990
Images for surgeons
7. Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Pers. Vasc. Surg. Endovasc. Ther. 2005; 17: 187–206. 8. Sharma BK, Jain S, Bali HK, Jain A, Kumari S. A follow-up study of balloon angioplasty and de-novo stenting in Takayasu arteritis. Int. J. Cardiol. 2000; 75 (Suppl. 1): S147–52.
Kapilamoorthy Tirur Raman,† MBBS, MD Unnikrishnan Madathipat,* MS, MCh *Cardiovascular and Thoracic Surgery and †Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
Ajay Savlania,* MBBS, MS Shashidhar Kallappa Parameshwarappa,* MBBS, MS Sidharth Viswanathan,* MBBS, MS
doi: 10.1111/ans.12358
Schwannoma of the sigmoid colon: a rare cause of sigmoidorectal intussusception A 25-year-old man presented complaining of 1 week history of bloody mucoid diarrhoea and abdominal pain. He had associated anorexia, nausea, vomiting and abdominal distension with a 10-lb weight loss. Although subjective fevers and chills were reported, the patient was afebrile and haemodynamically stable. He had hypoactive bowel sounds with a soft, moderately distended abdomen and mild tenderness to palpation in the lower quadrants without peritonitis. Digital rectal examination was unremarkable. Proctoscopy revealed bloody mucoid stool, hyperemic and friable proximal rectal mucosa without active bleeding, and no obvious mass. This examination was limited due to poor visualization, but the findings were thought to be consistent with an inflammatory process. Laboratory studies were significant for mild electrolyte abnormalities and leukocytosis with a white blood cell count of 11 400/mm3. Stool studies were without abnormalities. Abdominal series revealed multiple loops of large and small bowel with air-fluid levels and paucity of air in the distal rectum. Computed tomography of the abdomen and pelvis with oral and intravenous contrast demonstrated dilated loops of small and large bowel with multiple airfluid levels and markedly thickened bowel wall at the rectosigmoid area with secondary inflammatory changes. He was diagnosed with proctosigmoiditis and a partial bowel obstruction. This was initially managed with complete bowel rest and intravenous antibiotics. Subsequent colonoscopy revealed an obstructing rectal mass at approximately 9 cm from the anal verge. The colonoscope was unable to be passed beyond the mass but multiple biopsies were obtained. Pathologic examination of the specimen was non-diagnostic. The carcinoembryonic antigen level was within normal limits. Repeated abdominal and pelvis computed tomography, which now included rectal contrast, revealed thickened bowel wall at the rectosigmoid junction with a targetoid pattern suspicious for colonic intussusception. Given this obstructing lesion, the patient underwent exploratory laparotomy at which time dilated loops of small and large bowel were encountered with a transition point at the rectosigmoid junction. This obstructing transition point was caused by an intussusception of the sigmoid colon into the rectum. Rectosigmoid resection without reduction of the intussusception, Hartmann’s pouch and an end colostomy were performed. Ex vivo examination revealed a 4 × 3 × 3 cm haemorrhagic firm mass within the
distal sigmoid colon that had intussuscepted into the rectum (Figs 1,2). Based on immunohistochemical stains, this mass was found to be a schwannoma. C-kit and CD34 stains were negative, excluding the diagnosis of gastrointestinal stromal tumour. Smooth muscle actin and desmin were also negative, discounting the diagnosis of a leiomyoma/leiomyosarcoma. Positive immunoreactivity for S100 indicated neuroectodermal origin of the mass (Fig. 3). This particular schwannoma was consistent with Antoni type A histology with densely packed spindle cells. A mitotic index stain was also performed which showed low malignant potential. The surgical margins
Fig. 1. Gross specimen: origin of intussusception at the sigmoid colon (single arrow), sigmoid schwannoma lead point (double arrow) and cut edge of the rectum (arrow head).
© 2013 Royal Australasian College of Surgeons
Images for surgeons
Fig. 2. Gross specimen: transection of the sigmoid schwannoma (double arrow head).
