Clinical Imaging 38 (2014) 205–207
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Duodenal adenocarcinoma presenting as a mass with aneurismal dilatation Nadia Mama a,⁎, Aïda Ben Slama b, Nadia Arifa a, Khaled Kadri a, Badreddine Sriha c, Mehdi Ksiaa b, Hela Jemni a, Kalthoum Tlili-Graiess a a b c
Radiology Department, Sahloul Hospital, Route Ceinture, 4011 Sousse, Tunisia Gastroenterology Department, Sahloul Hospital, Route Ceinture, 4011 Sousse, Tunisia Pathology Department, CHU Farhat HACHED, Rue Ibn Jazzar, 4031 Ezzouhour Sousse, Tunisia
a r t i c l e
i n f o
Article history: Received 10 June 2013 Received in revised form 17 September 2013 Accepted 11 November 2013 Keywords: Duodenal adenocarcinoma CT Barium meal follow-through Aneurysmal dilatation
a b s t r a c t Duodenal adenocarcinoma is frequent. Aneurysmal dilatation of the small bowel is reported to be a lymphoma characteristic imaging finding. A 57-year-old male was found to have a duodenal adenocarcinoma with aneurismal dilatation on imaging which is an exceptional feature. On laparotomy, the wall thickening of the dilated duodenum extended to the first jejunal loop, with multiple mesenteric lymph nodes and ascites. Segmental palliative resection with gastro-entero-anastomosis was done. Histopathology revealed a moderately differentiated adenocarcinoma with neuro-endocrine differentiation foci. Wide areas of necrosis and vascular emboli were responsible for the radiological feature of the dilated duodenum with wall thickening. © 2014 Elsevier Inc. All rights reserved.
1. Introduction Aneurysmal dilatation of the small bowel is defined as an abnormal focal dilatation of the small bowel loop contrasting with associated wall thickening or a bowel mass. It is reported to be a characteristic imaging finding of lymphoma. Duodenal adenocarcinoma is frequent and appears typically as a focal area of wall thickening. We report an exceptional case of adenocarcinoma with neuroendocrine differentiation that caused aneurysmal dilatation of the duodenum and the first jejunal loop. To our best knowledge, this is the first report of such presentation. 1.1. Case report A 57-year-old-male who underwent a prostate adenomectomy 4years ago presented with a two months history of epigastralgia and vomiting associated with weight loss and weakness. On physical examination, the patient was pale and there was a large palpable fixed mass in the epigastrium extending in the left upper abdomen. Blood analysis revealed iron-deficiency anemia at 4 g/100 ml [13–16 g/100 ml]. Upper gastro-intestinal endoscopy followed by biopsies showed isolated ulcerative budding tumor in the second portion of the duodenum. This endoscopy did not reach the third portion of the duodenum. Histopathology of biopsied material revealed a moderately differentiated adenocarcinoma. Contrast-enhanced computed tomography (CT) demonstrated a large tumor with aneurismal ⁎ Corresponding author. Tel.: +216 24 01 74 34; fax: +216 73 36 74 51. E-mail address: [email protected] (N. Mama). 0899-7071/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clinimag.2013.11.004
dilatation and a significant bowel wall thickening that involved the distal third portion of the duodenum and the duodenojejunal angle (Fig. 1a and b). The second duodenum lesion was not depicted since it was ulcerative and not enough thickened to be clear on CT. Numerous mesenteric lymph nodes and abundant ascites were noted (Fig. 1b, c). Barium meal follow-through confirmed the duodenojejunal dilatation with an irregular mucosal pattern and a large, and irregular central cavity (Fig. 2). Radiological exams suspected a lymphoma or a gastrointestinal stromal tumor. On surgery, the extensive wall thickening of the dilated third portion of the duodenum along with the first jejunal loop were confirmed, with an important exophytic extraluminal mass that was originally considered by the surgeon as a magma of lymph nodes fistulized in the duodenal lumina. Multiple mesenteric lymph nodes were observed; only a palliative resection in one piece of the second, third duodenum and the first jejuna loop was performed with gastro-entero anastomosis. Histopathology demonstrated a moderately differentiated adenocarcinoma with neuro-endocrine differentiation foci, as well as wide areas of necrosis and rare vascular emboli (Fig. 3). The extra-luminal mass had an intestinal wall. All the layers were invaded without extension beyond the serosa. The patient had a post-bleeding shock and died at the sixth day after surgery. 2. Discussion Adenocarcinoma is the most common primary malignancy of the small intestine, accounting for 40% of primary small bowel neoplasms with the duodenum as the most frequently involved site [1].
