Yolk Sac Tumor of Vagina
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《美国医学杂志》
Department of Pediatric Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
Abstract
Yolk sac tumors are common in children. By virtue of being chemosensitive, they are amenable to cure by chemotherapy alone and radical surgery is often not required. Yolk sac tumors occurring in the vagina are rare and thus may not be recognized early or may be inadvertently subjected to radical surgery. The authors report a case that presented to them after radical surgery with elevated Alpha-fetoprotein level is reported. The management of this case and review of the relevant literature are discussed here.
Keywords: Yolk sac tumor; Vagina
Case report
Case 1
An 11-month-old child (BR 21516), born of non-consanguineous marriage presented with history of vaginum of 3-month duration. She had developed urinary retention 20 days before she was taken to a local hospital where she was investigated. An abdominopelvic ultrasound revealed a mass measuring 6 cm × 4 cm × 3 cm in the vagina with elevation of the bladder base causing obstructive uropathy. She underwent a total abdominal hysterectomy with bilateral salingo-oophorectomy. Histopathological examination of the specimen revealed a yolk sac tumor of the vagina. The child was referred to the Tata Memorial Hospital for further management. A CT scan could not be done, but postoperative ultrasound did not reveal any residual mass. Preoperative Alpha-fetoprotein level was not available; the pre-operative level done 15 days later was 355 ng/ml (normal- 0.35 ng/ml). The histology was confirmed to be yolk sac tumor. Chest X-ray was normal.
The child received BEP regimen (bleomycin, etoposide and cisplatin) in the recommended doses and alpha-fetopeotein level was monitored with each cycle. After two such cycle the alpha-fetopeotein level was 3.1 ng/ml and a CT of the abdomen revealed a small mass measuring 1.1cm × 1cm in the region of the vaginal vault on the right side. There was no involvement of the urinary bladder and rectum. Other organs were normal. The child received two more cycles of etoposide and cisplatin. A CT scan at the end of 4 cycles of chemotherapy revealed the mass at the vaginal vault unchanged as compared to pervious scan with no abnormality in the rest of the abdomino-pelvic cavity.
The child has been kept under close observation and will be followed up every 3 months with alpha-fetoprotein level estimation and CT scans when necessary.
Case 2
A 1-year-old child (BS 6689), born of a non-consanguineous marriage presented with the history of bleeding per vaginum since one month. The child was investigated at a local hospital and was found to have tumor in the vagina. The alpha-fetoprotein level done outside before surgery was 19.4 ng/ml. A vaginectomy was planned, but the mass was large and was adherent to the lateral wall of the vagina and hence a total abdominal hysterectomy with the bilateral salpingo-oophorectomy was done. A histopathological examination of the specimen revealed a yolk sac tumor of the vagina. The child was then referred to the Tata Memorial Hospital for further management. The postoperative alpha-fetoprotein level done 12 days later was 249ng/ml.
A CT scan done 14 days later revealed an ill-defined enhancing area in the rectovesical pouch abutting the rectum. Her chest radiograph was normal. The child received BEP regimen (leomycin, etoposide and ciplatin) in the recommended dose. Alpha-fetoprotein level was monitored with each cycle. After two cycles, the level was 8ng/ml. The patient received two more cycles of the same protocol. The alpha-fetoprotein level done at the end of chemotherapy was 2.2 ng/ ml. A CT scan of the abdomen and pelvis at the end of therapy was normal with no residual tumor. The child has been kept under close observation and will be followed up every 3 months with an alpha-fetoprotein level and CT scan when necessary.
Discussion
Yolk sac tumor is the commonest germ cell tumor in children. [1],[2] It may occure as pure form or as a part of a mixed germ cell tumor. [2] Previously, the tumor was more often diagnosed as a clear cell carcinoma. The first demarcation was made by Gunnar Teilum et al [5] and since then this entity has been well established. The tumor was also called 'mesonephroma ovarri' due to the finding of glomeruli like structure - the Schiller Duval bodies. There are actually blood vessels surrounded by primordial germ cells, a finding first noted in rat endoderm.[5]
Yolk sac tumor of the vagina is a rare entity and seen almost exclusively in children less than 3 years of age.[4],[6] The presenting feature is usually frank bleeding per vaginum or blood stained vaginal discharge. Occasionally the child may develop urinary retention due to the tumor mechanically compressing the urethra. In more advanced cases, a mass may be seen protruding through the vaginal orifice. In the present study, the patients presented with bleeding per vaginum and the first patient also had signs and symptoms suggestive of pressure on the urethra.
