IMAGES FOR SURGEONS ANZJSurg.com
Sigmoid malignancy presenting as recto-sigmoid intussusception This case describes a young woman presenting with symptoms of large bowel obstruction secondary to intussusception, because of a malignant tumour acting as the lead point. A 36-year-old woman presented to the Accident and Emergency Department with a 6-day history of bleeding per rectum, constipation, lower abdominal pain, distension, nausea and vomiting, without prior symptoms. She had a family history of colorectal cancer as her father had died from the disease aged 54. Abdominal examination revealed a tense, tender abdomen without signs of peritonism, and digital rectal examination revealed a firm, smoothsurfaced mass palpable 5 cm from the anal verge, with bright red blood on the glove. Rigid sigmoidoscopy visualised an intussusceptum, characterized by a smooth-surfaced, circumferential, bloodstained mass, consistent with the digital findings. A computed tomography (CT) scan with intravenous and oral contrast confirmed an intussusception, seen as a ‘target sign’ (Fig. 1). A malignant cause for the intussusception was considered at this point thus the patient proceeded to laparotomy. Intraoperatively, the sigmoid colon was confirmed to have intussuscepted into the rectum, and the lead point was unable to be reduced. Oncological resection of the sigmoid and adjacent lymph nodes was performed, with a high tie of the inferior mesenteric artery. Once the ultra-low anterior resection with loop ileostomy was completed, an on-table colonic lavage was undertaken. Histopathological analysis revealed a 2.1-cm adenocarcinoma at the lead point (Fig. 2), extending to the serosal surface and being poorly differentiated with 14/27 lymph nodes containing metastatic disease, including the apical node. In addition, there was a mesenteric tumour
and lymphovascular invasion. The hereditary non-polyposis colorectal cancer immunohistochemical tumour staining was normal. The rectal mucosa involved in the intussusception showed patchy ulcerative and ischaemic changes (Fig. 3). Intussusception occurs when a proximal section of bowel with its mesentery telescopes or invaginates into the adjacent distal bowel.1 It occurs when a mass in the bowel lumen acts as a lead point and is pulled forward by normal peristalsis. Intussusception is more common in children, presenting with a classic triad of colicky abdominal pain, bloody diarrhoea and a palpable tender mass. Bowel intussusception in adults is a rare condition,2,3 but can present as bowel obstruction. The lead point is documented as malignant in roughly 60–80% of cases.4 Primary bowel cancers and lymphomas account for the majority of malignant causes of intussusception. Submucosal lipomas are the most common benign cause of colonic intussusception in adults, with adenomatous polyps and leiomyomas also contributing to cases.1,4 Rarer causes of intussusception can be secondary to functional loss of the bowel without any mural abnor-
Fig. 2. Pathology picture of tumour.
Fig. 1. Computed tomography picture. Characteristic concentric ring soft tissue mass indicating sigmoid telescoping into the rectum ‘target sign’ (arrow).
ANZ J Surg 84 (2014) 386–393
Fig. 3. Pathology picture of bowel.
© 2013 Royal Australasian College of Surgeons
Images for surgeons
mality, such as coeliac disease resulting in flaccid bowel.1,5 However, this does not usually present as large bowel obstruction. Iatrogenic causes for intussusception, such as intestinal tubes or jejenostomy feeding tubes, have also been reported. CT is the most sensitive test for investigating intussusception, which is usually seen as the pathognomonic ‘target sign’ (Fig. 1). Although a diagnosis of intussusception can be suspected radiologically, it is often difficult to identify the lead point or cause. Malignancy should be suspected in an adult, and the CT may also demonstrate additional pathology such as metastases. Although radiological reduction using therapeutic enemas is often successful in young children, adults with obstructive symptoms require operative management, as a lesion is usually acting as a lead point and more than half of these prove to be malignant. The lead point in itself is not usually visible at sigmoidoscopy or colonoscopy, and biopsies can demonstrate normal mucosa, having come from the intussusceptum rather than the lead point lesion. In summary, bowel intussusception is rare in adults and accounts for a minority of bowel obstructions, with a ‘target sign’ on abdominal CT being pathognomonic. Many prove to be malignant, even in
387
young patients thus operative management with an oncological resection should be considered early on.
References 1. Gayer G, Zissin R, Apter S, Papa M, Hertz M. Adult intussusception – a CT diagnosis. Br. J. Radiol. 2002; 75: 185–90. 2. Marinis A, Yiallourou A, Samonides L et al. Intussusception of the bowel in adults: a review. World J. Gastroenterol. 2009; 15: 407–11. 3. Wang N, Cui XY, Liu Y et al. Adult Intussusception: a retrospective review of 41 cases. World J. Gastroenterol. 2009; 15: 3303–8. 4. Begos DG, Sandor A, Modlin IM. The diagnosis and management of adult intussusception. Am. J. Surg. 1997; 173: 88–94. 5. Agha FP. Intussusception in adults. AJR Am. J. Roentgenol. 1986; 146: 527–31.
