scholarly journals A rare case of gastric-type mucinous adenocarcinoma in a woman with Peutz-Jeghers syndrome

2019 ◽  
Vol 62 (6) ◽  
pp. 474
Author(s):  
Yeorae Kim ◽  
Eun Yi Kim ◽  
Tae Jin Kim ◽  
Kyung Taek Lim ◽  
Ki Heon Lee ◽  
...  
Keyword(s):  
Mucinous Adenocarcinoma ◽  
Rare Case ◽  
Gastric Type ◽  
Peutz Jeghers Syndrome

scholarly journals Gastric-type mucinous adenocarcinoma of the cervix in a woman with Peutz-Jeghers syndrome

2021 ◽  
Author(s):  
Tong Tong ◽  
Qiong Fan ◽  
Shu Shi ◽  
Yuhong Li ◽  
Yudong Wang
Keyword(s):  
Human Papilloma Virus ◽  
Mucinous Adenocarcinoma ◽  
Clinical Stage ◽  
Young Patients ◽  
Hpv Infection ◽  
Sexual Life ◽  
Autosomal Dominant Disorder ◽  
Papilloma Virus ◽  
Gastric Type ◽  
Peutz Jeghers Syndrome

Abstract Background: Peutz-Jeghers syndrome(PJS) is a very rare autosomal dominant disorder with predisposition to multiple neoplasms. Gastric-type adenocarcinoma (GAS) is a less common carcinoma of the cervix than squamous cell carcinoma, which is more aggressive and has lower 5 year survival rate than usual type endocervical adenocarcinoma (ECA), and unrelated to human papilloma virus(HPV) infection as well. We present a 32 year-old patient with Peutz-Jeghers syndrome who was found to have gastric-type adenocarcinoma of the cervix.Case presentation: A 32-year-old woman without sexual life ever who was diagnosed Peutz-Jeghers syndrome when she was two years old presented with watery discharge for more than 6 months. A tumor around 6cm was found on the cervix and she was diagnosed gastric-type mucinous adenocarcinoma of the cervix clinical stage IB3. She was treated with artery intervention chemotherapy for one course followed by radical surgery and then systematic chemotherapy.Conclusions: The case suggests more thorough cancer screening for patients with PJS as the disorder is rare and has high risk of malignancies. Young patients with Peutz-Jeghers syndrome, including those without sexual life, who have watery discharge or bleeding should be screened for cervical carcinoma even if cytologic results or human papilloma virus (HPV) is negative.

scholarly journals Gastric-Type Mucinous Adenocarcinoma of the Cervix in A Woman With Peutz-Jeghers Syndrome

2021 ◽  
Author(s):  
tong tong ◽  
Qiong Fan ◽  
Shu Shi ◽  
Yuhong Li ◽  
Yudong Wang
Keyword(s):  
Human Papilloma Virus ◽  
Mucinous Adenocarcinoma ◽  
Clinical Stage ◽  
Young Patients ◽  
Hpv Infection ◽  
Sexual Life ◽  
Autosomal Dominant Disorder ◽  
Papilloma Virus ◽  
Gastric Type ◽  
Peutz Jeghers Syndrome

Abstract Background: Peutz-Jeghers syndrome(PJS) is a very rare autosomal dominant disorder with predisposition to multiple neoplasms. Gastric-type adenocarcinoma (GAS) is a less common carcinoma of the cervix than squamous cell carcinoma, which is more aggressive and has lower 5 year survival rate than usual type endocervical adenocarcinoma (ECA), and unrelated to human papilloma virus(HPV) infection as well. We present a 32 year-old patient with Peutz-Jeghers syndrome who was found to have gastric-type adenocarcinoma of the cervix. Case presentation: A 32-year-old woman without sexual life ever who was diagnosed Peutz-Jeghers syndrome when she was two years old presented with watery discharge for more than 6 months. A tumor around 6cm was found on the cervix and she was diagnosed gastric-type mucinous adenocarcinoma of the cervix clinical stage IB3. She was treated with artery intervention chemotherapy for one course followed by radical surgery and then systematic chemotherapy.Conclusions: The case suggests more thorough cancer screening for patients with PJS as the disorder is rare and has high risk of malignancies. Young patients with Peutz-Jeghers syndrome, including those without sexual life, who have watery discharge or bleeding should be screened for cervical carcinoma even if cytologic results or human papilloma virus (HPV) is negative.

