scholarly journals Giant adrenal cavernous hemangioma in a patient with familial adenomatous polyposis

2016 ◽  
Vol 6 (3) ◽  
Author(s):  
Dhouha Bacha ◽  
Abir Chaabane ◽  
Fatma Khanchel ◽  
Saloua Néchi ◽  
Hassen Touinsi ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Cavernous Hemangioma ◽  
Vascular Tumor ◽  
Extensive Study ◽  
Pathological Examination ◽  
Adenomatous Polyposis ◽  
Extracolonic Manifestations ◽  
Radiologic Evaluation ◽  
History Of ◽  
Patient Series

Adrenal hemangioma is an uncommon benign vascular tumor that is often discovered incidentally. It has never been reported in association with familial adenomatous polyposis. We report a case of a 60-year old man with a history of familial adenomatous polyposis, in whom a huge retroperitoneal cyst of 18×17 cm was discovered during routine radiologic evaluation. Because of the impossibility of ruling out the presence of malignancy, surgical cystectomy was performed, associated to a scheduled total colectomy. Pathological examination revealed that the cyst corresponded to an adrenal cavernous hemangioma. Colonic adenomas did not show signs of degeneration. Screening for adenomatous polyposis coli (<em>APC</em>) gene mutation was not carried out. As familial adenomatous polyposis is known to involve a variety of extracolonic manifestations, this finding raises the suspicion of a possible variant of this syndrome including adrenal hemangioma. An extensive study based on a larger patient series with genetic exploration is necessary.

scholarly journals Solid-pseudopapillary neoplasm of the pancreas in a patient with familial adenomatous polyposis: a case report

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Daishi Naoi ◽  
Koji Koinuma ◽  
Hideki Sasanuma ◽  
Yasunaru Sakuma ◽  
Hisanaga Horie ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Pancreatic Tumor ◽  
Laparoscopic Resection ◽  
Tumor Development ◽  
Colonic Polyps ◽  
Pancreatic Tumors ◽  
Beta Catenin ◽  
Adenomatous Polyposis ◽  
Solid Pseudopapillary Neoplasm ◽  
Extracolonic Manifestations

Abstract Background Familial adenomatous polyposis (FAP) is characterized by the presence of hundreds to thousands of colonic polyps, and extracolonic manifestations are likely to occur. Pancreatic tumors are rare extracolonic manifestations in patients with FAP, among which solid-pseudopapillary neoplasm (SPN) are extremely rare. We report here a patient with an SPN of the pancreas found during the follow-up of FAP. Case presentation A 20-year-old woman was diagnosed with FAP 3 years previously by colonoscopy which revealed less than 100 colonic polyps within the entire colon. She complained of left upper abdominal pain and a 10-cm solid and cystic pancreatic tumor was found by computed tomography scan. Solid and cystic components within the tumor were seen on abdominal magnetic resonance imaging. Simultaneous laparoscopic resection of the distal pancreas and subtotal colectomy was performed. Histopathological findings confirmed the pancreatic tumor as an SPN without malignancy. Abnormal staining of beta-catenin was observed by immunohistochemical study. Multiple polyps in the colorectum were not malignant. Molecular biological analysis from peripheral blood samples revealed a decrease in the copy number of the promoter 1A and 1B region of the APC gene, which resulted in decreased expression of the APC gene. Conclusions A rare association of SPN with FAP is reported. The genetic background with relation to beta-catenin abnormalities is interesting to consider tumor development. So far, there are few reports of SPN in a patient with FAP. Both lesions were treated simultaneously by laparoscopic resection.

