Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 4  |  Page : 505-507  

Ancient chest wall schwannoma: A case report with review of literature


1 Department of Surgical Oncology, Shri Sidhhivinayak Ganapati Cancer Hospital, Miraj, Maharashtra, India
2 Department of Pathology, Shri Sidhhivinayak Ganapati Cancer Hospital, Miraj, Maharashtra, India

Date of Web Publication14-Jul-2015

Correspondence Address:
Shriniketan S Kale
337, Amey, Radha Krishna Vasahat, Near Shahu Udyan, Sangli - 416 416, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.160811

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  Abstract 

Schwannoma is one of the most common benign tumors of the nerve sheath. In thoracic region, mediastinum is the prime site for the occurrence of schwannoma. Here, we present a case of chest wall schwannoma, which presented as incidentally detected chest wall tumors. Schwannoma being a slow growing benign tumor is incidentally detected on chest imaging done for vague complaints. Computerized tomography scan supports the diagnosis of schwannoma and is essential to assess the extent of the tumor. Treatment of choice is surgery. Now a day's thoracoscopic approach is preferred in suitable cases.

Keywords: Chest wall, pleural, S-100, schwannoma


How to cite this article:
Kale SS, Gosavi VS, Jagadale RV. Ancient chest wall schwannoma: A case report with review of literature. Med J DY Patil Univ 2015;8:505-7

How to cite this URL:
Kale SS, Gosavi VS, Jagadale RV. Ancient chest wall schwannoma: A case report with review of literature. Med J DY Patil Univ [serial online] 2015 [cited 2024 Mar 28];8:505-7. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2015/8/4/505/160811


  Introduction Top


Shwannoma is a benign peripheral nerve sheath tumor arising from the perineural cell called Schwann's cell, which forms the structural framework of a peripheral nerve. The tumor has predilection for head, neck, and flexor aspects of the extremities. Chest wall is very rare site for the schwannoma. Here, we present a case of chest wall schwannoma, which is rare due to its site of origin.


  Case Report Top


A 51-year-old lady presented with a history of pain in the right side of the chest and cough since 6 months. Pain was intermittent dull aching type. Cough was nonproductive and intermittent. On examination, air entry was reduced in the right mid and lower zone. Her X-ray chest showed right sided lung density in middle and lower part of the chest with obliteration of right cardiac border and right dome of the diaphragm with blunting of the costophrenic angle. Her contrast enhanced computerized tomography (CECT) scan showed right sided pleural based mass 14.1 cm × 9.1 cm × 8.8 cm occupying mid and lower zones of thorax. It was a well-circumscribed mass, which showed heterogeneous enhancement with internal nonenhancing necrotic areas as shown in [Figure 1]. There were no calcifications within the tumor. Right pleural cavity showed moderate to gross pleural effusion. Right lower lobe was collapsed and adjacent ribs and vertebral bodies didn't show any obvious destruction. Rest of the lung fields and mediastinal structures were normal. Liver showed two simple cysts.
Figure 1: Computerized tomography scan showing tumor in right thoracic cavity with cystic changes

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Computerized tomography guided biopsy was done which showed spindle shaped cells with oval to elongated nuclei and eosinophilic cytoplasm arranged in interlacing fascicles and whorls. Focal nuclear palisading was noted. However no necrosis, mitosis or nuclear atypia was noted. These features suggested a spindle cell tumor, possibly a solitary fibrous tumor. Pleural fluid cytology was negative for malignant cells and showed only reactive mesothelial cells. Her hematological and biochemical investigations were within normal limits. Her pulmonary function tests were adequate for thoracotomy and lung resection.

She was operated with right posteriolateral thoracotomy. Intraoperatively she was found to have a solid well encapsulated mass arising from posterior chest wall, which was free from lungs and important mediastinal structures like esophagus, azygos vein and pericardium. The mass was adherent to posterior chest wall with a stalk. There was half a liter of clear pleural fluid. The mass was excised with the pleura and adherent intercostal muscles. The chest was closed keeping an intercostal drain. Patient recovered well postoperatively. Grossly the tumor was well encapsulated [Figure 2]. On cut section, the tumor was partly solid and partly cystic. Solid areas were homogeneous, vaguely nodular yellowish gray with multiple cysts; largest measuring 6.5 cm × 5 cm. Microscopically the tumor showed spindle shaped cells with elongated wavy nuclei and eosinophilic cytoplasm arranged in fascicular pattern with intervening hypocellular areas as seen in [Figure 3]. There were focal lymphocytic clusters. There was focal nuclear atypia, but no mitosis or necrosis was noted. There were many areas of cystic degeneration. It was reported as a cystic schwannoma, but differential diagnoses of an inflammatory myofibroblastic tumor and a solitary fibrous tumor were considered. Immunohistochemistry (IHC) was performed. The tumor was positive for S-100 [Figure 4] and immunonegative for CD34, Mic-2 and bcl-2; hence the diagnosis of shwannoma was confirmed. In view of multiple necrotic areas and cysts in the tumor, it was labeled as a "ancient cystic schwannoma."
Figure 2: Gross specimen showing pedunculated mass which is well encapsulated

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Figure 3: Microscopic picture showing Antoni A and Antoni B areas

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Figure 4: Immunohistochemistry showing S-100 positivity with cystic changes

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  Discussion Top


Tumors of the chest wall are mostly metastatic in nature, and primary chest wall tumors are very rare. Only 1-2% of the chest wall tumors are primary in origin and a rare subset of them are schwannomas. [1]

