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Congenital vulvar teratoma in a newborn
CASE REPORTS
Congenital Vulvar Teratoma in a Newborn ¨ zbas¸ar, O ¨ . C¸andır, H. Kaya, and F. C¸ag˘layan By M. C¸akmak, C¸. Savas¸, D. O Kırıkkale, Turkey and Isparta, Turkey
The most common site of teratomas in neonates is in the sacrococcygeal region. Herein the authors describe a congenital teratoma, and to their knowledge it is the first reported case of vulvar site in a newborn. Tumor was removed on the fourth day of life, and she was completely normal 18 months after the operation. Because of possible malignancy
or recurrence, complete surgical excision of the tumor and careful follow-up is the treatment of choice. J Pediatr Surg 36:620-621. Copyright © 2001 by W.B. Saunders Company.
T
healthy after evaluation of the other organ systems. Hemogram and biochemistry findings were all within normal limits, except the unconjugated bilirubin level (8.7 mg/dL). Alpha-fetoprotein (AFP) and carcinoembryogenic antigen (CEA) levels also were normal (AFP, 0.02 ng/mL; CEA, 1.38 ng/mL). Plain abdominal and pelvic radiographs in both sides were normal. The patency of the urethra and the bladder capacity were normal in cysto-urethrogram. A computed tomography scan showed a polypoid and pedinculated mass, 5 cm in diameter and in fatty density (⫺40 HU). All the other abdominal and pelvic structures were normal. The tumor was resected on the fourth day of her life, and the space between the clitoris and urethra was approximated, which was the result of resection of the base of the tumor. The recovery was uneventful, and she was discharged at the fifth postoperative day. She was completely normal 18 months after the operation, and the CEA and AFP levels were normal.
HE MOST COMMON site of teratomas in neonates is in the sacrococcygeal and presacral region (47%). The other most common sites are the gonads (36%) and the mediastinum (6%).1 The remaining 11% are distributed in the mesenterium, retroperitoneum, pericardium, spinal canal, cervical, nasopharyngeal and orbital regions, and the neurocranium. In rare cases, hepatic, gastric, anorectal, adrenal, and umbilical cord teratomas also have been reported.1-5 In the current study, we report a congenital vulvar teratoma, which is a very unusual site in a newborn and a unique case appearing in vulva. We also review the literature relevant to teratomas and neonatal tumors of the vulva. CASE REPORT A girl, who is a first child of the family, was delivered vaginally in the 38th week of gestation. Her birth weight was 2,600 g. There was no familial relationship between the parents, and the pregnancy was uneventful. The neonate was transferred to the hospital because of a solid tumor in the vulva on her first day of life. A pedinculated spheric mass 55 ⫻ 45 ⫻ 40 mm in diameter was seen on her physical examination. The mass was soft and covered with skin. It was originating from the vulva with a pedicle, and the base of the mass was just below the clitoris and above the urethral opening. The base of the mass (its pedicle) was 1 cm in diameter. Two reddish openings on the mass were catheterized, and 2 blind sinuses approximately 1 cm in length were determined (Fig 1). All the external genital structures, urethral, and vaginal orifices were completely normal. The baby was found
From the Departments of Pediatric Surgery, Obstetrics and Gynecology, and Pathology, Su¨leyman Demirel University, Medical School, Isparta, Turkey, and the Department of Pediatric Surgery, Kırıkkale University, Medical School, Kırıkkale, Turkey. Address reprint requests to Murat C¸akmak, MD, Barıs¸ Sitesi, 80 Sok. No: 5, Mustafa Kemal Mah, Ankara, Turkey. Copyright © 2001 by W.B. Saunders Company 0022-3468/01/3604-0018$35.00/0 doi:10.1053/jpsu.2001.22302 620
INDEX WORDS: Teratoma, vulva.
Pathology The specimen was fixed in 10% formalin and embedded in paraffin, processed in the usual manner, and stained with H&E. Some sections also were immunostained with monoclonal antibodies to S100 (Dako Corp, Carpinteria, CA) and desmin (Dako Corp).
Macroscopy The mass was soft and largely covered by skin. There were 2 reddish openings that were blind sinuses. On the cut surface, yellow, lobulated adipous tissue, grayish-white areas, and several little cysts were seen.
Microscopy Microscopic examination found a mature teratoma consisting of a lipomalike mature fat tissue with fibrous septae, lymphoid follicles, and cystic areas, which were lined internally with intestinal epitelium changed into stratified squamous epitelium underlying fibrous connective tissue, which contained skin adnex, peripheral nerves, islands of fat, and smooth muscle. There were normal fibrous tissue of the resection margins.