A vaginal examination under anesthesia is the best way to diagnose a yolk sac tumor of the vagina.[6] Sarcoma botyroids - a variant of embryonal rhabdomyosarcoma is the most common differential diagnosis. It is a common vaginal tumor of infantcy and presents with vaginal bleeding and a mass. However, it has a characteristic grape like appearance and there are edematous and cellular areas formed of immature skeletal muscle cells which help in distinguishing it from a yolk sac tumor. Formerly yolk sac tumor of the vagina was confused with a clear cell carcinoma of the vagina. However, the former has always occurred in children less than 3 years of age and later has not been reported at that young age. The histopathological examination confirms the differentiation between the two. Alpha-fetoprotein level in blood is usually elevated at presentation and should be used as guide to monitor therapy as well as detect a recurrence. The alpha-fetoprotein level was raised in both the patients and returned to normal after therapy.
Radical surgery was standard of care earlier but was associated with significant surgical morbidity and relapses were common. Young and Scully's report of nine cases of yolk sac tumor of vagina suggested the role of chemotherapy for the first time.[6] Three patients underwent radical surgery alone without any chemotherapy. All three patients were dead at 2 years of diagnosis due to the disease. In contrast, six patients who received chemotherapy either before of after surgery were alive and disease free for 2 to 9 years after diagnosis. Anderson et al [7] reviewed the cases of yolk sac tumor of vagina treated from 1970. Sixteen cases who received local therapy alone, only four were alive and disease free at 2 years. As opposed to this 21out of the 22 patients treated with chemotherapy were alive and disease free at 2 years. Thus the prognosis has changed significantly due to the effective chemotherapy, which reduces the tumor burden and the extent of the surgical resection/ Platinum based combination chemotherapy gives excellent remission rates because of which, radical surgery is often not required. Induction chemotherapy followed by conservative surgery and adjuvant chemotherapy is an effective approach, which achieves long-term survival and organ preservation. Unfortunately in the present study the patients underwent radical surgery before coming to Tata Memorial Hospital.
An alpha-fetoprotein level estimation is mandatory in all young girls presenting with bleeding per vaginum or mass protruding through the vagina. If alpha-fetoprotein level is normal, an examination under anesthesia with a biopsy must be performed to establish the diagnosis and exclude other entities. This approach would obviate the need for extensive and unnecessary surgery.
Lastly, all those young girls who have undergone total abdominal hysterectomy with salpingo-oophorectomy as treatment for yolk sac tumor of vagina should be followed up and appropriate hormone replacement must be considered at an appropriate age.
References
1. Hawkins EP, Finegold MJ, Hawkins HK et al. Nongerminomtous malignancies malignant germ cell tumors in children - a review of 89 cases from the pediatric oncology group, 1971-1984. Cancer 1986; 58: 2579
2. Edith P Hawkins. Pathology of germ cell tumors in children. Critical Reviews in Hematology/ Oncology 1990; 10(2): 165
3. Kurman RJ, Norris HJ. Endodermal sinus tumor of the ovary. A Clinical and pathological analysis of 71 cases. Cancer 1979; 38: 2404-2419
4. Copeland LJ, Sneige N, Ordonez NG et al. Endodermal sinus tumor of the vagina and cervix. Cancer 1985; 55: 2558
5. Teilum G. Endrodermal Sinus tumor of the ovary and testis. Comparative Morphogenesis of the so-called mesonephroma ovarii (Schiller) and extraembryonic (yolk sac-allantoic) structures of the rat's placenta. Cancer 1959; 12: 1092-1911
6. Anderson WA, Sabio H, Durso N et al . Endodermal sinus tumor of vagina - the role of primary chemotherapy. Cancer 1985; 56: 1025
7. Christine Mauz Korholz, Dieter Horms, Gabriele Calaminus et al for the Maligne Keimzltumoren Study Group: Primary Chemotherapy and conservative surgery for vaginal yolk sac tumor. Lancet 2000; 355: 625(Deshmukh C, Bakshi A, Bha)
Abstract
Yolk sac tumors are common in children. By virtue of being chemosensitive, they are amenable to cure by chemotherapy alone and radical surgery is often not required. Yolk sac tumors occurring in the vagina are rare and thus may not be recognized early or may be inadvertently subjected to radical surgery. The authors report a case that presented to them after radical surgery with elevated Alpha-fetoprotein level is reported. The management of this case and review of the relevant literature are discussed here.
