Extragonadal Non-gestational Choriocarcinoma with Tonsillar Presentation
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Extragonadal Non‑gestational Choriocarcinoma with Tonsillar Presentation Thomas Payne1 · Silvana Di Palma2 · David Walker1 · Jonathan Dakin3 · Lester D. R. Thompson4 Received: 27 September 2020 / Accepted: 22 October 2020 © This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply 2020
Abstract Extragonadal non-gestational choriocarcinoma is a rare but well-described phenomenon occurring in patients with midline germ cell tumors. Choriocarcinoma (ChC) is an aggressive neoplasm usually developing in women as a rare complication of pregnancy. In male patients ChC occurs in the testes, usually as a component of mixed germ cell tumors. Very few patients develop extragonadal choriocarcinoma with the tumor occurring in midline locations, such as the mediastinum, retroperitoneum, and central nervous system (mostly pineal gland). Non-midline choriocarcinoma can occur in the lung, gastrointestinal tract, and breast, sometimes blended with another primary malignancy. A midline choriocarcinoma manifesting as a head and neck malignancy is exceptional. During an evaluation of multiple enlarged cervical lymph nodes suspected to be lymphoma in a 72-year-old man, a core biopsy was taken from one of the left neck lymph nodes which histologically showed a necrotic malignancy with strong diffuse pancytokeratin staining. After an initial interpretation of metastatic carcinoma, further samples were taken from both tonsils and from a right level 5 neck lymph node. Histologically, all samples contained the same tumor, showing profound pleomorphism and multinucleated syncytial-type giant cells. A panel of immunohistochemistry studies were performed, including β-human chorionic gonadotropin, with positive findings leading to a diagnosis of extragonadal non-gestational choriocarcinoma. Keywords Extragonadal non-trophoblastic choriocarcinoma · Midline · Metastases · Palatine tonsil · Male · β-Human chorionic gonadotropin · Immunohistochemistry
Introduction Male extragonadal non-gestational choriocarcinoma (ChC) is a rare but well-described phenomenon occurring in patients with midline germ cell tumors. Previous literature has reported that mediastinum, central nervous system, and retroperitoneum are the most common sites of extra-gonadal ChC, with the pineal region the most common CNS site * Lester D. R. Thompson [email protected] 1
Otolaryngology Department, Royal Surrey Hospital, Guildford, Surrey, UK
2
Histopathology Department, Royal Surrey Hospital, Guildford, Surrey, UK
3
Respiratory Medicine Department, Royal Surrey Hospital, Guildford, Surrey, UK
4
Department of Pathology, Southern California Permanente Medical Group, 5601 De Soto Avenue, Woodland Hills, CA 91365, USA
[1–4], whereas lung, breast, and gastrointestinal tract are the most prevalent organ locations beyond midline sites [5, 6]. Metastases to the oral cavity or oropharynx are rare. The most common tumor sites of origin are lungs (20%), kidney (16%), breast (11%), and gastroi
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