DISCUSSION
Teratomas are embryonal neoplasms consisting of tissues of the 3 germ layers.4 Excluding testicular teratomas, 75% of these tumors occur in girls, and approximately 80% are benign. Teratomas occuring in infancy Journal of Pediatric Surgery, Vol 36, No 4 (April), 2001: pp 620-621
VULVAR TERATOMA
621
Fig 1. A pedinculated spheric mass 55 ⴛ 45 ⴛ 40 mm in diameter and 2 reddish openings.
and early childhood usually are extragonadal, whereas older children predominantly present with gonadal teratomas.6 Although it has been reported that mature teratomas were the most common tumors among the neonatal solid tumors, teratomas are tumors that rarely exist in children.7 The most common site for a neonatal teratoma is the sacrococcygeal region. Their location in vulva, however, is an unusual finding.1 Congenital tumors of the vulva are rare conditions. Some benign or malignant tumors including benign hemangiopericytoma, neurofibroma, malignant schwannoma, rhabdomyosarcoma, and primary endodermal sinus tumor also should be considered in addition to the teratoma.8-10 Levard et al11 reported a case of congenital neoplasm of the clitoris, which was a choristoma. This was an aberrant rest or heterotopic tissue, located at the tip of the
clitoris, which contained intestinal tissue.11 The investigators explained that the urogenital membrane at the origin of the vulvar mucosa derives from endodermlike intestine. Choristoma is defined as microscopically normal cells or tissues that are present in abnormal locations as a result of embryologic developmental anomaly, whereas teratoma contains tissues of the 3 germ layers.12 Although there are different widely accepted theories about the histogenesis of teratomas, one is the germ cell theory. Because primitive germ cells possess all the DNA for any type of cell, they have the ability to form the tissues seen in teratomas. The migration pattern of these cells also explains the gonadal, midline, and paramedian locations of the teratomas.1 Jona3 reported the first case of congenital anorectal teratoma that originated in the rectal wall, which had no connection with the coccyx and presented as a prolapsing posterior perineal mass at birth. It contained 2 sinuses 1 and 2 cm in depth, and pathologic diagnosis was benign mature teratoma that contained intestinal tissue, like our case.3 There were 2 recurrences at 9 and 12 months in this case, which required reexcision. Congenital teratomas of the vulva are rare conditions seen in the newborn. Some other benign or malignant tumors of the vulva should also be considered. Because of possible malignancy or recurrence, complete resection of the tumor is the treatment of choice. Long-term follow-up and control of the patient by physical examination, tumor markers, and by radiologic studies must be done after the operation.
REFERENCES 1. Tapper D, Sawin R: Teratomas and other germ cell tumors, in O’Neill JA, Rowe MI, Grosfeld JL, et al (eds): Pediatric Surgery, St Louis, Mosby-Year Book, 1998, 447-460 2. Gengler JS, Ashcraft KW, Slattery P: Gastric teratoma: The sixth reported case in a female infant. J Pediatr Surg 30:889-890, 1995 3. Jona JZ: Congenital anorectal teratoma: Report of a case. J Pediatr Surg 31:709-710, 1996 4. King-Yin L, Chung-Yau L: Teratoma in the region of adrenal gland: A unique entity masquerading as lipomatous adrenal tumor. Surgery 126:90-94, 1999 5. Kreczy A, Alge A, Menardi G, et al: Teratoma of the umbilical cord. Case report with review of the literature. Arch Pathol Lab Med 118:934-937, 1994
6. Azizkhan RG, Caty MG: Teratomas in childhood. Curr Opin Pediatr 8:287-292, 1996 7. Parkes SE, Muir KR, Southern L, et al: Neonatal tumors: A thirtyyear population based study. Med Pediatr Oncol 22:309-317, 1994 8. Bond SJ, Seibel N, Kapur S, et al: Rhabdomyosarcoma of the clitoris. Cancer 73:1984-1986, 1994 9. Brock JW, Morgan W, Anderson TL: Congenital hemangiopericytoma of the clitoris. J Urol 153:468-469, 1995 10. Kearse WS Jr, Ritchley ML: Clitoral enlargement secondary to neurofibromatosis. Clin Pediatr 32:303-304, 1993 11. Levard G, Podevin J, Levillain P, et al: Congenital neoplasm of the clitoris. J Urol 157:649, 1997 12. Cotran RS, Kumar V, Collins T: Pathologic Basis of Disease (ed 6). Philadelphia, PA, Saunders, 1999, p 263
Congenital Vulvar Teratoma in a Newborn ¨ zbas¸ar, O ¨ . C¸andır, H. Kaya, and F. C¸ag˘layan By M. C¸akmak, C¸. Savas¸, D. O Kırıkkale, Turkey and Isparta, Turkey
The most common site of teratomas in neonates is in the sacrococcygeal region. Herein the authors describe a congenital teratoma, and to their knowledge it is the first reported case of vulvar site in a newborn. Tumor was removed on the fourth day of life, and she was completely normal 18 months after the operation. Because of possible malignancy
or recurrence, complete surgical excision of the tumor and careful follow-up is the treatment of choice. J Pediatr Surg 36:620-621. Copyright © 2001 by W.B. Saunders Company.
