McKittrick Wheelock syndrome secondary to adenocarcinoma of the rectum: case report and diagnostic considerations

Introduction: McKittrick-Wheelock syndrome is a rare entity characterized by chronic diarrhea, acute kidney injury, and hydroelectrolytic imbalance associated with a large rectal tumor, frequently a villous adenoma. Case report: A 69-year-old male with chronic diarrhea with mucus. He underwent a colonoscopy with biopsies, reporting adenocarcinoma of the rectum in situ, and underwent a robot assisted intersphincteric resection with colo-anal anastomosis and a protecitive ileostomy. Discussion: Described in 1954, this syndrome is manifested by electrolyte imbalance and acute renal injury secondary to diarrhea associated with a rectal villous adenoma, often with long lasting symptoms. The most frequent symptom being watery diarrhea with mucus. The definitive treatment consists of surgical resection. Conclusion: Although this is a rare pathology, it should be considered as a differential diagnosis in cases of chronic diarrhea associated with water and electrolyte disorders. (AU)

Adenoma velloso de esófago en la mucosa ectópica gástrica: reporte de un caso / Villous adenoma of the esophagus in ectopic gastric mucosa: A case report

Resumen Introducción: Los adenomas vellosos son lesiones del tubo digestivo con tendencia alta a la malignidad. Su ubicación en parches de mucosa gástrica ectópica en el esófago cervical determina una presentación atípica de interés tanto clínico como patológico. Objetivo: Presentar un caso de adenoma velloso en la mucosa gástrica ectópica del esófago cervical. Métodos: Describir el caso de un paciente con diagnóstico de adenoma velloso y realizar una revisión de la literatura disponible hasta el momento. Resultados: Se identificó mediante un estudio histopatológico un adenoma tubulovelloso con displasia de bajo grado, cuya ubicación endoscópica era un parche de mucosa ectópica gástrica en el esófago cervical. Conclusiones: La ubicación de los adenomas vellosos en el esófago cervical puede predisponer al desarrollo de lesiones neoplásicas. La evaluación detallada de este segmento, con técnicas como la cromoendoscopia digital de alta definición, permiten la detección temprana de estas lesiones y su oportuna intervención.

Abstract Introduction: Villous adenomas are lesions of the digestive tract with a high tendency to malignancy. Its location in ectopic gastric mucosa patches in the cervical esophagus is an atypical presentation of clinical and pathological interest. Objective: To present a case of villous adenoma in ectopic gastric mucosa of the cervical esophagus. Methods: A case study of a patient with a diagnosis of villous adenoma is presented, as well as a review of the current literature. Results: A tubulovillous adenoma with low-grade dysplasia was identified by histopathological study. Its endoscopic location was a gastric ectopic mucosa patch in the cervical esophagus. Conclusions: The location of villous adenomas in the cervical esophagus may predispose to the development of neoplastic lesions. Detailed evaluation of this segment using techniques, such as high-definition digital chromoendoscopy, would allow for early detection and treatment of these lesions.

Tubulovillous adenoma of the duodenal papilla: radiological-endoscopic and anatomopathological correlation in the surgical proposal

SUMMARY Tubulovillous adenomas of the duodenal ampulla are rare neoplasms. The present report describes a case with radiological-endoscopic and pathological correlation in which the patient underwent duodenal pancreatectomy with good postoperative progression. With advanced imaging methods, especially magnetic resonance and endoscopic ultrasound, locoregional aspects and extraluminal, lymphovascular, and metastatic invasion have been increasingly discussed as contributors to therapeutic decision making. This progression improves lesion staging and is especially useful in selecting eligible candidates for endoscopic treatment.

RESUMO Os adenomas túbulo-vilosos da ampola duodenal são neoplasias raras. Neste trabalho apresentamos um caso com correlação radiológico-endoscópica e patológica, tendo a paciente sido submetida à duodenopancreatectomia com boa evolução pós-operatória. Com os avanços dos métodos de imagem, em especial da ressonância magnética e ultrassonografia endoscópica, aspectos locorregionais, além da invasão extraluminal, linfovascular e metastática, têm sido discutidos de maneira crescente como contribuintes na decisão terapêutica. Essa evolução contribui para o melhor estadiamento destas lesões e é especialmente útil para selecionar candidatos elegíveis ao tratamento endoscópico.