Gastric-type adenocarcinoma of the cervix in patients with Peutz-Jeghers syndrome: a systematic review of the literature with proposed screening guidelines

2021 ◽  
pp. ijgc-2021-002997
Author(s):  
Sushmita B Gordhandas ◽  
Ryan Kahn ◽  
Dib Sassine ◽  
Emeline M Aviki ◽  
Becky Baltich Nelson ◽  
...  
Keyword(s):  
Systematic Review ◽  
Overall Survival ◽  
Pap Smear ◽  
Early Stage ◽  
Case Reports ◽  
High Risk Population ◽  
Normal Cytology ◽  
Presenting Symptoms ◽  
Gastric Type ◽  
Peutz Jeghers Syndrome

ObjectivesTo perform a systematic review of gastric-type adenocarcinoma of the cervix and lobular endocervical glandular hyperplasia (a possible precursor lesion) in Peutz-Jeghers syndrome, and to analyze data from the literature, along with our institutional experience, to determine recommendations for screening and detection.MethodsA comprehensive literature searc and retrospective search of pathology records at our institutio were conducted. Articles were screened by two independent reviewers. Case reports/series on lobular endocervical glandular hyperplasia/gastric-type adenocarcinoma of the cervix in Peutz-Jeghers syndrome were included. Demographic, clinical, and radiologic information was collected.ResultsA total of 1564 publications were reviewed; 38 met the inclusion criteria. Forty-nine were included in the analysis (43 from the literature, 6 from our institution). Forty-three reported on gastric-type adenocarcinoma alone, 4 on lobular endocervical glandular hyperplasia alone, and 2 on concurrent lobular endocervical glandular hyperplasia/gastric-type adenocarcinoma. Median age at diagnosis was 17 (range, 4–52) for patients with lobular endocervical glandular hyperplasia alone and 35 (range, 15–72) for those with gastric-type adenocarcinoma. The most common presenting symptoms were abdominal/pelvic pain and vaginal bleeding/discharge. Imaging was reported for 27 patients; 24 (89%) had abnormal cervical features. Papanicolaou (Pap) smear prior to diagnosis was reported for 12 patients; 6 (50%) had normal cytology, 4 (33%) atypical glandular cells, and 2 (17%) atypical cells not otherwise specified. Patients with gastric-type adenocarcinoma (n=45) were treated with surgery alone (n=16), surgery/chemotherapy/radiation (n=11), surgery/chemotherapy (n=9), surgery/radiation (n=5), or radiation/chemotherapy (n=4). Twelve (27%) of 45 patients recurred; median progression-free survival was 10 months (range, 1–148). Twenty patients (44%) died; median overall survival was 26 months (range, 2–156). Thirteen patients (27%) were alive with no evidence of disease.ConclusionsGastric-type adenocarcinoma in Peutz-Jeghers syndrome is associated with poor outcomes and short progression-free and overall survival. Screening recommendations, including pathognomonic symptom review and physical examination, with a low threshold for imaging and biopsy, may detect precursor lesions and early-stage gastric-type adenocarcinoma, leading to better outcomes in this high-risk population.PROSPERO registration numberCRD42019118151

Tailgut cyst and a very rare case of a tailgut cyst with mucinous adenocarcinoma in a 73 year old woman treated for buttock abscess with fistula

2019 ◽  
Vol 91 (3) ◽  
pp. 38-42
Author(s):  
Franciszek Rachel ◽  
Andrzej Guzik ◽  
Dariusz Szczerba ◽  
Karolina Kozieł ◽  
Krystian Gutterch
Keyword(s):  
Mucinous Adenocarcinoma ◽  
Rare Case ◽  
Tailgut Cyst