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appearance of an apple core is occasionally observed [2]. Because of the rigid and fibrotic feature, these tumors induce early obstruction. This case associates two contiguous moderately differentiated adenocarcinomas of the duodenum which were separated by a safe interval. The first was ulcerative and quite typical although it was not depicted on imaging; the second was with aneurysmatic feature. Aneurysmal dilatation of the small bowel was first described as a typical imaging finding of lymphoma [3–7]. It has also been reported in other primary tumors such as leimyosarcoma, malignant GIST (gastrointestinal stromal tumors) [8,9] and in necrotic metastasis, first melanoma, followed by lung cancer, colon cancer and breast cancer [10,11]. Aneurysmal dilatation of the duodenum or the jejunum can be non tumoral related to focal amyloidosis with extracellular deposition of amyloid protein. Intestinal involvement without extraintestinal manifestations is rare and typically presents as multiple polypoid lesions or large amyloid tumors of the duodenum and jejunum [12,13]. Aneurismal pattern of adenocarcinoma is exceptional. This presentation can be explained by the extent of the original large mucosal tumor outside the lumen with extraluminal component. Cavitation has appeared secondarily induced by tumoral necrosis and leading to aneurismal dilatation appearance [10]. In the present case, the surgeon thought that the primary tumor is the ulcerative lesion located in the second portion of the duodenum and considered the tumoral dilated duodenum as a bulky metastatic lymphadenopathy that is widely fistulizing in the duodenum. Histopathology demonstrated that the extraluminal mass had an intestinal wall with tumoral invasion of all the intestinal layers. This observation supports the hypothesis that every extraluminal tumor that grows and becomes necrotic will form a cavity connected to the small bowel [10]. Other factors that may play a role in the development of aneurismal dilatation were described such as the fact that the tumor damages the autonomic myenteric nervous plexus in the tunica muscularis [14], or infiltrates the muscle fibers so they lose their
Fig. 1. Enhanced CT scan with axial thin slice (A) and coronal reformation (B): aneurismal pattern of the third duodenum (solid arrows) extended to the first jejunal loop with thickened wall (empty arrow). Note the lymph node on the upper pole of the tumor (thin arrow). Axial thin slice on the pelvic (C): ascites in the Douglas pouch (asterisk).
Adenocarcinoma appears typically as a focal area of wall thickening with “overhanging edges” causing luminal narrowing. Less frequently, it appears as a polypoid mass or an infiltrative lesion and a classic
Fig. 2. Small bowel follow-through study showing aneurismal dilatation of the third portion of the duodenum with an irregular mucosa pattern.
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Fig. 3. Photomicrographs of tumor cells showing (A) a highly cellular tumor with glandular differentiation and wide areas of hemorrhage (asterisk) (HE, original magnification ×100). (B) Solid area with abundant figures of mitoses (HE, original magnification ×200). (C) High and intensive expression of cytokeratin relevant to the epithelial nature of the tumor cells (cytokeratine immunostaining; original magnification ×200). (D) Moderate expression of chromogranine by tumor cells demonstrating their endocrine nature (chromogranine immunostaining; original magnification ×200).
normal contracting ability. Tumor cells can also invade and block lymphatic and vascular channels with resulting anoxia of the muscle fibers and of the epithelial lining with subsequent necrosis [15]. All these factors have been described as mechanisms for aneurismal dilatation in lymphoma. 3. Conclusion Aneurismal dilatation of the small bowel is characterized by a focally dilated small loop with thickened wall or bowel mass. It has been described initially as typical involvement of lymphoma. Although uncommon, this radiologic feature can be encountered in metastatic lesions and in leiomyosarcoma. It is rather exceptional for primary adenocarcinoma of the small bowel but it should be included as a differential diagnosis. References [1] Horton KM, Fishman EK. Multidetector-row computed tomography and 3dimensional computed tomography imaging of small bowel neoplasms: current concept in diagnosis. J Comput Assist Tomogr 2004;28:106–16. [2] Buckley JA, Fishman EK. CT evaluation of small bowel neoplasms: spectrum of disease. Radiographics 1998;18:379–92. [3] Zissin R, Osadchy A, Gayer G, Shapiro-Feinberg M. Pictorial review. CT of duodenal pathology. Br J Radiol 2002;75:78–84.
