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AJR 2001; 177:652
© American Roentgen Ray Society


Radiologic-Pathologic Conferences of the
University of Texas M. D. Anderson Cancer Center

Benign Schwannoma in the Porta Hepatis

Haesun Choi1, Gary Whitman1, Jae Ro2 and Chusilp Charnsangavej3

1 Division of Diagnostic Radiology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Box 057, Houston, TX 77030.
2 Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030.
3 Division of Diagnostic Imaging, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030.

Received January 16, 2001; accepted after revision March 1, 2001.

 
From the weekly radiologic—pathologic correlation conferences conducted by Gary J. Whitman.

Address correspondence to H. Choi.


Introduction
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Introduction
References
 
A 37-year-old asymptomatic man was referred for evaluation of a mass in the porta hepatis found incidentally during a workup performed after the patient's hepatitis serology test had shown positive results. Sonograms showed a well-defined, 5-cm complex mass at the porta hepatis with a large hyperechoic solid component (Fig. 1A). MR imaging revealed a heterogeneous mass consisting of cystic and gradually enhancing solid areas. CT scans showed the solid component enhancing gradually during a 10-min period (Figs. 1B and 1C). At surgery, the mass was found to be encasing the right hepatic artery and appeared to be arising from it. The mass was otherwise well separated from adjacent solid organs, bile ducts, and other major vessels. Gross cystic degenerative changes with areas of calcification and prominent vessels were present in the solid mass. Microscopically, the mass consisted of bundles of spindle cells with hypercellular and hypocellular areas (Fig. 1D). Final pathologic diagnosis was a benign schwannoma.



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Fig. 1A. Benign schwannoma in porta hepatis in 37-year-old man. Sonogram reveals complex mass (arrow) at porta hepatis.

 


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Fig. 1B. Benign schwannoma in porta hepatis in 37-year-old man. Arterial phase of triphase dynamic CT scan shows mass of low attenuation (arrow).

 


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Fig. 1C. Benign schwannoma in porta hepatis in 37-year-old man. Delayed venous phase of CT scan shows gradually enhancing solid component (arrows) at posterior aspect of mass.

 


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Fig. 1D. Benign schwannoma in porta hepatis in 37-year-old man. Photomicrograph reveals bundles of spindle cells (arrow) with hypercellular (Antoni type A) and hypocellular (Antoni type B) areas. (H and E, x200)

 

Benign schwannomas, also referred to as neurilemomas, neurinomas, and perineural fibroblastomas, are encapsulated nerve sheath tumors. They are usually slow-growing and may occur at any age, although they are most commonly seen in patients who are between the ages of 30 and 60 [1]. Slightly more common in women than in men, schwannomas occur most frequently in the head, neck, and flexor surfaces of the upper and lower extremities. Deep-seated tumors may occur in the mediastinum, rarely in the retroperitoneum, and very rarely in the mesentery [1, 2]. To our knowledge, schwannoma in the porta hepatis has not been previously reported. Symptoms and signs vary depending on the anatomic site and size of the neoplasm; however, most schwannomas present as an asymptomatic or painless mass [1].

Histologically, schwannomas are encapsulated tumors that arise within the nerve sheaths and consist of a highly ordered cellular component (Antoni type A area) and a loose myxoid component (Antoni type B area). Occasionally they may degenerate and become cystic because of either hemorrhage or necrosis. Dystrophic calcification and xanthomatous degeneration can occur. Schwannomas can also be quite hypervascular. The presence of encapsulation, the two types of Antoni areas, and uniformly intense immunostaining for S-100 protein distinguish schwannomas from neurofibromas [1].

The radiologic appearance of schwannomas varies depending on the distribution of the different histologies (Antoni A and Antoni B areas) and the degrees or types of degeneration. In general, schwannomas are well defined with smooth margins and are relatively heterogeneous on CT and MR images [3, 4]. They are usually hypointense on T1-weighted images and reveal increased signal intensity on T2-weighted images. They may be cystic, hemorrhagic, or calcified. A target appearance with a hyperintense rim attributable to peripheral myxomatous tissue has been described as a useful diagnostic finding on MR images of benign nerve sheath tumors [4]. Central enhancement related to hypercellular Antoni A areas has also been described on CT and MR images [2]. Prolonged delayed enhancement has been reported [5].

Treatment of benign schwannomas is simple excision. Recurrent disease is extremely rare even after partial resection. Incomplete excision is warranted in patients in whom permanent nerve damage is likely to occur [1].


References
Top
Introduction
References
 

  1. Enzinger FM, Weiss SW. Benign tumors of peripheral nerves. In: Soft tissue tumors, 3rd ed. St. Louis: Mosby 1995: 829-842
  2. Ogose A, Hotta T, Morita T, et al. Tumors of peripheral nerves: correlation of symptoms, clinical signs, imaging features, and histologic diagnosis. Skeletal Radiol 1999;28:183 -188[Medline]
  3. Cohen LM, Schwartz AN, Rockoff SD. Benign schwannomas: pathologic basis for CT inhomogeneities. AJR 1986;147:141 -143[Abstract/Free Full Text]
  4. Varma DGK, Moulopoulos A, Sara AS, et al. MR imaging of extracranial nerve sheath tumors. J Comput Assist Tomogr 1992;16:448 -453[Medline]
  5. Hayasaka K, Tanaka Y, Hupper S, Clauseen CD. MR findings in primary retroperitoneal schwannoma. Acta Radiol 1999;40:78 -82[Medline]

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