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| CASE REPORT |
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| Year : 1992 | Volume
: 38
| Issue : 1 | Page : 44-5 |
Endodermal sinus tumor of vulva (a case report).
SJ Penkar, S Irani, VL Prabhu, FP Candes, SA Nimbkar
Department of Obstetrics and Gynecology, R.N. Cooper Hospital, Andheri West, Bombay.
Correspondence Address:
S J Penkar
Department of Obstetrics and Gynecology, R.N. Cooper Hospital, Andheri West, Bombay.
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 0001380986 
An unusual case of endodermal sinus tumor (EST) of the ovary at an extragonadal site-vulva, in an unmarried female of 25 yr is reported. The patient presented only with a vulval swelling on the right side without any other signs or symptoms. The internal genital organs mainly the ovaries were normal.
Keywords: Adult, Case Report, Female, Human, Mesonephroma, pathology,surgery,Tumor Markers, Biological, analysis,Vulva, pathology,surgery,Vulvar Neoplasms, pathology,surgery,alpha-Fetoproteins, analysis,
How to cite this article:
Penkar S J, Irani S, Prabhu V L, Candes F P, Nimbkar S A. Endodermal sinus tumor of vulva (a case report). J Postgrad Med 1992;38:44 |
Endodermal sinus tumor (EST) is a rare type of ovarian germ cell tumour, initially distinguished and described 2 by Teifurn Earlier it was included in Schiller's original description of mesonephroma together with ovarian tumours, now designated as clear cell carcinoma. Like other germ cell tumours of the ovary EST is rare and occurs commonly in young women.
The purpose of this presentation is to put on record yet another case of pure endodermal sinus tumour at an extra gonadal site (18th case) in the region of the vulva (3rd case) in the world literature[4].
Miss MP, a 25 year old unmarried woman presented in the gynaecology OPD of Dr. RN Cooper Hospital, Bombay, India, in the month of January 1988, with a complaint of a swelling noticed on the right of the vulval region, extending over the public sumphysis, since two months without any increase in size.
The patient was one of five siblings all alive (three brothers and two sisters). Her menstrual history was normal, the, last menstrual period was on 8-1-88.
General and systemic examinations were normal. A very firm, well circumscribed growth was present on the right labia majora which was 12 cm x 8cm in size, oval in shape, with a nodular feel, non tender and firm in consistency. The skin covering the mass was smooth and uninvolved. The mass was free from the public bone underneath. No other mass or any significant enlargement of inguinal lymph nodes was present.
Routine investigations were within normal limits. Ultrasonography showed normal ovaries and normal uterine endometrial echoes. Laparoscopic examination also showed a normal uterus, tubes and ovaries.
On 1-2-88 total excision of the vulval swelling was done. The mass with surrounding fibrofatty tissue was removed. The mass occupied the anterior 2/3rds of the right labium majus extending over the public bone upto the external inguinal ring. It was free from the skin and the pubic bone. Postoperative recovery was uneventful. Suture removal was done on the 9th day. The wound healed with minimal induration.
Postoperatively estimation of serum alpha feto proteins showed elevated levels of 335 ng/ml (normal 35 ng/ml) and oncology opinion was sought for further management of the case.
The excised mass was 10 cm x 7cm in size and very firm to feel. The surface was greyish white and lobulated with areas of haemorrhage and necrosis. A few solitary nodules were seen in the surrounding fibrofatty tissue.
Microscopic examination of the paraffin section, stained with haematoxylin eosin showed a tumour formed by small round to oval cells, having scanty cytoplasm. The nuclei were hyperchromatic, vesicular and showed increased mitotic activity. Loose reticular network and cystic areas lined by flattened and oval mesothelial were also seen. Schiller - Duval bodies with perivascular mantling were not seen. Numerous yolksac vesicular structures lined by columnar epithelium were seen. The vesicles contained eosinophilic material and PAS positive hyaline globules. These features are adequate to label the lesion as EST.
Endodermal sinus tumour is so called because of its histologic similarity to the estra-embryonal structures of the early embryo. It is an highly malignant extraembryonal germ cell tumour, showing selective growth of yolk sac endoderm. It is associated with a worse prognosis, the fatality being 90% at the end of three years[3].
The occurrence of this tumour at an extragonadal site is extremely rare. Ungerleider et al[4] cited from literature 17 cases of this tumour at extragonadal site 10 cases in the vagina, 2 cases in the pelvis, one each in the broad ligament, 'retroperitoneum, maxillary sinus, cervic, brain and at the same time reported the first case in the region of the valva.
Misplaced germ cells during embryonic life, is believed to be the source for extragonadal occurrence of this tumour. Onset of syrntoms, diagnosis and an invariable death within a very short span is a common sequence. Recurrence and metastasis following simple or extended surgical excision is the rule. Abdominal total hysterectomy with bilateral salpingo-oophorectomy has been the standard treatment followed so far, but recently adjuvant chemotherapy with combination of multiple chemotherapeutic agents has improved the salvage rate. Higher levels of alpha feto proteins are observed in these patients and used as a marker during the treatment.
| :: References | |  |
| 1. |
Teilum G. Endodernal sinus tumor of the ovary and testis. Comparative morphogenesis of the so-called megonephroma ovarii (Schiller) and extraembryonic (yolk sac-allantoic) structures of the rat's placenta. Cancer 1959; 12:1092-1105.  |
| 2. | Schill W. Mesonephroma ovarii. Amer J Cancer 1939; 35:1-21. |
| 3. | Santesson L, Marrubine G. Clinical and pathological survery of embryonal carcinoma, including so called "mesonephromas" (Schiller) or 'mesoblastomas') Teilum), treated at the Radiumhemment. Acta Obstet & Gynaecol Scand 1957; 36:399-419. |
| 4. | Ungerleider RS, Donaldson SS, Warnke RA, Wilbur JR. Endodermal sinus tumour. The Stanford experience and the first reported case arising in the valua. Cancer 1978; 41:1627-1634.
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