suggestive of a vaginal EST. The serum alpha fetoprotein (AFP) was elevated. She underwent vaginohysterectomy. The tumor was excised in toto and patient received four courses of cisplatin, etoposide and bleomycin. At one year of follow up, patient was symptom free and serum AFP remained at normal level. EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age. It is both locally aggressive and capable of metastasis. Even though more conservative surgery is advised to maintain sexual and reproductive function, at times, radical surgery is mandatory depending on the infiltration of the tumor to the surrounding structures. Simple tumor excision is not sufficient, as residual cells induce recurrence and make chemotherapy ineffective. The serum AFP level is a useful marker for diagnosis and monitoring the recurrence of vaginal EST in infants.
Keywords: Pediatric germ cell tumor; Vaginal endodermal sinus tumor
Malignant germ cell tumors account less than 3% of all cancers in children.[1] Of the different histological subtypes, endodermal sinus tumor (EST) is the most common in the pediatric population.[2] The common primary sites are ovary, testis and sacrococcygeal region.[3] We report an EST of the vagina, an extremely rare pediatric malignancy primarily affecting infants.[4]
Case Report
A one-year-old female was admitted with a history of bleeding per vagina of 1-month duration. The child was apparently healthy before the onset of bleeding.
On examination the baby had pallor. Rectal examination revealed a mass situated anterior to the rectum. Ultrasound showed a 4x3x3.5 cm soft tissue echogenic lesion posterior to the bladder. A fine needle aspiration biopsy of the tumor was done per rectally. Mass showed tumor tissue arranged in a loose reticulum and cystic spaces, with tumor cells disposed in varying patterns- solid sheets, trabeculae, glandular arrangement and microcystic areas. Schiller-Duval Bodies More Details were identified and a diagnosis of vaginal EST was made. Magnetic Resonance Imaging (MRI) showed the extension of tumor into cervix and abutting the bladder anteriorly and rectum posteriorly with no loss of fat plane between them and the tumor mass. There was no calcification or evidence of any lymphadenopathy. Serum alpha-fetoprotein (AFP) was elevated at 9331 ng/ml. Bone marrow biopsy was normal and bone scan did not show any evidence of bony spread.
The patient was taken up for surgery and a vaginohysterectomy was done. The tumor was seen to arise from the lateral wall of the vagina infiltrating the cervix. There was no infiltration into posterior wall of the bladder and anterior wall of the rectum. The serum AFP levels returned to normal 4 weeks after surgery. Patient received four courses of PEB (cisplatinum, etoposide and bleomycin) regimen. There is no evidence of recurrence at one year of follow-up.
Discussion
EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age.[4] Literature search revealed only about 50 cases reported so far.
The histogenesis of this rare tumor is uncertain. As in the case of other primary extra gonadal germ cell tumors, the aberrant migration of germ cells during early embryonic life from the yolk sac to the gonadal ridges remains a distinct possibility.[5]
The clinical presentation of this tumor includes a history of bloody or blood tinged vaginal discharge, sometimes accompanied by a polypoid mass protruding from the vagina.
Imaging by ultrasonogram, CT or MRI may demonstrate tumor extension into surrounding structures. However, ultrasonogram may be associated with unacceptable false-negative results.[6] The histologic diagnosis of EST is based on the finding of Schiller-Duval bodies, as was demonstrated in our case. Assay of serum AFP can potentially aid in the preoperative diagnosis, monitor effectiveness of therapy, and detect recurrences before clinical manifestations.[7]
Because of the rarity of vaginal EST, its natural history is poorly understood and ideal management is not obvious. It is both locally aggressive and capable of metastasis via hematogenous and lymphatic pathways. Different modalities of treatment for vaginal EST, including surgery, radiation and chemotherapy, have had varying degree of success.[6] In the past, extent of surgery varied from pelvic exenteration to sample excision with preservation of child bearing potential. Vawter[8] introduced chemotherapy for EST. EST is chemosensitive and various regimens currently recommended for treatment are - PEB (cisplatin, etoposide, bleomycin), PVB (cisplatin, vinblastin, bleomycin) and JEB (carboplatin, etoposide, bleomycin).[9] Even though more conservative surgery is advised to maintain sexual and reproductive function,[6] at times radical surgery is mandatory depending upon the infiltration of the tumor to the surrounding structures.[10] Long-term remission can be achieved in at least 60% of the cases.
The patient reported here underwent vaginohysterectomy and received four cycles of combination chemotherapy with PEB.
To conclude, we emphasize on the need for complete evaluation of infants presenting with painless vaginal bleeding. As the source of blood found in diapers of young girls without overt cause is uncertain, one should maintain a high index of suspicion for any potential underlying pathological condition. Apart from radiological investigations as diagnostic tools, one might consider measuring AFP in such patients.
