Background. Intestinal-type primary vaginal adenocarcinoma is an extremely rare neoplasm, and very few cases are reported in the literature. The differential diagnosis of an intestinal-type adenocarcinoma at vaginal level (an organ almost free from glandular tissue) is particularly challenging given the anatomical complexity and different embryologic derivations of organs in this district. The main diagnostic issue consists in determining whether the vaginal neoplasm is primary, or it is a metastatic disease that extends to the vagina. A fundamental role in guiding diagnostic and therapeutic pathways is played by the pathologist. Objective. This study is a systematic review of the literature in order to summarize and analyze different diagnostic and therapeutic approaches chosen for every single case of intestinal-type primary vaginal adenocarcinoma described. Moreover, we report the case diagnosed and managed at our center. Methods. PubMed, ClinicalTrials.gov, Scopus, and Web of Science databases were systematically searched for records from January 1st, 1989, to December 1st, 2019. Results. Overall, 23 cases of intestinal-type primary vaginal adenocarcinoma are reported in the literature. This tumor often presents with atypical vaginal discharge (64.7% of cases) and it affects mainly the posterior wall (54.5%) and the lower third (83.3%) of the vagina. The average age at its presentation is 53.6 years. Diagnostic workup looks at ruling out possible primary distant sources of the disease and colonoscopy is often performed. The immunohistochemical profile of the lesion has a major role, and the key markers investigated are CEA, CK20, CK7, and CDX2. Most patients are diagnosed with early-stage disease (85% of patients FIGO I) and the lesion average size is 3 cm. Of 18 patients with available data, a surgical approach was adopted in 8 cases. Ten patients underwent radiotherapy. Patients managed surgically, compared with those who underwent radiotherapy, were younger and with a smaller mass at diagnosis, although differences were not statistically significant. Treatment options depended on clinical evaluation, patient’s comorbidities, and patient’s preferences. Conclusions. Intestinal-type primary vaginal adenocarcinoma is a rare tumor, and no specific guidelines addressing this disease are available. Ruling out a metastatic disease at the vaginal level is fundamental, and the pathologist plays a major role in the differential diagnosis. A multidisciplinary approach to the disease is of fundamental importance and the treatment choice should be tailored considering the patient’s comorbidities and the holistic evaluation of the case.

Contesto. L’adenocarcinoma vaginale primario di tipo intestinale è una neoplasia estremamente rara con pochissimi casi riportati in letteratura. La diagnosi differenziale per l’adenocarcinoma di tipo intestinale a livello vaginale (organo pressoché privo di tessuto ghiandolare) è particolarmente impegnativa data la complessità anatomica e le diverse derivazioni embriologiche degli organi di questo distretto. Il principale problema diagnostico consiste nel determinare se la neoplasia vaginale è primaria o è una malattia metastatica che si estende alla vagina, e a questo scopo, un ruolo fondamentale, nella guida dei percorsi diagnostici e terapeutici, è svolto dal patologo. Obiettivo. Questo studio è una revisione sistematica della letteratura al fine di riassumere e analizzare i diversi approcci diagnostici e terapeutici scelti per ogni singolo caso descritto di adenocarcinoma vaginale primario di tipo intestinale. Segnaliamo inoltre un caso diagnosticato e gestito presso il nostro centro. Metodi. Una ricerca sistematica della letteratura è stata condotta nei database PubMed, ClinicalTrials.gov, Scopus, e Web of Science dall’1 gennaio 1989 all’1 dicembre 2019. Risultati. In letteratura sono riportati 23 casi di adenocarcinoma vaginale primario di tipo intestinale. Questo tumore si presenta spesso con perdite vaginali atipiche (64.7% dei casi) e colpisce principalmente la parete posteriore (54.5%) e il terzo inferiore (83.3%) della vagina. L’età media alla sua presentazione è di 53.6 anni. L’iter diagnostico parte esaminando le possibili fonti primarie della malattia e viene spesso eseguita la colonscopia. Il profilo immunoistochimico della lesione ha un ruolo importante e gli indicatori chiave studiati sono CEA, CK20, CK7 e CDX2. Alla maggior parte dei pazienti viene diagnosticata una malattia allo stadio iniziale (85% dei pazienti FIGO I) e la dimensione media della lesione è di 3 cm. Dei 18 pazienti con dati disponibili, in 8 casi è stato adottato un approccio chirurgico. Dieci pazienti sono stati sottoposti a radioterapia. I pazienti gestiti chirurgicamente, rispetto a quelli sottoposti a radioterapia, erano più giovani e con una massa inferiore alla diagnosi, sebbene le differenze non fossero statisticamente significative. Le opzioni di trattamento dipendevano dalla valutazione clinica, dalle comorbilità del paziente e dalle preferenze del paziente. Conclusioni. L’adenocarcinoma vaginale primitivo di tipo intestinale è un tumore raro e non sono disponibili linee guida specifiche per affrontare questa malattia. È fondamentale escludere una malattia metastatica a livello vaginale e il patologo svolge un ruolo importante nella diagnosi differenziale. Un approccio multidisciplinare alla malattia è di fondamentale importanza e la scelta del trattamento deve essere adattata alle comorbilità del paziente e ad una valutazione olistica del caso.

