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ABDOMINAL IMAGING Table of Contents   
Year : 2009  |  Volume : 19  |  Issue : 3  |  Page : 213-215
Case report: Benign porta hepatic schwannoma


Department of Radiology, Kasturba Medical College, Manipal-576 104, Karnataka, India

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Date of Web Publication4-Aug-2009
 

   Abstract 

Schwannoma is a myelin sheath tumor that can occur almost anywhere in the body. The most common locations are the central nervous system, extremities, neck, mediastinum and retroperitoneum. Benign schwannomas in the porta hepatis are extremely rare and radiologically are diagnosed as either enlarged lymph nodes or bowel masses, such as gastrointestinal stromal tumors. In this location they usually produce symptoms by compressing adjacent structures and often present with obstructive jaundice. The preoperative diagnosis can be extremely difficult.

Keywords: Obstructive jaundice; porta hepatis; Schwannoma

How to cite this article:
Kulkarni N, Andrews SJ, Rao V, Rajagopal K V. Case report: Benign porta hepatic schwannoma. Indian J Radiol Imaging 2009;19:213-5

How to cite this URL:
Kulkarni N, Andrews SJ, Rao V, Rajagopal K V. Case report: Benign porta hepatic schwannoma. Indian J Radiol Imaging [serial online] 2009 [cited 2020 Jul 3];19:213-5. Available from: http://www.ijri.org/text.asp?2009/19/3/213/54888

   Introduction Top


Benign schwannomas, also referred to as neurilemomas and neurinomas, are encapsulated myelin sheath tumors. They are usually benign and their presentation depends on the anatomical location and tumor extent. [1] A benign schwannoma in the porta hepatis may be completely asymptomatic, being detected incidentally, [1],[2] or may present with features of obstructive jaundice. In the literature, only two cases of benign schwannoma in the porta hepatis have been reported till date.


   Case Report Top


A 38-year-old man was admitted to our institute with upper abdominal pain, poor appetite, loss of weight and yellowish discoloration of the eyes for 2 months. His clinical and family histories were noncontributory. There was no history of drug intake. Per abdominal examination revealed a soft abdomen with no evidence of hepatomegaly or splenomegaly. There was no lymphadenopathy. Laboratory investigations revealed elevated values of total bilirubin (7.4 mg/dl), direct bilirubin (4.3 mg/dl) and alkaline phosphatase (567 U/l). Total protein, albumin, ALT (alanine aminotransferase) and AST (aspartate aminotransferase) were normal. These findings were consistent with obstructive jaundice. CT scan of the abdomen revealed a heterogeneously enhancing lesion measuring 4.7 × 3.4 × 3.2 cm (craniocaudal × anteroposterior × transverse) in the region of the porta hepatis [Figure 1],[Figure 2], extending caudally till the second part of the duodenum. The lesion was seen to be compressing the supraduodenal portion of the common bile duct, causing dilatation of the proximal common bile duct and the intrahepatic biliary system. The patient underwent exploratory laparotomy, which revealed a 5 × 4 cm firm lesion [Figure 3]A that was inseparable from the common bile duct. Cholecystectomy and Roux-en-Y hepaticojejunostomy were performed and the lesion, along with the common bile duct, was excised. The resected specimen [Figure 3]B showed the lesion inseparably attached to the common bile duct and the gallbladder. The histopathologic findings were consistent with schwannoma composed of Antoni A and B areas [Figure 4].


   Discussion Top


Benign schwannoma is a tumor arising from Schwann cells, which form the neural sheaths of the peripheral nerves. The most common locations are the cranial nerves (especially the eighth cranial nerve ) and the peripheral nerves in the region of the neck, mediastinum and extremities. [3] Theoretically, the tumor can affect almost any organ or nerve trunk in the body other than the optic and olfactory nerves, which do not possess Schwann cells. [3] Our review of literature showed that two cases of benign schwannoma in the porta hepatis and nine cases of extrahepatic biliary schwannoma have been reported in the literature. [4] Patients with schwannoma in the porta hepatis may be asymptomatic or may present with obstructive jaundice. [5],[6] Gastrointestinal schwannomas appear as sharply demarcated, round or oval mass with homogeneous attenuation on both unenhanced and contrast-enhanced CT scans. They frequently lack a tumor capsule, necrosis, cavitation and calcification on CT scan. [7]