T
healthy after evaluation of the other organ systems. Hemogram and biochemistry findings were all within normal limits, except the unconjugated bilirubin level (8.7 mg/dL). Alpha-fetoprotein (AFP) and carcinoembryogenic antigen (CEA) levels also were normal (AFP, 0.02 ng/mL; CEA, 1.38 ng/mL). Plain abdominal and pelvic radiographs in both sides were normal. The patency of the urethra and the bladder capacity were normal in cysto-urethrogram. A computed tomography scan showed a polypoid and pedinculated mass, 5 cm in diameter and in fatty density (⫺40 HU). All the other abdominal and pelvic structures were normal. The tumor was resected on the fourth day of her life, and the space between the clitoris and urethra was approximated, which was the result of resection of the base of the tumor. The recovery was uneventful, and she was discharged at the fifth postoperative day. She was completely normal 18 months after the operation, and the CEA and AFP levels were normal.
HE MOST COMMON site of teratomas in neonates is in the sacrococcygeal and presacral region (47%). The other most common sites are the gonads (36%) and the mediastinum (6%).1 The remaining 11% are distributed in the mesenterium, retroperitoneum, pericardium, spinal canal, cervical, nasopharyngeal and orbital regions, and the neurocranium. In rare cases, hepatic, gastric, anorectal, adrenal, and umbilical cord teratomas also have been reported.1-5 In the current study, we report a congenital vulvar teratoma, which is a very unusual site in a newborn and a unique case appearing in vulva. We also review the literature relevant to teratomas and neonatal tumors of the vulva. CASE REPORT A girl, who is a first child of the family, was delivered vaginally in the 38th week of gestation. Her birth weight was 2,600 g. There was no familial relationship between the parents, and the pregnancy was uneventful. The neonate was transferred to the hospital because of a solid tumor in the vulva on her first day of life. A pedinculated spheric mass 55 ⫻ 45 ⫻ 40 mm in diameter was seen on her physical examination. The mass was soft and covered with skin. It was originating from the vulva with a pedicle, and the base of the mass was just below the clitoris and above the urethral opening. The base of the mass (its pedicle) was 1 cm in diameter. Two reddish openings on the mass were catheterized, and 2 blind sinuses approximately 1 cm in length were determined (Fig 1). All the external genital structures, urethral, and vaginal orifices were completely normal. The baby was found
From the Departments of Pediatric Surgery, Obstetrics and Gynecology, and Pathology, Su¨leyman Demirel University, Medical School, Isparta, Turkey, and the Department of Pediatric Surgery, Kırıkkale University, Medical School, Kırıkkale, Turkey. Address reprint requests to Murat C¸akmak, MD, Barıs¸ Sitesi, 80 Sok. No: 5, Mustafa Kemal Mah, Ankara, Turkey. Copyright © 2001 by W.B. Saunders Company 0022-3468/01/3604-0018$35.00/0 doi:10.1053/jpsu.2001.22302 620
INDEX WORDS: Teratoma, vulva.
Pathology The specimen was fixed in 10% formalin and embedded in paraffin, processed in the usual manner, and stained with H&E. Some sections also were immunostained with monoclonal antibodies to S100 (Dako Corp, Carpinteria, CA) and desmin (Dako Corp).
Macroscopy The mass was soft and largely covered by skin. There were 2 reddish openings that were blind sinuses. On the cut surface, yellow, lobulated adipous tissue, grayish-white areas, and several little cysts were seen.
Microscopy Microscopic examination found a mature teratoma consisting of a lipomalike mature fat tissue with fibrous septae, lymphoid follicles, and cystic areas, which were lined internally with intestinal epitelium changed into stratified squamous epitelium underlying fibrous connective tissue, which contained skin adnex, peripheral nerves, islands of fat, and smooth muscle. There were normal fibrous tissue of the resection margins.