Autorzy przedstawiają u 73 letniej kobiety torbiel ogonowo-jelitową przestrzeni pozaodbytniczej, która uległą zezłośłiwieniu. Już w trzecim roku życia była leczona, jako przetoka ropna pośladka prawego, która się okresowo uaktywniała. W 2016roku zgłosiła się do Poardni Chirurgicznej w Gorlicach z olbrzymim ropniakiem pośladka prawego z przetoką ropną . Na oddziale chirurgicznym w Gorlicach operacyjnie usunieto ropniak o ścianach dobrze ukształtowanych razem z przetoką. Torbiel zawierała ponad litr ropy. Po wygojeniu w bliźnie pojawiłą się przetoka śluzowa .Badaniem histologicznym rozpoznano retrocecal cyst hamartoma z komórkami mucinos adenocarcinoma. W czasie następonego pobytu chorej w szpitalu po wykonaniu rezonansu magnetycznego stwierdzono resztkowa zmianę w okolicy przedkrzyżowej sięgająca do trzeciego kręgu krzyżowego. Zmianę usunięto radykalnie potwierdzoną badaniem histopatologicznym. U chorej nawrotu nie zaobserwowano.

scholarly journals Mucinous adenocarcinoma arising in an anorectal fistula

Medicina ◽  
2008 ◽  
Vol 45 (4) ◽  
pp. 286 ◽  
Author(s):  
Linas Venclauskas ◽  
Žilvinas Saladžinskas ◽  
Algimantas Tamelis ◽  
Darius Pranys ◽  
Dainius Pavalkis
Keyword(s):  
Computed Tomography ◽  
Soft Tissue ◽  
Histological Examination ◽  
Abdominoperineal Resection ◽  
Mucinous Adenocarcinoma ◽  
Rare Case ◽  
Anorectal Fistula ◽  
Computed Tomography Scan ◽  
Perineal Abscess ◽  
Tomography Scan

Mucinous adenocarcinoma in association with chronic anal fistula is a rare case in clinical practice. The aim of this article was to report a rare case of anal gland mucinous adenocarcinoma in a patient who was treated in the Hospital of Kaunas University of Medicine. Case report. A 70-year-old male was treated for anorectal fistula in the surgical department. Four operations were performed for perineal abscess during the period of 15 years. During the period of 15 years, the patient complained of purulent secretion from the perineal abscess. After the last operation, anorectal fistula developed. Multiple biopsies and scrapings of the fistulous track were taken for histological examination. Histological examination revealed mucinous adenocarcinoma, G2. Subsequently, the patient underwent endoanal ultrasound, computed tomography scan, and colonoscopy. The computed tomography scan did not show pathology in the abdomen, but showed soft tissue induration at the site of anorectal fistula. Colonoscopy investigation did not show any pathology in the rectum and bowels. Endoanal ultrasound findings showed soft tissue induration at the site of anorectal fistula, no tumor in the rectum wall. The patient underwent abdominoperineal resection. Histological examination after abdominoperineal resection revealed anal duct mucinous adenocarcinoma pT2 N0 L0 V0 R0, G2. Metastases to the mesenteric lymph nodes were not detected. On the eight day after abdominoperineal resection, the patient was discharged from the hospital for follow-up. Summary. Mucinous adenocarcinoma in anorectal fistula is a rare condition. If surgical treatment for perineal abscess or anorectal fistula is not successful for a long time, mucinous adenocarcinoma should be suspected.