In the present case, CT scan revealed a heterogeneous lesion at the porta hepatis, which was initially suspected to be a lymph nodal mass or an exophytic gastrointestinal stromal tumor (GIST). Gastrointestinal stromal tumors are a distinct group of mesenchymal tumors arising from the muscularis propria layer of the digestive tract; they often project exophytically and/or intraluminally. [8],[9] On CT scan, GISTs are usually well-demarcated spherical masses, with evidence of necrosis and cavitation within the lesion, both of which are uncommon in gastrointestinal schwannomas. [7],[10] The other likely differential diagnosis of a solitary lesion occurring at this site is a polypoid extrahepatic cholangiocarcinoma. [11] A clinical diagnosis of schwannoma or polypoid extrahepatic cholangiocarcinoma is difficult unless the mass is palpable. [12]

On histopathology, the characteristic features include the presence of alternating Antoni A and Antoni B areas. The Antoni A area is composed of spindle-shaped Schwann cells arranged in interlacing fascicles. The Antoni B area consists of a loose meshwork of gelatinous and microcystic tissue. Large, irregularly spaced, thick-walled blood vessels can be seen in Antoni B areas. These may contain intraluminal thrombi. [13]

This case illustrates the difficulty in making the diagnosis of a schwannoma in the porta hepatis based on CT imaging features alone. Schwannoma can be mistaken for GIST and polypoid extrahepatic cholangiocarcinoma, and it is not possible to pre-operatively make a diagnosis based on imaging findings alone. [14]

In this case, the postoperative course was uneventful and at a follow-up visit after 3 months, the patient was clinically asymptomatic and free of icterus.

 
   References Top

1.Reed JC, Hallet KK, Feigin DS. Neural tumors of the thorax: subject review from the AFIP. Radiology 1978;126:9-17.  Back to cited text no. 1  [PUBMED]  
2.Kim SH, Choi BI, Han MC, Kim YI. Retroperitoneal neurilemoma: CT and MR findings. AJR Am J Roentgenol 1992;159:1023-6.   Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Gooder P, Farrington T. Extracranial neurilemmoma of the head and neck. J Laryngol Otol 1980;94:243-9.  Back to cited text no. 3  [PUBMED]  
4.Fenoglio L, Severini S, Cena P. Common bile duct schwannoma: A case report and review of literature. World J Gastroenterol 2007;13:1275-8.  Back to cited text no. 4    
5.Jung JH, Joo KR, Chae MJ. Extrahepatic biliary schwannomas: a case report. J Korean Med Sci 2007;22:549-52.  Back to cited text no. 5    
6.Park MK, Lee KT, Choi YS. A case of benign schwannoma in the porta hepatis. Korean J Gastroenterol 2006;47:164-7.  Back to cited text no. 6    
7.Levy AD, Quiles AM, Miettinen M. Gastrointestinal schwannomas: CT features with clinicopathologic correlation. AJR Am J Roentgenol 2005;184:797-802.  Back to cited text no. 7    
8.Cichoz-Lach H, Kasztelan-Szczerbinska B, Slomka M. Gastrointestinal stromal tumors: epidemiology, clinical picture, diagnosis, prognosis and treatment. Pol Arch Med Wewn 2008;118:216-21.  Back to cited text no. 8    
9.Hughes JA, Cook JV, Said A, Chong SK, Towu E, Reidy J. Gastrointestinal stromal tumor of the duodenum in a 7-year-old boy. Pediatr Radiol 2004;34:1024-7.   Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Hong HS, Ha HK, Won HJ. Gastric schwannomas: radiological features with endoscopic and pathological correlation. Clin Radiol 2008;63:536-42.  Back to cited text no. 10    
11.Han JK, Choi BI, Kim AY, An SK, Lee JW, Kim TK, et al . Cholangiocarcinoma: Pictorial essay of CT and cholangiographic findings. Radiographics 2002;22:173-87.  Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.de Diego Rodriguez E, Roca Edreira A, Martin Garcia B. Retroperitoneal benign schwannoma: Report of a new case. Actas Urol Esp 2000;24:685-8.  Back to cited text no. 12    
13.Sharma S, Sarkar C, Mathur M. Benign nerve sheath tumors: A light microscopic, electron microscopic and immunohistochemical study of 102 cases. Pathology 1990;22:191-5.  Back to cited text no. 13    
14.Kwon MS, Lee SS, Ahn GH. S chwannomas of the gastrointestinal tract: Clinicopathological features of 12 cases including a case of esophageal tumor compared with those of gastrointestinal stromal tumors and leiomyomas of the gastrointestinal tract. Pathol Res Pract 2002;198:605-13.  Back to cited text no. 14  [PUBMED]  

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Correspondence Address:
Naveen Kulkarni
Department of Radiodiagnosis and Imaging, Kasturba Medical College, Manipal - 576 104, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.54888

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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