References
1. Rescoria FJ. Germ cell tumors. Semin Pediatr Surg 1997; 6: 29-37.
2. Davidoff AM, Hebra A, Bunin N, S hochat SJ, Schnaufer L. Endodermal sinus tumor in children. J Pediatr Surg 1996; 31: 1075- 1078.
3. Giller, RH. Germ cell tumors. I. Tumor biology and classification. In C Pochedly, ed. Neoplastic Diseases of Childhood. New York; Harwood Academic Publications, 1994; 43: 827-847.
4. Hwang EH, Han SJ, Lee MK. 1996. Clinical experience with conservative surgery for vaginal endodermal sinus tumor. J Pediatr Surg 1996; 31: 219-222.
5. U Chatterjee et al. Endodermal sinus tumor of vagina. J Indian Assoc Pediatr Surg 2003; 8: 235-238.
6. Liann NH, Stephen MS, Roger HG, Brian SG, Mark AL, Martin AK, New Perspectives on therapy for vaginal endodermal sinus tumors. J Urology 2002; 168: 687-690.
7. Talerman A, Haije WG, Baggerman L. Serum alfafetoprotein (AFP) in the diagnosis and management of endodermal(yolk sac) tumor and mixed germ cell tumor of the ovary. Cancer 1978; 41: 272-278.
8. Vawter GF. Carcinoma of the vagina in infancy. Cancer 1965. 18: 1479-1484.
9. Castleberry RP, Cushing B, Perlman E, Hawkins EP. Germ cell tumors. In Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology, 3rd edn. Philadelphia; Lippincott-Raven Publishers, 1997; 921-945.
10. Larry J. Copeland, Nour S, Nelson GO, Kenneth CH, David MG, Patton BS, John JK. Endodermal sinus tumor of the vagina and cervix. Cancer June 1985; 2558-2565.(Kumar Vijay, Kini Pushpa,)
2 Departments of Pediatrics, Kasturba Hospital, Kasturba Medical College Manipal, Karnataka, India
Abstract
Malignant germ cell tumors are rare tumors of childhood accounting less than 3% of pediatric malignancies, and endodermal sinus tumor (EST) is the most common histological subtype. The vagina is an extremely rare site for germ cell tumors (GCT). A one-year female was admitted with history of bleeding per vagina. She had pallor and a mass was palpable anteriorly on rectal examination. Magnetic Resonance Imaging (MRI) showed a tumor mass at the junction of cervix and vagina and biopsy was suggestive of a vaginal EST. The serum alpha fetoprotein (AFP) was elevated. She underwent vaginohysterectomy. The tumor was excised in toto and patient received four courses of cisplatin, etoposide and bleomycin. At one year of follow up, patient was symptom free and serum AFP remained at normal level. EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age. It is both locally aggressive and capable of metastasis. Even though more conservative surgery is advised to maintain sexual and reproductive function, at times, radical surgery is mandatory depending on the infiltration of the tumor to the surrounding structures. Simple tumor excision is not sufficient, as residual cells induce recurrence and make chemotherapy ineffective. The serum AFP level is a useful marker for diagnosis and monitoring the recurrence of vaginal EST in infants.
Keywords: Pediatric germ cell tumor; Vaginal endodermal sinus tumor
Malignant germ cell tumors account less than 3% of all cancers in children.[1] Of the different histological subtypes, endodermal sinus tumor (EST) is the most common in the pediatric population.[2] The common primary sites are ovary, testis and sacrococcygeal region.[3] We report an EST of the vagina, an extremely rare pediatric malignancy primarily affecting infants.[4]
Case Report
A one-year-old female was admitted with a history of bleeding per vagina of 1-month duration. The child was apparently healthy before the onset of bleeding.
On examination the baby had pallor. Rectal examination revealed a mass situated anterior to the rectum. Ultrasound showed a 4x3x3.5 cm soft tissue echogenic lesion posterior to the bladder. A fine needle aspiration biopsy of the tumor was done per rectally. Mass showed tumor tissue arranged in a loose reticulum and cystic spaces, with tumor cells disposed in varying patterns- solid sheets, trabeculae, glandular arrangement and microcystic areas. Schiller-Duval Bodies More Details were identified and a diagnosis of vaginal EST was made. Magnetic Resonance Imaging (MRI) showed the extension of tumor into cervix and abutting the bladder anteriorly and rectum posteriorly with no loss of fat plane between them and the tumor mass. There was no calcification or evidence of any lymphadenopathy. Serum alpha-fetoprotein (AFP) was elevated at 9331 ng/ml. Bone marrow biopsy was normal and bone scan did not show any evidence of bony spread.
