Case 2

Gynocologic Pathology

Case 2: Endodermal Sinus Tumor of the Vulva

  • Contributed by Eric Thompson, MD
    Department of Pathology, University of Oklahoma Health Sciences Center
  • Published on-line in November 1999

Patient History:

The patient was a Caucasian female who presented at 18 years-of-age with a 9 month history of a right vulvar labial mass. On physical examination a mass (4.0 x 2.5 x 2.0 cm) of the right labia was noted. A Pathologist initially took a fine needle biopsy of the mass, but the specimen proved to be non-diagnostic. Thus, an excision biopsy was taken which resulted in a diagnosis of an endodermal sinus tumor of the vulva. Metastatic work-up, including diagnostic laparoscopy, was negative. The patient underwent a modified radical vulvectomy with concurrent ipsilateral inguinal lymph node dissection. The lymph nodes showed no evidence of metastasis and all margins of the excised tumor were free of tumor cells. The patient was treated parentally for three cycles with adjuvant platinum-based chemotherapy consisting of bleomycin, etoposide, and cisplatin (BEC), which has been reported to improve survival over vincristime, actinomycin, and cyclophosphamide (VAC) chemotherapy in gonadal endodermal sinus tumors. Part of the Clinical Pathology Laboratory work-up included measurement of the patient's serum alpha-fetoprotein; pre-treatment was 29.3 ng/dI (normal < 5 ng/dL), but this fell and remained in the normal range following treatment. Subsequently, she became pregnant and, after an antenatal course complicated only by intermittent mid- and upper-back pain, delivered a healthy male infant. Unfortunately, evaluation of the patient after delivery, due to progression of her back pain, revealed a 5 x 5 cm lower right lung mass and several mediastinal and supraclavicular lymph node masses by radiography and computerized tomography. A fine needle aspiration (FNA) of the supraclavicular lymph node was taken and sent to the Cytopathology Laboratory for examination. Cell preparations were stained with Papanicolaou stain for general morphology or with Periodic Acid/Schiff's Reagent for carbohydrates. 

Cytopathology:

The aspirate comprised many irregular sheets, cell balls, and modest numbers of single cells, indicating a moderate cohesiveness of the cells. The individual cells were large and pleomorphic with "bubbly" cytoplasm and one to several prominent nucleoli, some cells showing abnormal mitoses. Poorly formed Schiller-Duval bodies and many intracellular and extracellular, PAS-positive hyaline globules were present which are diagnostic features of endodermal sinus tumor. 

Slide 1
Papanicolaou stained cellular preparations of the endodermal sinus tumor.
(Click on the images for closer view)

Final Diagnosis:

The histology confirmed the suspected diagnosis of recurrent metastatic endodermal sinus tumor.

Treatment and Outcome:

The patient was aggressively treated with chemotherapy, consisting of etoposide, cisplatin, vincristine, actinomycin, and cyclophosphamide (EPO-VAC). In spite of aggressive therapy, she expired five months after diagnosis of her recurrence.

Discussion:

Endodermal sinus tumor of the vulva is a rare tumor; only seven cases have been reported in the literature (1-7). Endodermal sinus tumors typically locate to the gonads; they form the second most common malignant germ cell tumor of the ovary and account for 2/3rd's of germ cell tumors of the testes of infants less than 2 years-of-age (1,8). When occurring in the vulva, this malignancy appears to follow a much more aggressive course than its gonadal counterparts. Only one patient with vulvar endodermal sinus tumor has been reported to be disease-free at five years (2). Three other documented cases in which patients were treated with surgery, followed by chemotherapy and/or pelvic radiotherapy, succumbed to the disease within 12 months of diagnosis (3-5). To our knowledge, this is the first reported use of a fine needle aspiration in the diagnosis of an endodermal sinus tumor. Our case demonstrates a valuable method for diagnosing and monitoring endodermal sinus tumors and illustrates the poor prognosis and importance of long-term follow-up associated with such tumors originating in the vulva.

References:

  1. Dudley AG, Young RH, Lawrence WD, Scully RE: Endodermal sinus tumor of the vulva in an infant. Obstet Gynecol1983;61:76S-79S.
  2. Flanagan CW, Parker JR, Mannel RS, Min KW, Kida M: Primary Endodermal Sinus Tumor of the Vulva: A Case Report and Review of the Literature. Gynecol Oncol. 1997 Sep;66(3):515-8. Review.
  3. Castaldo TW, Petrelli SE, Ballon SC, Voet RL, LaGasse LD, Lubens R: Endodermal sinus tumor of the clitoris. Gynecol Oncol. 1980;9:376-80.
  4. Ungerleider RS, Donaldson SS, Warnke RA, Wilbur JR: Endodermal sinus tumor: The Stanford experience and the first reported case arising in the vulva. Cancer 1978;4 1:1627-34.
  5. Krishnamurthy SC, Sampat MB: Case report: Endodermal sinus (Yolk sac ) tumor of the vulva in a pregnant female.Gynecol OncoL 1981;11:379-82.
  6. Craighead PS, du Toit PFN: Endodermal sinus tumour of the vulva: An interesting clinicopathological problem. Euro JSurg OncoL 1993;19:203-5.
  7. Penkar SJ, Irani S, Prabhu VL, Candes FP, Nimbkar SA: Endodermal sinus tumor of the vulva: A case report. JPostgradMed1992;38:44-5.
  8. Young RH and Scully RE. Testicular Tumors. Chicago: ASCP Press, 1990: Chpt. 3; 38-40.