Gayatri Asokan,* MBBS Paul Hollington,† MBBS, PhD, FRACS *General Surgery and †Colorectal Surgery, Flinders Medical Centre, Adelaide, South Australia, Australia doi: 10.1111/ans.12232
Lipoblastoma: an interesting differential of paediatric lipoma A 12-month-old boy was referred to the ear, nose and throat clinic at a tertiary hospital in Melbourne, Australia, for a large right side neck lump. The lump had been present for the last six months and was clinically consistent with a lipoma, which was supported by ultrasound. The lump had been observed for 6 months, but increased in size in the preceding 2 months, prompting referral. The child had no other medical problems and was otherwise well and thriving. On examination, there was a large mobile, subcutaneous soft mass in the right posterior triangle, with no compressive airway symptoms or signs, normal accessory nerve function and no torticollis. There were no other findings on examination of the head and neck and no other lipomas identified. Magnetic resonance imaging (MRI) performed under general anaesthetic showed features consistent with lipoma, revealing a 3.6 × 3 × 4.8-cm lobular mass deep to sternocleidomastoid in the right side of the neck, with fatty signal and enhancing septa passing throughout (Fig. 1). The patient was discussed at a multidisciplinary meeting and underwent surgery to remove the mass, which further increased in size prior to the operation. The accessory nerve was pushed up superficially by the underlying tumour, but was protected during the operation, as the large well-encapsulated tumour was removed intact (Fig. 2). There were no complications and the patient was discharged on day 2, post-operatively. Histopatholgy revealed a lesion measuring 5.7 × 5.2 × 3.3 cm, resembling brown fat macroscopically. Microscopic evaluation showed a well-circumscribed lesion divided into lobules containing a mixture of fat cells divided by bands of fibrous tissue and skeletal muscle. The majority resembled mature adipocytes and a minority had an immature signet ring and multi-vacuolated cytoplasmic appearance. There were scant regions of myxoid stroma. No mitotic © 2013 Royal Australasian College of Surgeons
Fig. 1. T1 sagittal magnetic resonance imaging showing lobular mass right side neck posterior triangle deep to sternocleidomastoid.
figures or atypical cells were identified. A diagnosis of lipoblastoma was made and there was no adjuvant treatment given. Lipoblastomas are rare, rapidly growing, benign neoplasms derived from embryonic white fat. They are most commonly seen in the trunk and extremities and are rare in the head and neck.1 80% to 90% of cases present before the age of three years, with up to 40%
Sigmoid malignancy presenting as recto-sigmoid intussusception This case describes a young woman presenting with symptoms of large bowel obstruction secondary to intussusception, because of a malignant tumour acting as the lead point. A 36-year-old woman presented to the Accident and Emergency Department with a 6-day history of bleeding per rectum, constipation, lower abdominal pain, distension, nausea and vomiting, without prior symptoms. She had a family history of colorectal cancer as her father had died from the disease aged 54. Abdominal examination revealed a tense, tender abdomen without signs of peritonism, and digital rectal examination revealed a firm, smoothsurfaced mass palpable 5 cm from the anal verge, with bright red blood on the glove. Rigid sigmoidoscopy visualised an intussusceptum, characterized by a smooth-surfaced, circumferential, bloodstained mass, consistent with the digital findings. A computed tomography (CT) scan with intravenous and oral contrast confirmed an intussusception, seen as a ‘target sign’ (Fig. 1). A malignant cause for the intussusception was considered at this point thus the patient proceeded to laparotomy. Intraoperatively, the sigmoid colon was confirmed to have intussuscepted into the rectum, and the lead point was unable to be reduced. Oncological resection of the sigmoid and adjacent lymph nodes was performed, with a high tie of the inferior mesenteric artery. Once the ultra-low anterior resection with loop ileostomy was completed, an on-table colonic lavage was undertaken. Histopathological analysis revealed a 2.1-cm adenocarcinoma at the lead point (Fig. 2), extending to the serosal surface and being poorly differentiated with 14/27 lymph nodes containing metastatic disease, including the apical node. In addition, there was a mesenteric tumour
and lymphovascular invasion. The hereditary non-polyposis colorectal cancer immunohistochemical tumour staining was normal. The rectal mucosa involved in the intussusception showed patchy ulcerative and ischaemic changes (Fig. 3). Intussusception occurs when a proximal section of bowel with its mesentery telescopes or invaginates into the adjacent distal bowel.1 It occurs when a mass in the bowel lumen acts as a lead point and is pulled forward by normal peristalsis. Intussusception is more common in children, presenting with a classic triad of colicky abdominal pain, bloody diarrhoea and a palpable tender mass. Bowel intussusception in adults is a rare condition,2,3 but can present as bowel obstruction. The lead point is documented as malignant in roughly 60–80% of cases.4 Primary bowel cancers and lymphomas account for the majority of malignant causes of intussusception. Submucosal lipomas are the most common benign cause of colonic intussusception in adults, with adenomatous polyps and leiomyomas also contributing to cases.1,4 Rarer causes of intussusception can be secondary to functional loss of the bowel without any mural abnor-
Fig. 2. Pathology picture of tumour.