[4] Cupps RE, Hodgson JR, Dockerty MB, Adson MA. Primary lymphoma in the small intestine: problems of roentgenologic diagnosis. Radiology 1969;92:1354–62. [5] Laurent F, Raynaud M, Biset JM. Boisserie- Lacroix M, Grelet P, Drouillard J. Diagnosis and computerization of small bowel neoplasms: role of computed tomography. Gastrointest Radiol 1991;16:115–9. [6] Paulsen SR, Huprich JE, Fletcher JG, et al. CT enterography as a diagnostic tool in evaluating small bowel disorders: review of clinical experience with over 700 cases. Radiographics 2006;26:641–57. [7] Gore RM, Mehta UK, Berlin JW, Rao V, Newmark GM. Diagnosis and staging of small bowel tumours. Cancer Imaging 2006;6:209–12. [8] Sandrasegaran K, Rajesh A, Rydberg J, Rushing DA, Akisik FM, Henley JD. Gastrointestinal stromal tumors: clinical, radiologic, and pathologic features. AJR Am J Roentgenol 2005;184:803–11. [9] Kim W, Baek JM, Suh YJ, Jeon HM, Kim JA. Ileal malignant melanoma presenting as a mass with aneurysmal dilatation: a case report. J Korean Med Sci 2004;19: 297–301. [10] Pantongrag-Brown L. Aneurysmal dilatation of the small bowel. Thai J Gastroenterol 2009;10:111–3. [11] Maizlin ZV, Brown JA, Buckley MR, Filipenko D, Barnard SA, Wong X, et al. Case of the season: aneurysmal dilatation of the small bowel (not only lymphoma). Semin Roentgenol 2006;41:248–9. [12] Mainenti PP, Segreto S, Mancini M, Rispo A, Cozzolino I, Masone S, et al. Intestinal amyloidosis: two cases with different patterns of clinical and imaging presentation. World J Gastroenterol 2010;16:2566–70. [13] Saindane AM, Losada M, Macari M. Focal amyloidoma of the small bowel mimicking adenocarcinoma on CT. AJR Am J Roentgenol 2005;185:1187–9. [14] Paul Guillerman R. Primary Intestinal non-Hodgkin lymphoma. J Pediatr Hematol Oncol 2000;22:476–8. [15] Kinoshita T, Yashiro N, Yasuda R, O'uchi T, Narita M. Leiomyosarcoma presenting aneurysmal dilatation of the jejunum on CT. J Comput Assist Tomogr 2000;24: 421–2.
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Duodenal adenocarcinoma presenting as a mass with aneurismal dilatation Nadia Mama a,⁎, Aïda Ben Slama b, Nadia Arifa a, Khaled Kadri a, Badreddine Sriha c, Mehdi Ksiaa b, Hela Jemni a, Kalthoum Tlili-Graiess a a b c
Radiology Department, Sahloul Hospital, Route Ceinture, 4011 Sousse, Tunisia Gastroenterology Department, Sahloul Hospital, Route Ceinture, 4011 Sousse, Tunisia Pathology Department, CHU Farhat HACHED, Rue Ibn Jazzar, 4031 Ezzouhour Sousse, Tunisia
a r t i c l e
i n f o
Article history: Received 10 June 2013 Received in revised form 17 September 2013 Accepted 11 November 2013 Keywords: Duodenal adenocarcinoma CT Barium meal follow-through Aneurysmal dilatation
a b s t r a c t Duodenal adenocarcinoma is frequent. Aneurysmal dilatation of the small bowel is reported to be a lymphoma characteristic imaging finding. A 57-year-old male was found to have a duodenal adenocarcinoma with aneurismal dilatation on imaging which is an exceptional feature. On laparotomy, the wall thickening of the dilated duodenum extended to the first jejunal loop, with multiple mesenteric lymph nodes and ascites. Segmental palliative resection with gastro-entero-anastomosis was done. Histopathology revealed a moderately differentiated adenocarcinoma with neuro-endocrine differentiation foci. Wide areas of necrosis and vascular emboli were responsible for the radiological feature of the dilated duodenum with wall thickening. © 2014 Elsevier Inc. All rights reserved.