The patient was taken up for surgery and a vaginohysterectomy was done. The tumor was seen to arise from the lateral wall of the vagina infiltrating the cervix. There was no infiltration into posterior wall of the bladder and anterior wall of the rectum. The serum AFP levels returned to normal 4 weeks after surgery. Patient received four courses of PEB (cisplatinum, etoposide and bleomycin) regimen. There is no evidence of recurrence at one year of follow-up.
Discussion
EST of the vagina is a rare, highly malignant GCT that exclusively involves children less than 3 years of age.[4] Literature search revealed only about 50 cases reported so far.
The histogenesis of this rare tumor is uncertain. As in the case of other primary extra gonadal germ cell tumors, the aberrant migration of germ cells during early embryonic life from the yolk sac to the gonadal ridges remains a distinct possibility.[5]
The clinical presentation of this tumor includes a history of bloody or blood tinged vaginal discharge, sometimes accompanied by a polypoid mass protruding from the vagina.
Imaging by ultrasonogram, CT or MRI may demonstrate tumor extension into surrounding structures. However, ultrasonogram may be associated with unacceptable false-negative results.[6] The histologic diagnosis of EST is based on the finding of Schiller-Duval bodies, as was demonstrated in our case. Assay of serum AFP can potentially aid in the preoperative diagnosis, monitor effectiveness of therapy, and detect recurrences before clinical manifestations.[7]
Because of the rarity of vaginal EST, its natural history is poorly understood and ideal management is not obvious. It is both locally aggressive and capable of metastasis via hematogenous and lymphatic pathways. Different modalities of treatment for vaginal EST, including surgery, radiation and chemotherapy, have had varying degree of success.[6] In the past, extent of surgery varied from pelvic exenteration to sample excision with preservation of child bearing potential. Vawter[8] introduced chemotherapy for EST. EST is chemosensitive and various regimens currently recommended for treatment are - PEB (cisplatin, etoposide, bleomycin), PVB (cisplatin, vinblastin, bleomycin) and JEB (carboplatin, etoposide, bleomycin).[9] Even though more conservative surgery is advised to maintain sexual and reproductive function,[6] at times radical surgery is mandatory depending upon the infiltration of the tumor to the surrounding structures.[10] Long-term remission can be achieved in at least 60% of the cases.
The patient reported here underwent vaginohysterectomy and received four cycles of combination chemotherapy with PEB.
To conclude, we emphasize on the need for complete evaluation of infants presenting with painless vaginal bleeding. As the source of blood found in diapers of young girls without overt cause is uncertain, one should maintain a high index of suspicion for any potential underlying pathological condition. Apart from radiological investigations as diagnostic tools, one might consider measuring AFP in such patients.
References
1. Rescoria FJ. Germ cell tumors. Semin Pediatr Surg 1997; 6: 29-37.
2. Davidoff AM, Hebra A, Bunin N, S hochat SJ, Schnaufer L. Endodermal sinus tumor in children. J Pediatr Surg 1996; 31: 1075- 1078.
3. Giller, RH. Germ cell tumors. I. Tumor biology and classification. In C Pochedly, ed. Neoplastic Diseases of Childhood. New York; Harwood Academic Publications, 1994; 43: 827-847.
4. Hwang EH, Han SJ, Lee MK. 1996. Clinical experience with conservative surgery for vaginal endodermal sinus tumor. J Pediatr Surg 1996; 31: 219-222.
5. U Chatterjee et al. Endodermal sinus tumor of vagina. J Indian Assoc Pediatr Surg 2003; 8: 235-238.
6. Liann NH, Stephen MS, Roger HG, Brian SG, Mark AL, Martin AK, New Perspectives on therapy for vaginal endodermal sinus tumors. J Urology 2002; 168: 687-690.
7. Talerman A, Haije WG, Baggerman L. Serum alfafetoprotein (AFP) in the diagnosis and management of endodermal(yolk sac) tumor and mixed germ cell tumor of the ovary. Cancer 1978; 41: 272-278.
8. Vawter GF. Carcinoma of the vagina in infancy. Cancer 1965. 18: 1479-1484.
9. Castleberry RP, Cushing B, Perlman E, Hawkins EP. Germ cell tumors. In Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology, 3rd edn. Philadelphia; Lippincott-Raven Publishers, 1997; 921-945.
10. Larry J. Copeland, Nour S, Nelson GO, Kenneth CH, David MG, Patton BS, John JK. Endodermal sinus tumor of the vagina and cervix. Cancer June 1985; 2558-2565.(Kumar Vijay, Kini Pushpa,)