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26 May 2011 Case of the Week #207
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Thanks to Kristin Olson, University of California, Los Angeles (USA), for contributing this case. This case was reviewed in May 2020 by Dr. Jennifer Bennett, University of Chicago and Dr. Carlos Parra-Herran, University of Toronto.
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Case of the Week #207
An 8 year old girl presented to her primary care physician with precocious puberty. On physical examination, she had a large pelvic mass. The well circumscribed mass was resected. It weighed 413 grams and measured 13.5 x 9 x 6 cm. Sectioning demonstrated a variegated yellow-tan to red-brown cystic and hemorrhagic mass. One 2.5 cm cyst contained sebaceous material, and scattered calcifications were identified.
Yellow-tan to red-brown cystic and hemorrhagic mass
2.5 cm cyst
What is your diagnosis?
Mixed Malignant Germ Cell Tumor - Choriocarcinoma and Mature Teratoma
Microscopic examination showed the mass to be a mixed malignant germ cell tumor consisting of choriocarcinoma (70%) and mature teratoma (30%). Angiolymphatic invasion is present, as shown on the CD34 stain. The choriocarcinoma component is highlighted with the beta-hCG stain, and is associated with extensive hemorrhagic necrosis. AFP is negative.
Left to right: CD34, beta-hCG, AFP
Ovarian choriocarcinoma is classified as gestational or nongestational. Tumors are definitively classified as nongestational only in a pre-pubertal patient (as in this case), in a woman unable to conceive, or based on DNA analysis to determine if paternal DNA is present in the genome of the tumor.
Histologically, the tumor consists of a mixture of syncytiotrophoblastic and cytotrophoblastic elements in a hemorrhagic and necrotic background. Tumor cells are strongly immunoreactive for beta-hCG, and negative for AFP. However, these tumors are often part of a mixed germ cell tumor, which may contain components that are immunoreactive for AFP.
Nongestational choriocarcinoma is very rare. Historically, these tumors were usually fatal and unresponsive to chemotherapy. However, their prognosis has improved (Int J Gynecol Cancer 2010;20:299, Gynecol Oncol 2001;80:262) and it is currently unclear if their prognosis differs from the good prognosis of gestational choriocarcinoma. Both types of choriocarcinoma have high levels of serum hCG, and monitoring serum levels may be helpful in predicting recurrence.
In this case, at diagnosis, the beta hCG was 140,000. Radiologic evaluation for metastases was negative, and the patient underwent three cycles of chemotherapy. One year after completing the therapeutic regimen, there was no evidence of recurrent or metastatic disease.
Nat Pernick, M.D., President
and Liz Parker, B.A., Associate Medical Editor
30100 Telegraph Road, Suite 408
Bingham Farms, Michigan (USA) 48025
Alternate email: NatPernick@gmail.com