991
the neural sheath.2 They share a wide range of common histological and immunohistochemical characteristics with leiomyomas or leiomyosarcomas. Therefore, special stains are often required to differentiate schwannoma from leiomyoma/leiomyosarcoma.3 They occur most frequently in the small bowel and sigmoid schwannomas are extremely rare.1–5 The most common colorectal locations of schwannomas, in decreasing order of frequency, are the cecum, sigmoid, rectosigmoid, transverse colon, descending colon and rectum.4 Patients with distal colonic or rectal schwannomas frequently present with symptoms of rectal bleeding, colonic obstruction and abdominal pain. Like schwannomas, adult intussusceptions are uncommon accounting for only 5–16% of all intussusceptions and are responsible for less than 1% of adult intestinal obstructions.6 They are associated with primary or secondary neoplasms in 20–50% of cases.7 In the adult large bowel, the most common lead point of an intussusception is a lipoma originating from the cecum.8 Therefore, it is distinctly unusual for a schwannoma to serve as a lead point in an intussusception. In this case, an infrequent adult intussusception was caused by an equally rare sigmoid schwannoma. The prognosis for this patient is good as most colorectal schwannomas behave benignly without aggressive growth or metastasis. Radical excision with disease-free margins is the standard treatment.2 As with colorectal schwannomas, patients with adult intussusceptions also have a good prognosis. This patient had two unusual findings that were successfully managed surgically.
References
Fig. 3. S100 immunohistochemical stain of the mass demonstrating diffuse positivity.
were negative. The patient was discharged on the fifth post-operative day after an uncomplicated hospital course and subsequently had the colostomy reversed. Schwannomas are part of the family of the gastrointestinal autonomic nerve tumours. Gastrointestinal autonomic nerve tumours account for 0.1% of benign tumours of the gastrointestinal tract and are a subgroup of gastrointestinal stromal tumour.1 Schwannomas constitute only 2–6% of these stromal tumours and are derived from © 2013 Royal Australasian College of Surgeons
1. Maciejewski A, Lange D, Wloch J. Case report of schwannoma of the rectum – clinical and pathological contribution. Med. Sci. Monit. 2000; 6: 779–82. 2. Fotiadis CI, Kouerinis IA, Papandreou I, Zografos GC, Agapitos G. Sigmoid schwannoma: a rare case. World J. Gastroenterol. 2005; 11: 5079–81. 3. Mulchandani MH, Chattopadhyay D, Obafunwa JO, Joypaul VB. Gastrointestinal autonomic nerve tumors – report of a case and review of literature. World J. Surg. Oncol. 2005; 3: 46–53. 4. Miettinen M, Shekitka KM, Sobin LH. Schwannomas in the colon and rectum: a clinicopathologic and immunohistochemical study of 20 cases. Am. J. Surg. Pathol. 2001; 25: 846–55. 5. Lev D, Kariv Y, Messer GY, Isakov J, Gutnam M. Gastrointestinal autonomic nerve (GAN) tumor of the rectum. J. Clin. Gastroenterol. 2000; 30: 438–40. 6. Medina-Franco H, Ramos-de la Medina A, Arista-Nasr J. Intussusception in adults. Case report with uncommon etiology and review of the literature. Rev. Gastroenterol. Mex. 2002; 67: 103–6. 7. Azar T, Berger DL. Adult intussusception. Ann. Surg. 1997; 226: 134–8. 8. Shah RK, Shaw SA, Marroquin S et al. Adult intussusception as a cause of abdominal symptoms: a case report and review of literature. Emerg. Radiol. 2003; 10: 53–6.