ID: 3524040 THE NATURAL HISTORY OF AMPULLARY ADENOMAS IN FAMILIAL ADENOMATOUS POLYPOSIS SYNDROME: LONG-TERM FOLLOW-UP

2021 ◽  
Vol 93 (6) ◽  
pp. AB350
Author(s):  
Achintya D. Singh ◽  
Amit Bhatt ◽  
Abel Joseph ◽  
Neal Mehta ◽  
Gautam N. Mankaney ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Natural History ◽  
Adenomatous Polyposis ◽  
Polyposis Syndrome ◽  
Long Term Follow Up ◽  
History Of

scholarly journals A rare case of cavernous hemangioma accompanied with diffuse hepatic hemangiomatosis

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takuji Ota ◽  
Toshiya Kamiyama ◽  
Takuya Kato ◽  
Takayuki Hanamoto ◽  
Kunihiro Hirose ◽  
...  
Keyword(s):  
Oral Contraceptives ◽  
Cavernous Hemangioma ◽  
Contraceptive Use ◽  
Careful Examination ◽  
Pathological Examination ◽  
Surgical Removal ◽  
Adjacent Area ◽  
Oral Contraceptive Use ◽  
History Of ◽  
Magnetic Resonance Imaging Mri

Abstract Background Hepatic cavernous hemangioma (CH) is the most common hepatic benign tumor. Most cases are solitary, asymptomatic, and found incidentally. In symptomatic cases with rapidly growing tumors and coagulopathy, surgical treatment is considered. In rare cases, diffuse hepatic hemangiomatosis (DHH) is reported as a comorbidity. The etiology of DHH is unknown. Case presentation A 29-year-old female patient had a history of endometriosis treated with oral contraceptives. Hepatic CH was incidentally detected in the segment IVa of the liver according to the Couinaud classification. Follow-up computed tomography (CT) and ultrasound sonography showed the growth of the lesion and formation of multiple new lesions near the first. Enhanced CT and magnetic resonance imaging (MRI) revealed that the new lesions were different from CH. Although oral contraceptives were stopped, all lesions grew in size. Malignancy and possibility of rupture of these tumors were considered due to the clinical course, and we opted for surgical removal of the tumors. Left liver lobectomy and cholecystectomy were performed. Surgical findings were small red spot spreading and a mass in segment IV of the liver. Pathological examination revealed a circumscribed sponge-like tumor with diffuse irregular extension to the adjacent area. Both of the lesions consisted of blood-filled dilated vascular spaces lined by flat endothelium without atypia. The diagnosis was hepatic CH with DHH. The patient was discharged on postoperative day 12 uneventfully. Conclusion We report the successful resection of CH with DHH. The case findings suggest a relationship between oral contraceptive use and enlargement of CH and DHH. Although DHH has been poorly understood, a few previously published cases reported DHH occurrence in patients using oral contraceptives. In such cases, the decision to perform surgical resection should be made after careful examination.

Natural history of ampullary adenoma in familial adenomatous polyposis: reconfirmation of benign nature during extended surveillance

2000 ◽  
Vol 95 (6) ◽  
pp. 1557-1562 ◽  
Author(s):  
Takayuki Matsumoto ◽  
Mitsuo Iida ◽  
Shotaro Nakamura ◽  
Kazuoki Hizawa ◽  
Takashi Yao ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Natural History ◽  
Adenomatous Polyposis ◽  
Ampullary Adenoma ◽  
History Of ◽  
Benign Nature

scholarly journals Inducible loss of one Apc allele in Lrig1-expressing progenitor cells results in multiple distal colonic tumors with features of familial adenomatous polyposis

2014 ◽  
Vol 307 (1) ◽  
pp. G16-G23 ◽  
Author(s):  
Anne E. Powell ◽  
Gregory Vlacich ◽  
Zhen-Yang Zhao ◽  
Eliot T. McKinley ◽  
M. Kay Washington ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Progenitor Cells ◽  
Pigment Epithelium ◽  
Retinal Pigment ◽  
Tumor Formation ◽  
Therapeutic Interventions ◽  
Adenomatous Polyposis ◽  
Extracolonic Manifestations ◽  
Predictable Pattern ◽  
Leucine Rich Repeats

