Title Multiple rare primary malignancies: a mixed squamous neuroendocrine adenocarcinoma of the cervix, metastasized carcinosarcoma and extramammary vulvar Paget’s disease case report /
Authors Andreika, Linas ; Vankevičienė, Karolina ; Plioplytė, Marija ; Bitinaitytė, Monika ; Rudaitis, Vilius
DOI 10.3390/medicina59050995
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Is Part of Medicina.. Basel : MDPI. 2023, vol. 59, iss. 5, art. no. 995, p. [1-10].. ISSN 1010-660X. eISSN 1648-9144
Keywords [eng] carcinosarcoma ; extramammary vulvar Paget’s disease ; Malignant Mixed Müllerian Tumor ; multiple primary malignancies ; Müllerian carcinosarcoma ; neuroendocrine adenocarcinoma
Abstract [eng] The occurrence of more than one primary malignant tumor in a single patient is rare. Multiple primary malignancies can pose difficulties in differential diagnosis between primary tumors and metastasis. Here, we present a case report with multiple primary malignancies. The patient is a 45-year-old female who was diagnosed with cervical mixed squamous neuroendocrine adenocarcinoma, metastasized carcinosarcoma and extramammary vulvar Paget’s disease. The patient was first diagnosed with a microinvasive squamous cervical carcinoma in situ. After a few months, the amputation of a small residual tumor and histological evaluation revealed an IA1-stage poorly differentiated (G3) mixed squamous and neuroendocrine cervical adenocarcinoma. After two years, the disease had progressed and biopsies from altered sites were taken. Histological diagnosis from an ulcerated vulvar region revealed extramammary vulvar Paget’s disease. A biopsy from vagina polyp revealed an earlier diagnosed mixed squamous and neuroendocrine cervical adenocarcinoma. However, histological diagnosis from an inguinal lymph node biopsy was unexpected and revealed carcinosarcoma. It indicated either the development of another primary malignancy, or an unusual spread of metastasis. Clinical presentation as well as diagnostic and treatment challenges are discussed in this case report. This case report shows that multiple primary malignancy cases are difficult to manage both for clinicians and the patient because the therapeutic options can become limited. This complex case was managed by a multidisciplinary team.
Published Basel : MDPI
Type Journal article
Language English
Publication date 2023
CC license CC license description