Fig. 1. Computed tomography picture. Characteristic concentric ring soft tissue mass indicating sigmoid telescoping into the rectum ‘target sign’ (arrow).
ANZ J Surg 84 (2014) 386–393
Fig. 3. Pathology picture of bowel.
© 2013 Royal Australasian College of Surgeons
Images for surgeons
mality, such as coeliac disease resulting in flaccid bowel.1,5 However, this does not usually present as large bowel obstruction. Iatrogenic causes for intussusception, such as intestinal tubes or jejenostomy feeding tubes, have also been reported. CT is the most sensitive test for investigating intussusception, which is usually seen as the pathognomonic ‘target sign’ (Fig. 1). Although a diagnosis of intussusception can be suspected radiologically, it is often difficult to identify the lead point or cause. Malignancy should be suspected in an adult, and the CT may also demonstrate additional pathology such as metastases. Although radiological reduction using therapeutic enemas is often successful in young children, adults with obstructive symptoms require operative management, as a lesion is usually acting as a lead point and more than half of these prove to be malignant. The lead point in itself is not usually visible at sigmoidoscopy or colonoscopy, and biopsies can demonstrate normal mucosa, having come from the intussusceptum rather than the lead point lesion. In summary, bowel intussusception is rare in adults and accounts for a minority of bowel obstructions, with a ‘target sign’ on abdominal CT being pathognomonic. Many prove to be malignant, even in
387
young patients thus operative management with an oncological resection should be considered early on.
References 1. Gayer G, Zissin R, Apter S, Papa M, Hertz M. Adult intussusception – a CT diagnosis. Br. J. Radiol. 2002; 75: 185–90. 2. Marinis A, Yiallourou A, Samonides L et al. Intussusception of the bowel in adults: a review. World J. Gastroenterol. 2009; 15: 407–11. 3. Wang N, Cui XY, Liu Y et al. Adult Intussusception: a retrospective review of 41 cases. World J. Gastroenterol. 2009; 15: 3303–8. 4. Begos DG, Sandor A, Modlin IM. The diagnosis and management of adult intussusception. Am. J. Surg. 1997; 173: 88–94. 5. Agha FP. Intussusception in adults. AJR Am. J. Roentgenol. 1986; 146: 527–31.
Gayatri Asokan,* MBBS Paul Hollington,† MBBS, PhD, FRACS *General Surgery and †Colorectal Surgery, Flinders Medical Centre, Adelaide, South Australia, Australia doi: 10.1111/ans.12232
Lipoblastoma: an interesting differential of paediatric lipoma A 12-month-old boy was referred to the ear, nose and throat clinic at a tertiary hospital in Melbourne, Australia, for a large right side neck lump. The lump had been present for the last six months and was clinically consistent with a lipoma, which was supported by ultrasound. The lump had been observed for 6 months, but increased in size in the preceding 2 months, prompting referral. The child had no other medical problems and was otherwise well and thriving. On examination, there was a large mobile, subcutaneous soft mass in the right posterior triangle, with no compressive airway symptoms or signs, normal accessory nerve function and no torticollis. There were no other findings on examination of the head and neck and no other lipomas identified. Magnetic resonance imaging (MRI) performed under general anaesthetic showed features consistent with lipoma, revealing a 3.6 × 3 × 4.8-cm lobular mass deep to sternocleidomastoid in the right side of the neck, with fatty signal and enhancing septa passing throughout (Fig. 1). The patient was discussed at a multidisciplinary meeting and underwent surgery to remove the mass, which further increased in size prior to the operation. The accessory nerve was pushed up superficially by the underlying tumour, but was protected during the operation, as the large well-encapsulated tumour was removed intact (Fig. 2). There were no complications and the patient was discharged on day 2, post-operatively. Histopatholgy revealed a lesion measuring 5.7 × 5.2 × 3.3 cm, resembling brown fat macroscopically. Microscopic evaluation showed a well-circumscribed lesion divided into lobules containing a mixture of fat cells divided by bands of fibrous tissue and skeletal muscle. The majority resembled mature adipocytes and a minority had an immature signet ring and multi-vacuolated cytoplasmic appearance. There were scant regions of myxoid stroma. No mitotic © 2013 Royal Australasian College of Surgeons
Fig. 1. T1 sagittal magnetic resonance imaging showing lobular mass right side neck posterior triangle deep to sternocleidomastoid.
figures or atypical cells were identified. A diagnosis of lipoblastoma was made and there was no adjuvant treatment given. Lipoblastomas are rare, rapidly growing, benign neoplasms derived from embryonic white fat. They are most commonly seen in the trunk and extremities and are rare in the head and neck.1 80% to 90% of cases present before the age of three years, with up to 40%