Keywords: Yolk sac tumor; Vagina
Case report
Case 1
An 11-month-old child (BR 21516), born of non-consanguineous marriage presented with history of vaginum of 3-month duration. She had developed urinary retention 20 days before she was taken to a local hospital where she was investigated. An abdominopelvic ultrasound revealed a mass measuring 6 cm × 4 cm × 3 cm in the vagina with elevation of the bladder base causing obstructive uropathy. She underwent a total abdominal hysterectomy with bilateral salingo-oophorectomy. Histopathological examination of the specimen revealed a yolk sac tumor of the vagina. The child was referred to the Tata Memorial Hospital for further management. A CT scan could not be done, but postoperative ultrasound did not reveal any residual mass. Preoperative Alpha-fetoprotein level was not available; the pre-operative level done 15 days later was 355 ng/ml (normal- 0.35 ng/ml). The histology was confirmed to be yolk sac tumor. Chest X-ray was normal.
The child received BEP regimen (bleomycin, etoposide and cisplatin) in the recommended doses and alpha-fetopeotein level was monitored with each cycle. After two such cycle the alpha-fetopeotein level was 3.1 ng/ml and a CT of the abdomen revealed a small mass measuring 1.1cm × 1cm in the region of the vaginal vault on the right side. There was no involvement of the urinary bladder and rectum. Other organs were normal. The child received two more cycles of etoposide and cisplatin. A CT scan at the end of 4 cycles of chemotherapy revealed the mass at the vaginal vault unchanged as compared to pervious scan with no abnormality in the rest of the abdomino-pelvic cavity.
The child has been kept under close observation and will be followed up every 3 months with alpha-fetoprotein level estimation and CT scans when necessary.
Case 2
A 1-year-old child (BS 6689), born of a non-consanguineous marriage presented with the history of bleeding per vaginum since one month. The child was investigated at a local hospital and was found to have tumor in the vagina. The alpha-fetoprotein level done outside before surgery was 19.4 ng/ml. A vaginectomy was planned, but the mass was large and was adherent to the lateral wall of the vagina and hence a total abdominal hysterectomy with the bilateral salpingo-oophorectomy was done. A histopathological examination of the specimen revealed a yolk sac tumor of the vagina. The child was then referred to the Tata Memorial Hospital for further management. The postoperative alpha-fetoprotein level done 12 days later was 249ng/ml.
A CT scan done 14 days later revealed an ill-defined enhancing area in the rectovesical pouch abutting the rectum. Her chest radiograph was normal. The child received BEP regimen (leomycin, etoposide and ciplatin) in the recommended dose. Alpha-fetoprotein level was monitored with each cycle. After two cycles, the level was 8ng/ml. The patient received two more cycles of the same protocol. The alpha-fetoprotein level done at the end of chemotherapy was 2.2 ng/ ml. A CT scan of the abdomen and pelvis at the end of therapy was normal with no residual tumor. The child has been kept under close observation and will be followed up every 3 months with an alpha-fetoprotein level and CT scan when necessary.
Discussion
Yolk sac tumor is the commonest germ cell tumor in children. [1],[2] It may occure as pure form or as a part of a mixed germ cell tumor. [2] Previously, the tumor was more often diagnosed as a clear cell carcinoma. The first demarcation was made by Gunnar Teilum et al [5] and since then this entity has been well established. The tumor was also called 'mesonephroma ovarri' due to the finding of glomeruli like structure - the Schiller Duval bodies. There are actually blood vessels surrounded by primordial germ cells, a finding first noted in rat endoderm.[5]
Yolk sac tumor of the vagina is a rare entity and seen almost exclusively in children less than 3 years of age.[4],[6] The presenting feature is usually frank bleeding per vaginum or blood stained vaginal discharge. Occasionally the child may develop urinary retention due to the tumor mechanically compressing the urethra. In more advanced cases, a mass may be seen protruding through the vaginal orifice. In the present study, the patients presented with bleeding per vaginum and the first patient also had signs and symptoms suggestive of pressure on the urethra.