DISCUSSION
Teratomas are embryonal neoplasms consisting of tissues of the 3 germ layers.4 Excluding testicular teratomas, 75% of these tumors occur in girls, and approximately 80% are benign. Teratomas occuring in infancy Journal of Pediatric Surgery, Vol 36, No 4 (April), 2001: pp 620-621
VULVAR TERATOMA
621
Fig 1. A pedinculated spheric mass 55 ⴛ 45 ⴛ 40 mm in diameter and 2 reddish openings.
and early childhood usually are extragonadal, whereas older children predominantly present with gonadal teratomas.6 Although it has been reported that mature teratomas were the most common tumors among the neonatal solid tumors, teratomas are tumors that rarely exist in children.7 The most common site for a neonatal teratoma is the sacrococcygeal region. Their location in vulva, however, is an unusual finding.1 Congenital tumors of the vulva are rare conditions. Some benign or malignant tumors including benign hemangiopericytoma, neurofibroma, malignant schwannoma, rhabdomyosarcoma, and primary endodermal sinus tumor also should be considered in addition to the teratoma.8-10 Levard et al11 reported a case of congenital neoplasm of the clitoris, which was a choristoma. This was an aberrant rest or heterotopic tissue, located at the tip of the
clitoris, which contained intestinal tissue.11 The investigators explained that the urogenital membrane at the origin of the vulvar mucosa derives from endodermlike intestine. Choristoma is defined as microscopically normal cells or tissues that are present in abnormal locations as a result of embryologic developmental anomaly, whereas teratoma contains tissues of the 3 germ layers.12 Although there are different widely accepted theories about the histogenesis of teratomas, one is the germ cell theory. Because primitive germ cells possess all the DNA for any type of cell, they have the ability to form the tissues seen in teratomas. The migration pattern of these cells also explains the gonadal, midline, and paramedian locations of the teratomas.1 Jona3 reported the first case of congenital anorectal teratoma that originated in the rectal wall, which had no connection with the coccyx and presented as a prolapsing posterior perineal mass at birth. It contained 2 sinuses 1 and 2 cm in depth, and pathologic diagnosis was benign mature teratoma that contained intestinal tissue, like our case.3 There were 2 recurrences at 9 and 12 months in this case, which required reexcision. Congenital teratomas of the vulva are rare conditions seen in the newborn. Some other benign or malignant tumors of the vulva should also be considered. Because of possible malignancy or recurrence, complete resection of the tumor is the treatment of choice. Long-term follow-up and control of the patient by physical examination, tumor markers, and by radiologic studies must be done after the operation.
REFERENCES 1. Tapper D, Sawin R: Teratomas and other germ cell tumors, in O’Neill JA, Rowe MI, Grosfeld JL, et al (eds): Pediatric Surgery, St Louis, Mosby-Year Book, 1998, 447-460 2. Gengler JS, Ashcraft KW, Slattery P: Gastric teratoma: The sixth reported case in a female infant. J Pediatr Surg 30:889-890, 1995 3. Jona JZ: Congenital anorectal teratoma: Report of a case. J Pediatr Surg 31:709-710, 1996 4. King-Yin L, Chung-Yau L: Teratoma in the region of adrenal gland: A unique entity masquerading as lipomatous adrenal tumor. Surgery 126:90-94, 1999 5. Kreczy A, Alge A, Menardi G, et al: Teratoma of the umbilical cord. Case report with review of the literature. Arch Pathol Lab Med 118:934-937, 1994
6. Azizkhan RG, Caty MG: Teratomas in childhood. Curr Opin Pediatr 8:287-292, 1996 7. Parkes SE, Muir KR, Southern L, et al: Neonatal tumors: A thirtyyear population based study. Med Pediatr Oncol 22:309-317, 1994 8. Bond SJ, Seibel N, Kapur S, et al: Rhabdomyosarcoma of the clitoris. Cancer 73:1984-1986, 1994 9. Brock JW, Morgan W, Anderson TL: Congenital hemangiopericytoma of the clitoris. J Urol 153:468-469, 1995 10. Kearse WS Jr, Ritchley ML: Clitoral enlargement secondary to neurofibromatosis. Clin Pediatr 32:303-304, 1993 11. Levard G, Podevin J, Levillain P, et al: Congenital neoplasm of the clitoris. J Urol 157:649, 1997 12. Cotran RS, Kumar V, Collins T: Pathologic Basis of Disease (ed 6). Philadelphia, PA, Saunders, 1999, p 263