1. Introduction Aneurysmal dilatation of the small bowel is defined as an abnormal focal dilatation of the small bowel loop contrasting with associated wall thickening or a bowel mass. It is reported to be a characteristic imaging finding of lymphoma. Duodenal adenocarcinoma is frequent and appears typically as a focal area of wall thickening. We report an exceptional case of adenocarcinoma with neuroendocrine differentiation that caused aneurysmal dilatation of the duodenum and the first jejunal loop. To our best knowledge, this is the first report of such presentation. 1.1. Case report A 57-year-old-male who underwent a prostate adenomectomy 4years ago presented with a two months history of epigastralgia and vomiting associated with weight loss and weakness. On physical examination, the patient was pale and there was a large palpable fixed mass in the epigastrium extending in the left upper abdomen. Blood analysis revealed iron-deficiency anemia at 4 g/100 ml [13–16 g/100 ml]. Upper gastro-intestinal endoscopy followed by biopsies showed isolated ulcerative budding tumor in the second portion of the duodenum. This endoscopy did not reach the third portion of the duodenum. Histopathology of biopsied material revealed a moderately differentiated adenocarcinoma. Contrast-enhanced computed tomography (CT) demonstrated a large tumor with aneurismal ⁎ Corresponding author. Tel.: +216 24 01 74 34; fax: +216 73 36 74 51. E-mail address: [email protected] (N. Mama). 0899-7071/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.clinimag.2013.11.004
dilatation and a significant bowel wall thickening that involved the distal third portion of the duodenum and the duodenojejunal angle (Fig. 1a and b). The second duodenum lesion was not depicted since it was ulcerative and not enough thickened to be clear on CT. Numerous mesenteric lymph nodes and abundant ascites were noted (Fig. 1b, c). Barium meal follow-through confirmed the duodenojejunal dilatation with an irregular mucosal pattern and a large, and irregular central cavity (Fig. 2). Radiological exams suspected a lymphoma or a gastrointestinal stromal tumor. On surgery, the extensive wall thickening of the dilated third portion of the duodenum along with the first jejunal loop were confirmed, with an important exophytic extraluminal mass that was originally considered by the surgeon as a magma of lymph nodes fistulized in the duodenal lumina. Multiple mesenteric lymph nodes were observed; only a palliative resection in one piece of the second, third duodenum and the first jejuna loop was performed with gastro-entero anastomosis. Histopathology demonstrated a moderately differentiated adenocarcinoma with neuro-endocrine differentiation foci, as well as wide areas of necrosis and rare vascular emboli (Fig. 3). The extra-luminal mass had an intestinal wall. All the layers were invaded without extension beyond the serosa. The patient had a post-bleeding shock and died at the sixth day after surgery. 2. Discussion Adenocarcinoma is the most common primary malignancy of the small intestine, accounting for 40% of primary small bowel neoplasms with the duodenum as the most frequently involved site [1].
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appearance of an apple core is occasionally observed [2]. Because of the rigid and fibrotic feature, these tumors induce early obstruction. This case associates two contiguous moderately differentiated adenocarcinomas of the duodenum which were separated by a safe interval. The first was ulcerative and quite typical although it was not depicted on imaging; the second was with aneurysmatic feature. Aneurysmal dilatation of the small bowel was first described as a typical imaging finding of lymphoma [3–7]. It has also been reported in other primary tumors such as leimyosarcoma, malignant GIST (gastrointestinal stromal tumors) [8,9] and in necrotic metastasis, first melanoma, followed by lung cancer, colon cancer and breast cancer [10,11]. Aneurysmal dilatation of the duodenum or the jejunum can be non tumoral related to focal amyloidosis with extracellular deposition of amyloid protein. Intestinal involvement without extraintestinal manifestations is rare and typically presents as multiple polypoid lesions or large amyloid tumors of the duodenum and jejunum [12,13]. Aneurismal pattern of adenocarcinoma is exceptional. This presentation can be explained by the extent of the original large mucosal tumor outside the lumen with extraluminal component. Cavitation has appeared secondarily induced by tumoral necrosis and leading to aneurismal dilatation appearance [10]. In the present case, the surgeon thought that the primary tumor is the ulcerative lesion located in the second portion of the duodenum and considered the tumoral dilated duodenum as a bulky metastatic lymphadenopathy that is widely fistulizing in the duodenum. Histopathology demonstrated that the extraluminal mass had an intestinal wall with tumoral invasion of all the intestinal layers. This observation supports the hypothesis that every extraluminal tumor that grows and becomes necrotic will form a cavity connected to the small bowel [10]. Other factors that may play a role in the development of aneurismal dilatation were described such as the fact that the tumor damages the autonomic myenteric nervous plexus in the tunica muscularis [14], or infiltrates the muscle fibers so they lose their
Fig. 1. Enhanced CT scan with axial thin slice (A) and coronal reformation (B): aneurismal pattern of the third duodenum (solid arrows) extended to the first jejunal loop with thickened wall (empty arrow). Note the lymph node on the upper pole of the tumor (thin arrow). Axial thin slice on the pelvic (C): ascites in the Douglas pouch (asterisk).