Prolapso rectal incarcerado secundario a adenoma velloso gigante / Incarcerated rectal prolapse due to a giant villous adenoma

Los adenomas vellosos pueden manifestarse con sangrado, diarrea, alteraciones hidroelectrolíticas (síndrome Mackittrick-Weelock), obstrucción intestinal, y, muy infrecuentemente, condicionar un prolapso rectal. El prolapso rectal es la protrusión de la pared completa del recto a través del canal anal, su presentación como prolapso rectal incarcerado es poco habitual. Cuando la reducción manual no es posible, la rectosigmoidectomía vía perineal o procedimiento de Altemeier es una buena opción quirúrgica; como alternativa puede realizarse una resección transanal del pólipo y la posterior reducción manual del prolapso rectal. Referiremos el caso de una mujer que acude al Servicio de urgencias presentando un prolapso rectal incarcerado con una masa ulcerada, friable, de 10 × 8 × 5 cm compatible con un pólipo velloso en la cara posterior del recto. Ante la imposibilidad de reducirlo se decide una resección transanal del pólipo con posterior reducción manual del prolapso rectal. Este caso es de interés por la infrecuente asociación entre un prolapso rectal incarcerado y un pólipo velloso gigante, con solo 4 casos comunicados en la literatura.

Villous adenomas may present with bleeding, diarrhea, electrolyte imbalance (Mackittrick-Weelock syndrome), obstruction, being a very rare cause of rectal prolapse. Rectal prolapse is a full thickness protrusion of the rectum through the anal canal and its presentation as an incarcerated rectal prolapse is very infrequent. If manual reduction is deemed impossible, perineal recto-sigmoidectomy, or Altemeier's procedure, is one of the best surgical options, as an alternative transanal excision of the polyp could be performed with subsequent manual reduction of the rectal prolapse. We report the case of a female patient, admitted to the emergency room presenting an incarcerated rectal prolapse with a friable ulcerated mass of 10 × 8 × 5 cm, compatible with a villous polyp in the back side of the rectum. Since manual reduction was considered not feasible, surgery was decided and a transanal excision of the polyp was performed, following a successful manual reduction of the rectal prolapse. This case is of particular interest for its unusual association of incarcerated rectal prolapse due to a giant villous adenoma, having only 4 cases been reported in the literature.

Adenoma Velloso de Estómago: Presentación de un caso / Gastric Villous Adenoma: One case presentation

Los adenomas vellosos son una patología infrecuente en el tubo digestivo y muy especialmente en el estómago. Presentamos un caso de adenoma velloso de estómago en una paciente de 73 años. Morfológicamente correspondió a una lesión de 1.5cm tipo ip al cual le realizamos resección mucosal endoscópica y la cual histológicamente fue descrita como una lesión de tipo velloso. La paciente presentó hemorragia en el sitio de la lesión posterior al tratamiento endoscópico y la cual fue tratada en forma satisfactoria con tratamiento endoscópico mínimamente invasivo a base de adrenalina y argón plasma.

Villous adenomas are uncommon pathology in the digestive tube and very especially in the stomach. We present a case of adenoma, villous stomach in a 73-year-old patient. Macroscopally corresponded to one ip type 1.5 cm lesion to which you we perform endoscopic mucosal resection and which histologically was described as a type villous injury. The patient presented bleeding at the site of the lesion after the endoscopic treatment and which was treated satisfactorily with endoscopic treatment minimally invasive based on adrenaline and argon plasma.

Resección transanal de un adenoma velloso rectal a través de un trocar monopuerto / Excision of a rectal villous adenoma using a transanal single port trocar

Single port access for minimally invasive surgery is feasible nowadays. We report a 55 years old female presenting with hematochezia. During a colonoscopy, a villous adenoma of 2 cm in diameter located 5 cm above the anal margin, was found. This lesion was excised through the anus, using a single port trocar. The procedure lasted 45 minutes, the postoperative evolution was uneventful and the patient was discharged 24 hours after the procedure.

Posterior al advenimiento de la cirugía laparoscópica colorrectal, desde comienzos de la década de los 90, se han desarrollado diversos tipos de accesos minimamente invasivos, siendo hoy en día el acceso por un puerto único una alternativa factible. Se presenta el caso clínico de una paciente de 55 años, a la cual por sintomatología se le diagnóstica una lesión rectal, la que luego de ser estudiada endoscópica y endosonográficamente, es resecada por monopuerto via anal. La cirugía duró 45 minutos y no presentó eventos adversos intraoperatorios. Su evolución quirúrgica fue satisfactoria con alta hospitalaria al día siguiente de la cirugía.