Beverley A. Petrie, MD Joyce M. Ho, MD Amy M. Tolan, MD Department of Surgery, Section of Colon and Rectal Surgery, Harbor-UCLA Medical Center, Torrance, California, USA doi: 10.1111/ans.12364
Copyright of ANZ Journal of Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Images for surgeons
7. Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Pers. Vasc. Surg. Endovasc. Ther. 2005; 17: 187–206. 8. Sharma BK, Jain S, Bali HK, Jain A, Kumari S. A follow-up study of balloon angioplasty and de-novo stenting in Takayasu arteritis. Int. J. Cardiol. 2000; 75 (Suppl. 1): S147–52.
Kapilamoorthy Tirur Raman,† MBBS, MD Unnikrishnan Madathipat,* MS, MCh *Cardiovascular and Thoracic Surgery and †Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
Ajay Savlania,* MBBS, MS Shashidhar Kallappa Parameshwarappa,* MBBS, MS Sidharth Viswanathan,* MBBS, MS
doi: 10.1111/ans.12358
Schwannoma of the sigmoid colon: a rare cause of sigmoidorectal intussusception A 25-year-old man presented complaining of 1 week history of bloody mucoid diarrhoea and abdominal pain. He had associated anorexia, nausea, vomiting and abdominal distension with a 10-lb weight loss. Although subjective fevers and chills were reported, the patient was afebrile and haemodynamically stable. He had hypoactive bowel sounds with a soft, moderately distended abdomen and mild tenderness to palpation in the lower quadrants without peritonitis. Digital rectal examination was unremarkable. Proctoscopy revealed bloody mucoid stool, hyperemic and friable proximal rectal mucosa without active bleeding, and no obvious mass. This examination was limited due to poor visualization, but the findings were thought to be consistent with an inflammatory process. Laboratory studies were significant for mild electrolyte abnormalities and leukocytosis with a white blood cell count of 11 400/mm3. Stool studies were without abnormalities. Abdominal series revealed multiple loops of large and small bowel with air-fluid levels and paucity of air in the distal rectum. Computed tomography of the abdomen and pelvis with oral and intravenous contrast demonstrated dilated loops of small and large bowel with multiple airfluid levels and markedly thickened bowel wall at the rectosigmoid area with secondary inflammatory changes. He was diagnosed with proctosigmoiditis and a partial bowel obstruction. This was initially managed with complete bowel rest and intravenous antibiotics. Subsequent colonoscopy revealed an obstructing rectal mass at approximately 9 cm from the anal verge. The colonoscope was unable to be passed beyond the mass but multiple biopsies were obtained. Pathologic examination of the specimen was non-diagnostic. The carcinoembryonic antigen level was within normal limits. Repeated abdominal and pelvis computed tomography, which now included rectal contrast, revealed thickened bowel wall at the rectosigmoid junction with a targetoid pattern suspicious for colonic intussusception. Given this obstructing lesion, the patient underwent exploratory laparotomy at which time dilated loops of small and large bowel were encountered with a transition point at the rectosigmoid junction. This obstructing transition point was caused by an intussusception of the sigmoid colon into the rectum. Rectosigmoid resection without reduction of the intussusception, Hartmann’s pouch and an end colostomy were performed. Ex vivo examination revealed a 4 × 3 × 3 cm haemorrhagic firm mass within the
distal sigmoid colon that had intussuscepted into the rectum (Figs 1,2). Based on immunohistochemical stains, this mass was found to be a schwannoma. C-kit and CD34 stains were negative, excluding the diagnosis of gastrointestinal stromal tumour. Smooth muscle actin and desmin were also negative, discounting the diagnosis of a leiomyoma/leiomyosarcoma. Positive immunoreactivity for S100 indicated neuroectodermal origin of the mass (Fig. 3). This particular schwannoma was consistent with Antoni type A histology with densely packed spindle cells. A mitotic index stain was also performed which showed low malignant potential. The surgical margins
Fig. 1. Gross specimen: origin of intussusception at the sigmoid colon (single arrow), sigmoid schwannoma lead point (double arrow) and cut edge of the rectum (arrow head).
© 2013 Royal Australasian College of Surgeons
Images for surgeons
Fig. 2. Gross specimen: transection of the sigmoid schwannoma (double arrow head).
