Metastatic HPV-related cervical adenocarcinomas presenting with thromboembolic events (Trousseau Syndrome): clinicopathologic characteristics of 2 cases.
Two cases of systemic thromboembolism (Trousseau syndrome) associated with metastatic human papillomavirus (HPV)-related endocervical adenocarcinomas are reported. The first patient, age 36, presented with bilateral lower extremity deep vein thromboses, pulmonary embolism, and supraclavicular and cervical lymphadenopathy. Lymph node biopsy revealed metastatic mucinous adenocarcinoma with focal signet ring cell differentiation. Imaging studies demonstrated metastatic disease without a defined primary site. Acute renal and respiratory failure developed and the patient expired shortly after initiation of chemotherapy, 7 weeks after presentation. Autopsy examination revealed widespread metastatic adenocarcinoma with a 2 cm cervical adenocarcinoma. The second patient, age 43, presented with left internal jugular vein thrombosis, acute thrombophlebitis, and bilateral axillary lymphadenopathy. She developed progressive venous thrombosis despite anticoagulation. Imaging studies demonstrated widespread lymphadenopathy and an adnexal mass. Diagnostic laparoscopy with biopsies and left oophorectomy revealed metastatic mucinous adenocarcinoma with signet ring cell differentiation involving peritoneum, ovary, cervix, and bladder without a defined primary site. Progressive thromboembolic disease with acute renal failure and multiple cerebral infarcts developed and the patient expired shortly after initiation of chemotherapy, 2 months after presentation. No autopsy was performed. HPV DNA was detected by in situ hybridization in the lymph node metastasis in the first case and in the cervical and ovarian tumor specimens in the second case. High-risk HPV-related endocervical adenocarcinomas occasionally exhibit signet ring cell differentiation and can present with Trousseau syndrome. These features more commonly suggest metastatic adenocarcinoma of upper gastrointestinal tract origin but the presence of HPV DNA within the tumors establishes them as cervical in origin.
Veras
Emanuela
E
Departments of Pathology, The Johns Hopkins University School of Medicine and Hospital, Baltimore, Maryland 21231, USA.
Srodon
Monica
M
Neijstrom
Eric S
ES
Ronnett
Brigitte M
BM
D000230:Adenocarcinoma / Q000150:complications* / Q000821:virology; D000328:Adult; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D030361:Papillomavirus Infections / Q000150:complications*; D013923:Thromboembolism / Q000209:etiology*; D002583:Uterine Cervical Neoplasms / Q000150:complications* / Q000821:virology