Uso del dispositivo SILS™ en cirugía transanal mínimamente invasiva para el manejo de lesiones benignas de recto / Using the device SILS ™ Transanal minimally invasive surgery for the management of benign rectal

Minimally invasive transanal microsurgery was described in 2009. It allows the excision polyps or small malignant lesions. We report a 73 years old female consulting for hematochezia. A sessile polyp located 9 cm above the anal margin was discovered during a colonoscopy that corresponded to a villous adenoma. The polyp was excised transanally, using a SILS* port for transanal endoscopic microsurgery.

La cirugía transanal mínimamente invasiva fue descrita inicialmente en el año 2009 como un abordaje híbrido para lograr la resección endoluminal de lesiones rectales, surgiendo como una alternativa más económica al uso del TEM (Transanal endoscopic microsurgery). La resección transanal de pólipos, así como de pequeñas lesiones malignas rectales con instrumentos laparoscópicos y de puerto único, utiliza la tecnología desarrollada inicialmente para procedimientos desde un orificio natural como es el recto. Nuestra intención es presentar la técnica quirúrgica, así como su aplicación en la resección de lesiones benignas como malignas del recto no realizables a través de una resección transanal convencional.

A case of giant rectal villous tumor with severe fluid-electrolyte imbalance treated by laparoscopic low anterior resection / 대한외과학회지

McKittrick-Wheelock syndrome is a disorder caused by fluid and electrolyte hypersecretion from a colorectal tumor. To present the case of a patient with a giant rectal villous tumor with McKittrick-Wheelock syndrome who was successfully treated with laparoscopic surgery. The case of a 59-year-old man who came to the emergency department with syncope, prerenal azotemia, and electrolyte disturbances with a background of chronic diarrhea is reported. His condition was the result of fluid and electrolyte hypersecretion caused by rectal villotubular adenomas. Laparoscopic low anterior resection and subsequent volume and electrolyte replacement therapy resulted in complete recovery. A microscopic examination revealed multiple, well-differentiated adenocarcinomas arising in villotubular adenomas. Laparoscopic surgical resection is a feasible therapeutic modality for McKittrick-Wheelock syndrome.

A Case of Colorectal Villous Adenoma Found with PET-CT / 대한소화기내시경학회지

Adenomatous polyp in colorectal area is clinically important as being a precursor of colorectal cancer. Early detection of cancer reduces colorectal cancer mortality. Finding and removing precursor adenomas reduces colorectal cancer incidence especially in high risk groups. FDG-PET/CT is a new imaging technology in which a number of clinical applications has been recognized in oncologic imaging. FDG-PET has been shown to detect a wide variety of tumor foci including lymphoma, melanoma, lung cancer and colorectal cancer. Colorectal adenoma can also be detected by FDG-PET. In this case, we describe a colorectal villous adenoma, which was found by FDG-PET/CT. There was a mass of intensely increased FDG uptake in rectal area. The adenoma was confirmed with sigmoidoscopy and removed by polypectomy.

Adenoma viloso com transformação carcinomatosa da ampola de Vater / Villous adenoma with carcinomatous degeneration of the ampulla of Vater

Villous adenomas of the duodenum and ampulla of Vater are uncommon, but they have been diagnosed more frequently with the increasing use of upper gastrointestinal endoscopy. Differential diagnosis with villous adenocarcinoma may be difficult. The authors present a case of a 47-year-old man with a giant villous adenoma of the duodenum, with intermittent jaundice, that was treated by pancreatoduodenectomy.

A Villous Adenoma with Foci of Adenocarcinoma Arising in the Common Hepatic Duct / 대한소화기내시경학회지

Villous adenoma of the extrahepatic duct is a rare disease. Even though it is benign, it has malignant potential and is considered a premalignant lesion. We here report one case of villous adenoma with foci of adenocarcinoma arising in the common hepatic duct. A 60-year-old male was admitted to our hospital because of epigastric pain. The cholangiogram revealed a large filling defect in the right intrahepatic and common hepatic duct with intrahepatic bile duct dilatation. The patient underwent right lobectomy. Grossly, stalked papillay tumor originated in the common hepatic duct was overriding the right intrahepatic duct. Microscopically, the tumor was composed of stratified tall columnar cells with various dysplasia and there were foci of invasive adenocarcinoma.

Villous adenoma of the bile ducts: a case report and a review of the reported cases in Korea

Villous adenomas are benign epithelial lesions with malignant potential which can occur at any site in the gastrointestinal tract. They are usually encountered in the rectum and colon, less frequently in the small bowel and very rarely in the biliary trees. Nine cases of bile duct villous adenomas have been reported in the literature. However, 4 cases of bile duct villous adenomas have been reported in the Korean literature. Recently, we experienced a case of villous adenoma in the common hepatic duct in a 77-year-old man presenting with obstructive jaundice in which preoperative histologic diagnosis of villous adenoma played a critical role in managing this patient. Herein, we present a case report of bile duct villous adenoma and a review of the reported cases in Korea to help define and manage this rare disease entity in the bile ducts. In addition, confusing nomenclature of bile duct adenomas is discussed.

