|Year : 2018 | Volume
| Issue : 12 | Page : 1220-1222
Multiple intercostal neurilemmomas in a Chinese woman
Wen-Kui Sun1, Wen Yang2, Chen-Hui Ma2, Xin-Wu Xiao2, Yi Shi2, Yong Song2
1 Department of Respiratory and Critical Care Medicine, Jinling Hospital, Nanjing University Medical School; Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, People's Republic of China
2 Department of Respiratory and Critical Care Medicine, Jinling Hospital, Nanjing University Medical School, Nanjing, People's Republic of China
|Date of Web Publication||11-Dec-2018|
Department of Respiratory Medicine, Jinling Hospital, Nanjing University School of Medicine, #305, East Zhongshan Road, Nanjing 210002
People's Republic of China
Source of Support: None, Conflict of Interest: None
Neurilemmomas are rare tumors of neural crest cell origin that occur most commonly in the head and neck region. Intercostal neurilemmomas are extremely rare and are mostly seen as solitary tumors in the posterior mediastinum. Only one case report of multiple intercostal neurilemmomas has been documented previously. In this article, we report a case of multiple intercostal neurilemmomas in a 54-year-old woman who had initially presented with progressive dull left chest pain over a 1-year period. A computed tomography scan revealed three tumors in the left thoracic cavity which were distributed as a string of beads along the third intercostal nerve. Histological and immunohistochemical testing confirmed a diagnosis of neurilemmomas. The patient underwent successful radical excision of the tumors through a thoracotomy approach, and her postoperative course was uneventful. Following the operation, she had no evidence of recurrences.
Keywords: Diagnosis, intercostal nerve, neurilemmoma, schwannoma, treatment
|How to cite this article:|
Sun WK, Yang W, Ma CH, Xiao XW, Shi Y, Song Y. Multiple intercostal neurilemmomas in a Chinese woman. J Can Res Ther 2018;14, Suppl S5:1220-2
| > Introduction|| |
Neurilemmomas (schwannomas and neurinomas) are neurogenic tumors arising from the Schwann cells of the neural sheath. They are usually benign and frequently arise from a spinal nerve root and occur most commonly in the head and neck region. Intercostal neurilemmomas, which are extremely rare, originate in the intercostal and subcostal nerves. They are mostly slow-glowing and benign tumors. There are no obvious early clinical symptoms, and they can manifest as intercostal neuralgia when the tumor causes intercostal nerve compression. In this report, we present a rare case of neurilemmomas arising from the intercostal nerve and describe their characteristic radiological features.
| > Case Report|| |
A 54-year-old woman was referred to our hospital because of progressive dull left chest pain over a 1-year period, which was aggravated by heavy breathing. She denied having a cough, fever, chills, night sweats, hemoptysis, or weight loss. In 2003, the patient had undergone uterine myomectomy.
A chest radiograph and computed tomography (CT) scan of the chest showed multiple nodular lesions [Figure 1]. Topographically, they were localized to the pleural cavity. A microscopic examination revealed that the tumors had arisen from the thoracic spine and were distributed as a string of beads along the third intercostal nerve. A CT-guided percutaneous lung biopsy was performed. The histological diagnosis was “neurilemmomas.”
|Figure 1: A computed tomography scan revealed three tumors in the left thoracic cavity. No evidence of mediastinal lesions or pleural effusions was identified on this computed tomography scan|
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The patient underwent radical tumor excision through a left posterolateral thoracotomy. There was no pleural effusion in the left thoracic cavity, and three tumors were observed during the operation. The tumors measured 5 cm × 5 cm, 3 cm × 4 cm, and 1.5 cm × 1.5 cm, respectively, were distributed along the third intercostal nerve and were well encapsulated [Figure 2]. Microscopically, they were partly hypercellular tumors composed of fascicles of spindle cells with Antoni A areas [Figure 3]a. Immunohistochemically, the tumors were diffuse and intensely immunoreactive to S100 protein [Figure 3]b, and were classified as Vim+++, CKpan−, MC−, CR−, CD34−, CD68−, SMA−, and Ki67 <1%+. Thus, the pathological findings confirmed the diagnosis of neurilemmoma.
|Figure 2: The tumors measured 5 cm × 5 cm, 3 cm × 4 cm, and 1.5 cm × 1.5 cm, respectively. They were distributed as a string of beads along the third intercostal nerve and were well encapsulated|
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|Figure 3: (a) Histologic section from a biopsy specimen in higher-power view revealed long spindle cells in a fasciculated and disarrayed architecture, and nuclei in a palisading pattern (H and E, ×400). (b) Immunohistochemical stain with S100 protein antibody revealed that the tumor cells were diffuse and positive, including nuclei and cytoplasm (×400)|
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The patient's postoperative course was uneventful, and she was able to return to her home on the sixth postoperative day.
| > Discussion|| |
Intercostal neurilemmomas are extremely rare and mostly arise from intercostal nerves as a solitary tumor close to the spinal canal., We conducted PubMed literature search and found few reports of solitary intercostal neurilemmomas, and only one case report of lesions arising from distal intercostal nerves and manifesting as multiple lesions. Interestingly, in the patient described in this report, we found multiple tumors distributed as a string of beads along the third intercostal nerve during the thoracoscopic surgical exploration.
Intercostal neurilemmomas should be diagnosed differently from other tumors that occur in the thoracic cavity such as malignant pleural mesotheliomas, neurofibromas, and pulmonary malignancies. Fluorodeoxyglucose positron emission tomography/CT, chest CT, and chest magnetic resonance imaging are all reported to be useful for the confirmation of thoracic tumors. However, diagnosing an intercostal neurilemmoma by only imaging information is difficult. In our patient, chest CT was not useful for differentiating the neurilemmoma from a malignant pleural mesothelioma as there was no enough difference in density levels and morphology between them. We had speculated that the tumors were malignant pleural mesotheliomas until the pathological diagnosis of intercostal neurilemmomas was made. Consequently, the results from the percutaneous needle biopsy were necessary for confirming the diagnosis.
The histological features of neurilemmomas include an Antoni A tissue pattern with spindle-shaped tumor cells arranged in a palisading pattern or an Antoni B tissue pattern with loose cellularity in a reticular array. In general, most neurilemmomas exhibit the two patterns at the same time. Immunohistochemical stainings for S100 protein, vimentin, and CD34 are also useful.
As with most tumors, a precise diagnosis and treatment plan for intercostal neurilemmomas depend on pathological findings. The tumors in our patient showed typical neurilemmoma components, including both hypercellular Antoni A and hypocellular Antoni B regions. In addition, the tumors were positive for S100 protein, a neurilemmoma marker, and they had not infiltrated adjacent structures. In view of these observations, the final diagnosis was benign thoracic neurilemmomas, and complete surgical resection was successfully performed through a thoracotomy approach. Following the operation, the patient had no evidence of recurrences.
| > Conclusion|| |
Neurilemmomas are histologically benign and the prognosis of patients with this condition is good. Hence, every effort should be made to diagnose them by pre- or intra-operative frozen biopsy because the treatment of choice for intercostal neurilemmomas is gross complete resection.
We would like to acknowledge the Content Ed Net, Shanghai Co. Ltd., for editorial assistance.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]