Adenocarcinoma appears typically as a focal area of wall thickening with “overhanging edges” causing luminal narrowing. Less frequently, it appears as a polypoid mass or an infiltrative lesion and a classic
Fig. 2. Small bowel follow-through study showing aneurismal dilatation of the third portion of the duodenum with an irregular mucosa pattern.
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Fig. 3. Photomicrographs of tumor cells showing (A) a highly cellular tumor with glandular differentiation and wide areas of hemorrhage (asterisk) (HE, original magnification ×100). (B) Solid area with abundant figures of mitoses (HE, original magnification ×200). (C) High and intensive expression of cytokeratin relevant to the epithelial nature of the tumor cells (cytokeratine immunostaining; original magnification ×200). (D) Moderate expression of chromogranine by tumor cells demonstrating their endocrine nature (chromogranine immunostaining; original magnification ×200).
normal contracting ability. Tumor cells can also invade and block lymphatic and vascular channels with resulting anoxia of the muscle fibers and of the epithelial lining with subsequent necrosis [15]. All these factors have been described as mechanisms for aneurismal dilatation in lymphoma. 3. Conclusion Aneurismal dilatation of the small bowel is characterized by a focally dilated small loop with thickened wall or bowel mass. It has been described initially as typical involvement of lymphoma. Although uncommon, this radiologic feature can be encountered in metastatic lesions and in leiomyosarcoma. It is rather exceptional for primary adenocarcinoma of the small bowel but it should be included as a differential diagnosis. References [1] Horton KM, Fishman EK. Multidetector-row computed tomography and 3dimensional computed tomography imaging of small bowel neoplasms: current concept in diagnosis. J Comput Assist Tomogr 2004;28:106–16. [2] Buckley JA, Fishman EK. CT evaluation of small bowel neoplasms: spectrum of disease. Radiographics 1998;18:379–92. [3] Zissin R, Osadchy A, Gayer G, Shapiro-Feinberg M. Pictorial review. CT of duodenal pathology. Br J Radiol 2002;75:78–84.
[4] Cupps RE, Hodgson JR, Dockerty MB, Adson MA. Primary lymphoma in the small intestine: problems of roentgenologic diagnosis. Radiology 1969;92:1354–62. [5] Laurent F, Raynaud M, Biset JM. Boisserie- Lacroix M, Grelet P, Drouillard J. Diagnosis and computerization of small bowel neoplasms: role of computed tomography. Gastrointest Radiol 1991;16:115–9. [6] Paulsen SR, Huprich JE, Fletcher JG, et al. CT enterography as a diagnostic tool in evaluating small bowel disorders: review of clinical experience with over 700 cases. Radiographics 2006;26:641–57. [7] Gore RM, Mehta UK, Berlin JW, Rao V, Newmark GM. Diagnosis and staging of small bowel tumours. Cancer Imaging 2006;6:209–12. [8] Sandrasegaran K, Rajesh A, Rydberg J, Rushing DA, Akisik FM, Henley JD. Gastrointestinal stromal tumors: clinical, radiologic, and pathologic features. AJR Am J Roentgenol 2005;184:803–11. [9] Kim W, Baek JM, Suh YJ, Jeon HM, Kim JA. Ileal malignant melanoma presenting as a mass with aneurysmal dilatation: a case report. J Korean Med Sci 2004;19: 297–301. [10] Pantongrag-Brown L. Aneurysmal dilatation of the small bowel. Thai J Gastroenterol 2009;10:111–3. [11] Maizlin ZV, Brown JA, Buckley MR, Filipenko D, Barnard SA, Wong X, et al. Case of the season: aneurysmal dilatation of the small bowel (not only lymphoma). Semin Roentgenol 2006;41:248–9. [12] Mainenti PP, Segreto S, Mancini M, Rispo A, Cozzolino I, Masone S, et al. Intestinal amyloidosis: two cases with different patterns of clinical and imaging presentation. World J Gastroenterol 2010;16:2566–70. [13] Saindane AM, Losada M, Macari M. Focal amyloidoma of the small bowel mimicking adenocarcinoma on CT. AJR Am J Roentgenol 2005;185:1187–9. [14] Paul Guillerman R. Primary Intestinal non-Hodgkin lymphoma. J Pediatr Hematol Oncol 2000;22:476–8. [15] Kinoshita T, Yashiro N, Yasuda R, O'uchi T, Narita M. Leiomyosarcoma presenting aneurysmal dilatation of the jejunum on CT. J Comput Assist Tomogr 2000;24: 421–2.