A vaginal examination under anesthesia is the best way to diagnose a yolk sac tumor of the vagina.[6] Sarcoma botyroids - a variant of embryonal rhabdomyosarcoma is the most common differential diagnosis. It is a common vaginal tumor of infantcy and presents with vaginal bleeding and a mass. However, it has a characteristic grape like appearance and there are edematous and cellular areas formed of immature skeletal muscle cells which help in distinguishing it from a yolk sac tumor. Formerly yolk sac tumor of the vagina was confused with a clear cell carcinoma of the vagina. However, the former has always occurred in children less than 3 years of age and later has not been reported at that young age. The histopathological examination confirms the differentiation between the two. Alpha-fetoprotein level in blood is usually elevated at presentation and should be used as guide to monitor therapy as well as detect a recurrence. The alpha-fetoprotein level was raised in both the patients and returned to normal after therapy.
Radical surgery was standard of care earlier but was associated with significant surgical morbidity and relapses were common. Young and Scully's report of nine cases of yolk sac tumor of vagina suggested the role of chemotherapy for the first time.[6] Three patients underwent radical surgery alone without any chemotherapy. All three patients were dead at 2 years of diagnosis due to the disease. In contrast, six patients who received chemotherapy either before of after surgery were alive and disease free for 2 to 9 years after diagnosis. Anderson et al [7] reviewed the cases of yolk sac tumor of vagina treated from 1970. Sixteen cases who received local therapy alone, only four were alive and disease free at 2 years. As opposed to this 21out of the 22 patients treated with chemotherapy were alive and disease free at 2 years. Thus the prognosis has changed significantly due to the effective chemotherapy, which reduces the tumor burden and the extent of the surgical resection/ Platinum based combination chemotherapy gives excellent remission rates because of which, radical surgery is often not required. Induction chemotherapy followed by conservative surgery and adjuvant chemotherapy is an effective approach, which achieves long-term survival and organ preservation. Unfortunately in the present study the patients underwent radical surgery before coming to Tata Memorial Hospital.
An alpha-fetoprotein level estimation is mandatory in all young girls presenting with bleeding per vaginum or mass protruding through the vagina. If alpha-fetoprotein level is normal, an examination under anesthesia with a biopsy must be performed to establish the diagnosis and exclude other entities. This approach would obviate the need for extensive and unnecessary surgery.
Lastly, all those young girls who have undergone total abdominal hysterectomy with salpingo-oophorectomy as treatment for yolk sac tumor of vagina should be followed up and appropriate hormone replacement must be considered at an appropriate age.
References
1. Hawkins EP, Finegold MJ, Hawkins HK et al. Nongerminomtous malignancies malignant germ cell tumors in children - a review of 89 cases from the pediatric oncology group, 1971-1984. Cancer 1986; 58: 2579
2. Edith P Hawkins. Pathology of germ cell tumors in children. Critical Reviews in Hematology/ Oncology 1990; 10(2): 165
3. Kurman RJ, Norris HJ. Endodermal sinus tumor of the ovary. A Clinical and pathological analysis of 71 cases. Cancer 1979; 38: 2404-2419
4. Copeland LJ, Sneige N, Ordonez NG et al. Endodermal sinus tumor of the vagina and cervix. Cancer 1985; 55: 2558
5. Teilum G. Endrodermal Sinus tumor of the ovary and testis. Comparative Morphogenesis of the so-called mesonephroma ovarii (Schiller) and extraembryonic (yolk sac-allantoic) structures of the rat's placenta. Cancer 1959; 12: 1092-1911
6. Anderson WA, Sabio H, Durso N et al . Endodermal sinus tumor of vagina - the role of primary chemotherapy. Cancer 1985; 56: 1025
7. Christine Mauz Korholz, Dieter Horms, Gabriele Calaminus et al for the Maligne Keimzltumoren Study Group: Primary Chemotherapy and conservative surgery for vaginal yolk sac tumor. Lancet 2000; 355: 625(Deshmukh C, Bakshi A, Bha)