Intestinal-type primary vaginal adenocarcinoma. Review of the literature with report of a case: from diagnosis to management

Casarin, J.;Cromi, A.;Ghezzi, F.;
2021-01-01

Abstract

Background. Intestinal-type primary vaginal adenocarcinoma is an extremely rare neoplasm, and very few cases are reported in the literature. The differential diagnosis of an intestinal-type adenocarcinoma at vaginal level (an organ almost free from glandular tissue) is particularly challenging given the anatomical complexity and different embryologic derivations of organs in this district. The main diagnostic issue consists in determining whether the vaginal neoplasm is primary, or it is a metastatic disease that extends to the vagina. A fundamental role in guiding diagnostic and therapeutic pathways is played by the pathologist. Objective. This study is a systematic review of the literature in order to summarize and analyze different diagnostic and therapeutic approaches chosen for every single case of intestinal-type primary vaginal adenocarcinoma described. Moreover, we report the case diagnosed and managed at our center. Methods. PubMed, ClinicalTrials.gov, Scopus, and Web of Science databases were systematically searched for records from January 1st, 1989, to December 1st, 2019. Results. Overall, 23 cases of intestinal-type primary vaginal adenocarcinoma are reported in the literature. This tumor often presents with atypical vaginal discharge (64.7% of cases) and it affects mainly the posterior wall (54.5%) and the lower third (83.3%) of the vagina. The average age at its presentation is 53.6 years. Diagnostic workup looks at ruling out possible primary distant sources of the disease and colonoscopy is often performed. The immunohistochemical profile of the lesion has a major role, and the key markers investigated are CEA, CK20, CK7, and CDX2. Most patients are diagnosed with early-stage disease (85% of patients FIGO I) and the lesion average size is 3 cm. Of 18 patients with available data, a surgical approach was adopted in 8 cases. Ten patients underwent radiotherapy. Patients managed surgically, compared with those who underwent radiotherapy, were younger and with a smaller mass at diagnosis, although differences were not statistically significant. Treatment options depended on clinical evaluation, patient’s comorbidities, and patient’s preferences. Conclusions. Intestinal-type primary vaginal adenocarcinoma is a rare tumor, and no specific guidelines addressing this disease are available. Ruling out a metastatic disease at the vaginal level is fundamental, and the pathologist plays a major role in the differential diagnosis. A multidisciplinary approach to the disease is of fundamental importance and the treatment choice should be tailored considering the patient’s comorbidities and the holistic evaluation of the case.
2021
Immunohistochemistry; Intestinal type; Primary vaginal adenocarcinoma; Skene glands; Systematic review
Franchi, M.; Garzon, S.; Caliò, A.; Zorzato, P. C.; Bosco, M.; Casarin, J.; Festi, A.; Cromi, A.; Ghezzi, F.; Uccella, S.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11383/2151391
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