Individuals with familial adenomatous polyposis (FAP) harbor a germline mutation in adenomatous polyposis coli ( APC). The major clinical manifestation is development of multiple colonic tumors at a young age due to stochastic loss of the remaining APC allele. Extracolonic features, including periampullary tumors, gastric abnormalities, and congenital hypertrophy of the retinal pigment epithelium, may occur. The objective of this study was to develop a mouse model that simulates these features of FAP. We combined our Lrig1-CreERT2/+ mice with Apcfl/+ mice, eliminated one copy of Apc in leucine-rich repeats and immunoglobulin-like domains protein 1 (Lrig1)-positive (Lrig1+) progenitor cells with tamoxifen injection, and monitored tumor formation in the colon by colonoscopy and PET. Initial loss of one Apc allele in Lrig1+ cells results in a predictable pattern of preneoplastic changes, culminating in multiple distal colonic tumors within 50 days of induction, as well as the extracolonic manifestations of FAP mentioned above. We show that tumor formation can be monitored by noninvasive PET imaging. This inducible stem cell-driven model recapitulates features of FAP and offers a tractable platform on which therapeutic interventions can be monitored over time by colonoscopy and noninvasive imaging.

Pancreaticoduodenectomy for Dysplastic Duodenal Adenoma in a Patient with Familial Adenomatous Polyposis

2008 ◽  
Vol 94 (6) ◽  
pp. 882-884
Author(s):  
Roberto Merenda ◽  
Giuseppe Portale ◽  
Francesca Galeazzi ◽  
Chiara Tosolini ◽  
Giacomo Carlo Sturniolo ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Colonic Polyps ◽  
Clinical History ◽  
Adenomatous Polyposis ◽  
Duodenal Adenoma ◽  
Colorectal Polyposis ◽  
Duodenal Polyps ◽  
Younger Age ◽  
History Of ◽  
Malignant Lesions

Colorectal polyposis is the main feature of familial adenomatous polyposis (FAP), but benign and malignant lesions have also been described in the stomach, duodenum, small bowel, biliary tract and pancreas. There are few reports on FAP patients with duodenal polyps that developed at a younger age and even fewer on cases with dysplastic degeneration. The progression to carcinoma usually presents quite late in the clinical history of FAP patients, typically at least 20 to 25 years after proctocolectomy. This report described the rare case of a patient presenting with duodenal adenomas with dysplastic changes and tumor infiltration as the first sign of FAP, who was treated by pancreaticoduodenectomy followed by proctocolectomy for subsequent dysplastic changes in colonic polyps.

Morphology and natural history of familial adenomatous polyposis-associated dysplastic fundic gland polyps

2014 ◽  
Vol 65 (3) ◽  
pp. 353-362 ◽  
Author(s):  
Thomas Arnason ◽  
Wen-Yih Liang ◽  
Eduardo Alfaro ◽  
Paul Kelly ◽  
Daniel C Chung ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Natural History ◽  
Fundic Gland ◽  
Adenomatous Polyposis ◽  
Fundic Gland Polyps ◽  
History Of

scholarly journals Familial Adenomatous Polyposis Coli in East Africa: A Case Report and Review of the Literature

2020 ◽  
pp. 74-80
Author(s):  
Richard Wismayer
Keyword(s):  
Family History ◽  
Familial Adenomatous Polyposis ◽  
East Africa ◽  
Adenomatous Polyposis Coli ◽  
Treatment Options ◽  
Adenomatous Polyposis ◽  
Polyposis Coli ◽  
Strong Family History ◽  
Familial Adenomatous Polyposis Coli ◽  
History Of

Introduction: Familial adenomatous polyposis (FAP) is a rare diagnosis in East Africa. The author reports a case of a 21 year old gentleman presenting with occasional passage of blood stained stool, and found to have familial adenomatous polyposis coli. This is followed by a literature review on the pathogenesis, clinical features and treatment options of FAP in East Africa. Presentation of Case: This patient presented with a strong family history of familial adenomatous polyposis, blood stained stool and a rectal mass. A total proctocolectomy and ileoanal anastomosis was carried out. The postoperative course of this patient was uneventful. Discussion: The typical gross pathological and histological features of familial adenomatous polyposis and rectal adenocarcinoma were seen on the resected colorectal specimen. In addition this study reviews the literature regarding the clinical presentation, pathological characteristics and treatment options of familial adenomatous polyposis coli. Conclusion: FAP should always be considered in a young patient presenting with a strong family history of CRC. Colonoscopy should be performed on these patients with early symptoms and those patients with a strong family history of FAP. In East Africa, the creation of a permanent stoma is unacceptable and therefore a proctocolectomy and Brooke ileostomy will not be a desirable option in a young patient in this part of the world.

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