Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case Report

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 fe...

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Main Authors: Linas Andreika, Karolina Vankevičienė, Marija Plioplytė, Monika Bitinaitytė, Vilius Rudaitis
Format: Article
Language:English
Published: MDPI AG 2023-05-01
Series:Medicina
Subjects:
Online Access:https://www.mdpi.com/1648-9144/59/5/995
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author Linas Andreika
Karolina Vankevičienė
Marija Plioplytė
Monika Bitinaitytė
Vilius Rudaitis
author_facet Linas Andreika
Karolina Vankevičienė
Marija Plioplytė
Monika Bitinaitytė
Vilius Rudaitis
author_sort Linas Andreika
collection DOAJ
description 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.
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spelling doaj.art-3e4aa94953f54d25a35a0b3e6718e6552023-11-18T02:23:25ZengMDPI AGMedicina1010-660X1648-91442023-05-0159599510.3390/medicina59050995Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case ReportLinas Andreika0Karolina Vankevičienė1Marija Plioplytė2Monika Bitinaitytė3Vilius Rudaitis4Clinic of Obstetrics and Gynaecology, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Čiurlionio Str. 21/27, LT-03101 Vilnius, LithuaniaFaculty of Medicine, Vilnius University, M. K. Čiurlionio Str. 21/27, LT-03101 Vilnius, LithuaniaFaculty of Medicine, Lithuanian University of Health Sciences, A.Mickevičiaus Str. 9, LT-44307 Kaunas, LithuaniaFaculty of Medicine, Lithuanian University of Health Sciences, A.Mickevičiaus Str. 9, LT-44307 Kaunas, LithuaniaClinic of Obstetrics and Gynaecology, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Čiurlionio Str. 21/27, LT-03101 Vilnius, LithuaniaThe 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.https://www.mdpi.com/1648-9144/59/5/995carcinosarcomaextramammary vulvar Paget’s diseasemultiple primary malignanciesMüllerian carcinosarcomaMalignant Mixed Müllerian Tumorneuroendocrine adenocarcinoma
spellingShingle Linas Andreika
Karolina Vankevičienė
Marija Plioplytė
Monika Bitinaitytė
Vilius Rudaitis
Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case Report
Medicina
carcinosarcoma
extramammary vulvar Paget’s disease
multiple primary malignancies
Müllerian carcinosarcoma
Malignant Mixed Müllerian Tumor
neuroendocrine adenocarcinoma
title Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case Report
title_full Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case Report
title_fullStr Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case Report
title_full_unstemmed Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case Report
title_short Multiple Rare Primary Malignancies: A Mixed Squamous Neuroendocrine Adenocarcinoma of the Cervix, Metastasized Carcinosarcoma and Extramammary Vulvar Paget’s Disease Case Report
title_sort multiple rare primary malignancies a mixed squamous neuroendocrine adenocarcinoma of the cervix metastasized carcinosarcoma and extramammary vulvar paget s disease case report
topic carcinosarcoma
extramammary vulvar Paget’s disease
multiple primary malignancies
Müllerian carcinosarcoma
Malignant Mixed Müllerian Tumor
neuroendocrine adenocarcinoma
url https://www.mdpi.com/1648-9144/59/5/995
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