Schwannoma, formerly called as neurilemmoma is a benign tumor arising from the schwann cell sheath of any nerve. It is mostly asymptomatic and is commonly detected as an incidental mass on chest radiograph. The diagnosis is often delayed due to vague symptoms. Rarely schwannoma is associated with pleural effusion, which is mostly reactionary to the presence of a pleural tumor or hemorrhagic due to rupture of the tumor. Our patient had reactionary pleural effusion. Two such cases of benign schwannoma with pleural effusion were reported in a Korian article. [2],[3]

Commonly schwannoma is incidentally detected on chest radiograph. It is seen as an extrapulmonary lesion characterized by "incomplete border sign" that is, the border of the lesion is incomplete where it is adherent to the pleura. The second sign is "tapering margin sign" that is, margins of the well-defined intrathoracic density are tapering due to lifted pleura by the mass. [1]

For the work up of a chest wall tumor, CECT scan is a preferred investigation. It generally shows a homogeneous mass, which is less attenuated than the muscles. However, large masses are having heterogeneous areas, which are due to degeneration, xanthomatous changes or hypocellularity. [4] In a large series of 36 patients of thoracic schwannomas, Ko. found cystic and multiple hypodence areas in 17 (45.9%) patients which were also seen in our patient due to cystic degeneration. [5] Magnetic resonance imaging is indicted when specific information like intraspinal extension is to be asserted.

Grossly, schwannoma is encapsulated, firm, and greyish tumor. It has a whorled pattern on sectioning. In large tumor degeneration, cyst formation or hemorrhage is frequently seen. Pathologically, schwannoma is nerve sheath in origin. Because this tumor develops in nerve sheath, it is surrounded by true capsule. Microscopically tumor shows Antoni A areas and Antoni B areas, which are alternating. Hypercellular areas, which show spindle cells with nuclear palisading forming verocay bodies are called Antoni A areas. The hypocellular areas with loose myxoid stroma are labeled Antoni B bodies. Schwannoma shows variable microscopic picture, so they are likely to be misdiagnosed. There are many types of shwannamas, which can be differentiated microscopically such as conventional, cellular, plexiform, and melanotic. [6],[7] IHC is the diagnostic test for the schwannoma, which shows strong positivity for S-100. Closest differential diagnosis would be the solitary fibrous tumor. It shows the spindle cells in a random pattern called "pattern less pattern." IHC wise it is positive for CD34, CD99, bcl-2, and vimentin. [1] In our case, it was negative, again favoring the diagnosis of schwannoma. Ancient shwannoma is well described entity whenever there is nuclear atypia or degenerative changes in the tumor. Our case qualifies for the term because of the focal nuclear atypia and the cystic changes in the tumor, which suggest degenerative changes.

Treatment for the schwannoma is surgical. Excision is done with video assisted thoracoscopic surgery or through open surgical approach. [8] We have done excision by open technique, tumor being of large size. Part of pleura and chest wall soft tissue adjacent to the base of the tumor was excised for better margins.


  Conclusion Top


Schwannoma is a common peripheral nerve sheath tumor, which is rare in the chest wall. In this rare situation, the diagnosis can be confused with the solitary fibrous tumor due to its presentation as intrathoracic pedunculated mass. IHC plays a pivotal role in diagnosing schwannoma. Hence, schwannoma can be kept as a possible differential diagnosis of a chest wall tumor particularly if it is vaguely symptomatic and if it is slow growing.


  Acknowledgments Top


Department of anesthesiology, Shri Siddhi Vinayak Ganapati Cancer Hospital, Miraj.



 
  References Top

1.
Sawas FA, Lababede O, Meziane MA, Arrossi AV. A 54-year-old woman with incidentally discovered mass on a chest radiograph. Chest 2009;135:1673-8.  Back to cited text no. 1
    
2.
Cho JH, Kim KU, Park HK, Jeong YJ, Kim YD, Kim YS, et al. Two cases of benign solitary schwannoma with pleural effusion. Tuberc Respir Dis 2007;63:78-82.  Back to cited text no. 2
    
3.
Morimoto J, Nakajima T, Iizasa T, Ishikawa A, Nishimura H, Shingyouji M. Successful resection of schwannoma from an intercostal nerve causing bloody pleural effusion: Report of a case. Surg Today 2011;41:989-91.  Back to cited text no. 3
    
4.
Hu S, Chen Y, Wang Y, Chen KM, Song Q. Clinical and CT manifestation of pleural schwannoma. Acta Radiol 2012;53:1137-41.  Back to cited text no. 4
    
5.
Ko SF, Lee TY, Lin JW, Ng SH, Chen WJ, Hsieh MJ. Thoracic neurilemomas: An analysis of computed tomography findings in 36 patients. J Thorac Imaging 1998;13:21-6.  Back to cited text no. 5
    
6.
Ondo K, Sugio K, Yano T, Kase S, Yamazaki K, Yamaguchi M. Cystic schwannoma presenting as massive hemoptysis in an adult. Respiration 2000;67:327-9.  Back to cited text no. 6
    
7.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM. The pathobiologic spectrum of Schwannomas. Histol Histopathol 2003;18:925-34.  Back to cited text no. 7
    
8.
Ponce FA, Killory BD, Wait SD, Theodore N, Dickman CA. Endoscopic resection of intrathoracic tumors: Experience with and long-term results for 26 patients. J Neurosurg Spine 2011;14:377-81.  Back to cited text no. 8
    


    Figures

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


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