Surgical Review of the Rectal Villous Adenoma

PURPOSE: This study was undertaken to evaluate retrospectively the clinical results of surgery for the rectal villous adenoma. METHODS: The study took place from the period of Mar. of 1988 to Feb. 1998 at the Dept. of Surgery, Sungkyunkwan Univ., Medical college. The study consisted of : Among the 97 cases diagnosed with colon & rectal villous adenoma, and 59 were rectal villous adenoma, and 42 cases underwent resection. We focused on these 42 cases, especially on the position of tumor, its size, surgical technique, histologic results & keeping close follow up post-surgically. RESULTS: The sex ratio was 2:1 with male predominence, 18 cases were in their fifties with the average age of 55. In 30 cases, the lesion was situated within the 8 cm of anal verge. The average size of tumor was 3.64 cm. The applied methods were; rectotomy 19 cases, anterior resection 13 cases (including low anterior resection), endoscopic excision and transanal excision were 7 cases, and 2 cases, respectively. And in one case where the tumor size was 14.5 cm and which was situated within 3 cm of anal verge, Miles' operation was conducted. Death due to post-operative complication was not observed. 2 cases of wound infection in postoperation, one case of transient urinary incontinence & hematoma were found. And in the case of explo-laparotomy, anastomosis site leakage, in one case, anastomosis site stricture in 2 cases were noted. malignant cells were observed in total of 73.8%, among these, 80% were from villous adenoma, 70% from tubullovillous adenoma. In the case of tumor size less 1 cm, and tumor size greater than 1 cm, the probability of finding malignant lesion were 33.3% and 76.9%, respectively. CONCLUSIONS: We suggest that wideexcision through York-Mason approach is a safe and effective technique for huge villous adenomas of the rectum.

A Case of Villous Adenoma with Depletion Syndrome / 대한소화기내시경학회지

Massive secretory diarrhea with pre-renal insufficiency, hyponatremia, hypokalemia and metabolic alkalosis or acidosis is associated with some large villous adenomas of the rectum and is called with depletion syndrome. This characteristic fluid and electrolyte depletion syndrome is caused by secretion of sodium, potassium, and fluid from the tumor. PGE2 formation in the villous adenoma appears to be the cause of fluid secretion by the abnormal tumor epithelium. Surgical removal of villous adenoma is the only promising therapy, In case of inoperability, denial of surgical intervention or just for palliative treatment prior to surgery, the use of PG synthetase inhibitors may facilitate the correction of severe fluid-electrolyte deficits. We reported a case of large villous adenoma of the rectum with depletion syndrome aceompanied by secretory diarrhea and fluid and electrolyte depletion with metabolic alkalosis due to severe vomiting.

Tubulovillous and Villous Adenomas of the Colon and Rectum - Endoscopic Characteristics and Management / 대한소화기내시경학회지

BACKGROUND/AIMS: A villous tumor, histologically villous or tubulovillous adenoma, is a clinical challenge because of its higher potential for malignancy and higher recurrence rate. However, information and experience with these tumors in the Korean people is still lacking. For that reason, we designed this study to review and analyze the colonoscopic features, the potential for malignancy, and the treatiment with respect to the confirmation of guidelines for the accurate diagnosis and reasonable management of such tumors in the Korean population. MATERIALS AND METHODS: We performed 753 polypectomies, including 4 transanal excisions and several bowel resections, from January 1996 to May 1997 at Song-Do Colorectal Hospital in Seoul, Among them, 447 cases (59.4%) were adenomas, comprising 405 (53.8%) tubular adenomas, 31 (4.1%) tubulovillous adenomas, and 11 (1.5%) villous adenomas. We analyzed the 42 (5.6%) tubulovillous and villous adenomas.

Two Cases of Villous Adenoma of the Common Bile Duct: Endoscopic Diagnosis and Treatment / 대한소화기내시경학회지

Villous adenomas of the common bile duct are rare and accordingly it is difficult to render a firm preoperative diagnosis. They are the unusual cause of bile duct obstruction and can mimic malignant extrahepatic biliary tumors or stones. Until recently, only a few cases had been reported in the medical literature. Although some authors advocate open surgical excision, especially with recurrence or carcinomatous change, an endoscopic resection of a distal common bile duct adenoma is a viable alternative for those patients considered poor surgical risks. We present two cases of villous adenoma of the common bile duct diagnosed by an endoscopic biopsy and endoscopically resected, with review of the relevant literature on the subject.