Gastric-type endocervical glandular neoplasms associated with aberrant p16 expression and K-RAS gene mutation in Peutz-Jeghers syndrome

2014 ◽  
Vol 64 (6) ◽  
pp. 283-288 ◽  
Author(s):  
Shigemi Ito ◽  
Toru Tase ◽  
Kennichi Satoh ◽  
Miyuki Ueki ◽  
Ikuro Sato ◽  
...  
Keyword(s):  
Gene Mutation ◽  
Ras Gene ◽  
Gastric Type ◽  
Peutz Jeghers Syndrome ◽  
P16 Expression

scholarly journals Massive intussusception caused by a solitary Peutz–Jeghers type hamartomatous polyp

2018 ◽  
Vol 100 (4) ◽  
pp. e91-e93 ◽  
Author(s):  
V Kalliakmanis ◽  
I Perysinakis ◽  
K Koutsouvas ◽  
P Karras ◽  
E Margaris ◽  
...  
Keyword(s):  
Intestinal Obstruction ◽  
Preoperative Diagnosis ◽  
Rare Case ◽  
Hamartomatous Polyp ◽  
Genetic Investigation ◽  
Diagnostic Challenge ◽  
Postoperative Course ◽  
Presenting Symptoms ◽  
Intestinal Polyp ◽  
Peutz Jeghers Syndrome

Intussusception is a rare cause of intestinal obstruction in adults and represents a diagnostic challenge for the surgeon. In the majority of cases, presenting symptoms are not specific, making preoperative diagnosis difficult. Several medical conditions may cause intestinal intussusception. We present the case of a 16-year-old female patient with intussusception due to a hamartomatous Peutz–Jeghers type polyp. This is an extremely rare case in which the first manifestation of the intestinal polyp was jejunojejunal intussusception very close to the duodenojejunal junction, with a necrotic intussusceptum about 50 cm long. The patient was treated successfully with enterectomy and end-to-end anastomosis. Postoperative course was uneventful and the patient is currently under gastroenterological and genetic investigation to exclude the diagnosis of Peutz–Jeghers syndrome.

scholarly journals A rare case of synchronous colocolic intussusception in association with Peutz–Jeghers syndrome

2017 ◽  
Vol 3 (1) ◽  
pp. 20150314
Author(s):  
Om Biju Panta ◽  
Santosh Maharjan ◽  
Sujan Manandhar ◽  
Sharma Paudel ◽  
Ram Kumar Ghimire
Keyword(s):  
Rare Case ◽  
Peutz Jeghers Syndrome

scholarly journals A rare case of metachronous penile and urethral metastases from a rectal mucinous adenocarcinoma

2015 ◽  
pp. bcr2015212706 ◽  
Author(s):  
Michelle Christodoulidou ◽  
Varun Sahdev ◽  
Asif Muneer ◽  
Raj Nigam
Keyword(s):  
Mucinous Adenocarcinoma ◽  
Rare Case

scholarly journals Case Report: Mucinous Adenocarcinoma Arising From Congenital Ejaculatory Duct Cyst

2021 ◽  
Vol 27 ◽  
Author(s):  
Hua Shen ◽  
Kai Liao ◽  
Weili Wu ◽  
Gongyu Li ◽  
Shijin Chen ◽  
...  
Keyword(s):  
Case Report ◽  
Embryonic Development ◽  
Mucinous Adenocarcinoma ◽  
Rare Case ◽  
Development Process ◽  
Ejaculatory Duct ◽  
Pathological Examination ◽  
Resected Specimen ◽  
Duct Cyst ◽  
Evidenced Based

Herein we present a previously unreported rare case of mucinous adenocarcinoma arising from a congenital ejaculatory duct cyst. Radiographic and endoscopic examinations revealed the tumor occurred in a cyst running through the prostate. Initially, the immunohistochemical pathology results showed that it was a metastatic mucinous adenocarcinoma, but no other primary lesions were clinically evidenced. Based on the embryonic development process of the male urogenital tract, the malformation of the patient's ejaculatory duct, and the pathological examination of the resected specimen, we considered the tumor to be a primary mucinous adenocarcinoma which originating from the hypoplastic ejaculatory duct. The tumor may have developed from the foci of intestinal metaplasia from cloacal remnants during embryonic development.

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