991
the neural sheath.2 They share a wide range of common histological and immunohistochemical characteristics with leiomyomas or leiomyosarcomas. Therefore, special stains are often required to differentiate schwannoma from leiomyoma/leiomyosarcoma.3 They occur most frequently in the small bowel and sigmoid schwannomas are extremely rare.1–5 The most common colorectal locations of schwannomas, in decreasing order of frequency, are the cecum, sigmoid, rectosigmoid, transverse colon, descending colon and rectum.4 Patients with distal colonic or rectal schwannomas frequently present with symptoms of rectal bleeding, colonic obstruction and abdominal pain. Like schwannomas, adult intussusceptions are uncommon accounting for only 5–16% of all intussusceptions and are responsible for less than 1% of adult intestinal obstructions.6 They are associated with primary or secondary neoplasms in 20–50% of cases.7 In the adult large bowel, the most common lead point of an intussusception is a lipoma originating from the cecum.8 Therefore, it is distinctly unusual for a schwannoma to serve as a lead point in an intussusception. In this case, an infrequent adult intussusception was caused by an equally rare sigmoid schwannoma. The prognosis for this patient is good as most colorectal schwannomas behave benignly without aggressive growth or metastasis. Radical excision with disease-free margins is the standard treatment.2 As with colorectal schwannomas, patients with adult intussusceptions also have a good prognosis. This patient had two unusual findings that were successfully managed surgically.
References
Fig. 3. S100 immunohistochemical stain of the mass demonstrating diffuse positivity.
were negative. The patient was discharged on the fifth post-operative day after an uncomplicated hospital course and subsequently had the colostomy reversed. Schwannomas are part of the family of the gastrointestinal autonomic nerve tumours. Gastrointestinal autonomic nerve tumours account for 0.1% of benign tumours of the gastrointestinal tract and are a subgroup of gastrointestinal stromal tumour.1 Schwannomas constitute only 2–6% of these stromal tumours and are derived from © 2013 Royal Australasian College of Surgeons
1. Maciejewski A, Lange D, Wloch J. Case report of schwannoma of the rectum – clinical and pathological contribution. Med. Sci. Monit. 2000; 6: 779–82. 2. Fotiadis CI, Kouerinis IA, Papandreou I, Zografos GC, Agapitos G. Sigmoid schwannoma: a rare case. World J. Gastroenterol. 2005; 11: 5079–81. 3. Mulchandani MH, Chattopadhyay D, Obafunwa JO, Joypaul VB. Gastrointestinal autonomic nerve tumors – report of a case and review of literature. World J. Surg. Oncol. 2005; 3: 46–53. 4. Miettinen M, Shekitka KM, Sobin LH. Schwannomas in the colon and rectum: a clinicopathologic and immunohistochemical study of 20 cases. Am. J. Surg. Pathol. 2001; 25: 846–55. 5. Lev D, Kariv Y, Messer GY, Isakov J, Gutnam M. Gastrointestinal autonomic nerve (GAN) tumor of the rectum. J. Clin. Gastroenterol. 2000; 30: 438–40. 6. Medina-Franco H, Ramos-de la Medina A, Arista-Nasr J. Intussusception in adults. Case report with uncommon etiology and review of the literature. Rev. Gastroenterol. Mex. 2002; 67: 103–6. 7. Azar T, Berger DL. Adult intussusception. Ann. Surg. 1997; 226: 134–8. 8. Shah RK, Shaw SA, Marroquin S et al. Adult intussusception as a cause of abdominal symptoms: a case report and review of literature. Emerg. Radiol. 2003; 10: 53–6.
Beverley A. Petrie, MD Joyce M. Ho, MD Amy M. Tolan, MD Department of Surgery, Section of Colon and Rectal Surgery, Harbor-UCLA Medical Center, Torrance, California, USA doi: 10.1111/ans.12364
Copyright of ANZ Journal of Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