A Case of Villous Adenomas in the Common Bile Duct and Cystic Duct / 대한내과학회지

Benign tumors of the extrahepatic bile duct are rare. They occur mostly in the common bile duct. Among them, papillomas and adenomas are most common. We experienced a case of villous adenomas in the common bile duct and also in the cystic duct. The ultrasound and the CT scan of the abdomen revealed markedly dilated common bile duct, subtle filling defect, and irregular mass narrowing the lumen of the common bile duct. The cholangiogram demonstrated movable, feathery, and marginated papillary mass with tumors, a broad base, and biopsy of this mass was done through the PTBD. The biopsy specimen showed villous adenoma. Even though it was diagnosed as benign, cholecystectomy and excision with Roux-en-Y hepaticojejunostomy was performed because of the high incidence of recurrences and malignant potential.

Pathologic Review of the Rectal Villous Adenoma

Neoplastic polyps of colon are one of the most risky factors for colorectal cancer. Particularly villous adenomas have more malignant potential than other neoplastic polyps. The preoperative detection of malignant change in villous adenoma is very important to determine treatment modality of patients. We conducted that total twenty-four cases of villous adenoma who were diagnosed and treated at our institution between January 1990 and December 1995 were reviewed retrospectively. The peak incidence of age was 7th decades. And male to female ratio was 2 : 1. Size of the adenoma ranged from 1.2 to 10.7 cm with a mean diameter of 3.9 cm. Five cases(20.8%) were between 1 and 2 cm, 10(41.7%) between 2 and 4 cm, and 9(31.5%) greater than or equal to 4 cm. Location of the adenoma were 10 cases(41.7%) in upper rectum, 8(33.3%) in rectosigmoid, and 6(25.0%) in lower rectum respectively. Pathologic examination of the resected specimen were diagnosed 9(37.5%) as benign villous adenomas, 10(41.7%) as in situ carcinoma, and 5(20.8%) as invasive carcinoma. The diagnostic accuracy to detect in situ carcinoma or invasive carcinoma was endoscopic examination in 66.7 percent and digital examination in 86.7 percent. The grade of in situ carcinoma had not significantly relationship with the size of the adenoma on our study. However, invasive carcinoma was significantly related to adenoma size(P<0.05) as following results zero percent between 1 and 2 cm, 20 percent between 2 and 4 cm, and 33.3 percent greater than or equal to 4 cm. In conclusion, the larger villous adenoma has higher malignant potential, especially greater than or equal to 4 cm in size. Added, the clinical impression of the malignancy on digital examination can be more accurate than preoperative endoscopic examination on our study.

Mucin - secreting Villous Adenoma of The Common Hepatic Duct Causing Mucoid Biliary Obstruction / 대한소화기내시경학회지

Obstructive jaundice of the bile duct resulting from tumor-producing copious, thick mucin, causing ductal obstruction and dilatation, has been reported very rarely. Also, the benign bile duct neoplasm is extremely rare. We report a case of a mucin-secreting villous adenoma of common hepatic duct causing obstructive jaundice and cholangitis. The patient was a 72-year-old male and had a 3 yeared history of common hepatic mass unchanged remarkably in size. The abdominal ultrasonogram and computed tomography revealed well circumscribed, more than 2cm sized mass on the CHD and ductal dilatation. We were able to get an interesting cholangioram showing irregular, nodular, ill defined filling defect in the extrahepatic bile duct, different from US and CT findings, And it was just caused by thick, copious mucin from the tumar. This case was confirmed as mucin-secreting villous adenoma after surgery.

A Case of Villous Adenoma of the Ampulla of Vater with Malignant Change / 대한소화기내시경학회지

Ampullary adenoma is a benign neoplasm with malignant potential that arises from the glandular epithelium of the ampulla of Vater. When the tumor is confined to the ampulla, abdominal sonogram and CT scan can show dilatation of the common bile duct or pancreatic duct, but the mass itself may not be seen. And even biopsies are done, the confirmation of malignant change is frequently missed, and it may be impossible to assess the presence of carcinoma in situ or invasive carcinoma without complete exicision of the lesion. So complete surgical resection is recommended because of extensive growth of the ampullary adenoma and its malignant potential. Recently, we experienced a case of about 0.8 x 1.0 cm sized tumor of the ampulla of Vater that was diagnosed as villous adenoma on endoscopic biopsy, and malignant change was found on resected surgical specimen.