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Neurofibrom-Schwannom Hybrid Tumor in the Chest Wall

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Neurofibrom-Schwannom Hybrid Tumor in the Chest Wall Muharrem C, et al. J Pulm Med Respir Res 2020, 6: 047 DOI: 10.24966/PMRR-0177/100047 HSOA Journal of Pulmonary Medicine and Respiratory Research Case Report Contrast-enhanced computed tomography of the chest was re- quested to see the extent of the lesion on the chest wall. On the thorax Neurofibrom-Schwannom CT was seen lesion in size 51x28x25cm in the axial axis at anterolat- eral 5th intercostal space in extrapulmonary localization. Density of Hybrid Tumor in the Chest Wall protruding noduler lesion was average 10 HU (Figure 1). Cakmak Muharrem1* and Daglı Adile Ferda2 1Department of Thoracic Surgery, Fırat University Faculty of Medicine, Elazig, Turkey 2Department of Pathology, Fırat University Faculty of Medicine, Elazig, Turkey Abstract Schwannoma originates from the roots of spinal nerve. Chest wall shwannoma originating from intercostal nerves is very rare. In this study, we aimed to share a schawannoma-neurofibroma hybrid tu- mor located in the chest wall. Keywords: Chest wall; Mass; Neurofibroma; Schwannoma Introduction Schwannoma is a benign, encapsulated, neurogenic tumor orig- inating from schwann cells. They often originate from the roots of Figure 1: Tomography image of the patient (axial). the spinal nerve. Neurofibroma is a tumor originating from the nerve The patient’s medical history was unremarkable, and laboratory tests were sheath anywhere in the body [1]. Although chest wall shwannoma and normal. The operation was planned. The mass was completely excised (Figure neurofibromas originating from intercostal nerves have been reported 2). The postoperative chest wall defect was reconstructed using surrounding rarely, schwannoma-neurofibroma hybrid tumor has not been report- intact tissues. The pathology result was reported as schawannoma-neurofibro- ed. Nerve system rooted tumors are seen equally in women and men ma hybrid tumor (Figures 3(a-d)). No recurrence was observed in the patient [2]. They are usually asymptomatic and benign. Malignancy is rarely who was followed for one year. seen. Symptomatic ones often present as compression-related pain or slow-growing, painless, prominent masses. Neurofibromas are mostly associated with neurofibromatosis. Radiological data are helpful in the diagnosis. Treatment is surgical excision, but long-term follow-up is essential for recurrence [3]. In this study, we aimed to share a scha- wannoma-neurofibroma hybrid tumor located in the chest wall. Case Report A 47-year-old female patient was admitted to our clinic with the complaint of a painful swelling in her chest wall that had gradually increased in the last year. On examination, a painful lesion measuring approximately 50x25mm was detected on the lateral of the left hemi- thorax. *Corresponding author: Cakmak Muharrem, Department of Thoracic Surgery, Fırat University Faculty of Medicine, Elazig, Turkey, Tel: +90 4242333555 (2130); E-mail: [email protected] Citation: Muharrem C, Ferda DA (2020) Neurofibrom-Schwannom Hybrid Tumor Figure 2: Intraoperative surgical incision and appearance of the mass. in the Chest Wall. J Pulm Med Respir Res 6: 047. Discussion Received: October 22, 2020; Accepted: November 09, 2020; Published: No- vember 16, 2020 Schwannoma is a benign, encapsulated, neurogenic tumor origi- nating from schwann cells [1]. It is a peripheral nerve sheath tumor Copyright: © 2020 Muharrem C, et al. This is an open-access article distributed and often originates from the spinal nerve roots. Neurofibroma is a under the terms of the Creative Commons Attribution License, which permits un- restricted use, distribution and reproduction in any medium, provided the original tumor originating from the nerve sheath anywhere in the body. Al- author and source are credited. though chest wall shwannoma and neurofibromas originating from Citation: Muharrem C, Ferda DA (2020) Neurofibrom-Schwannom Hybrid Tumor in the Chest Wall. J Pulm Med Respir Res 6: 047. • Page 2 of 3 • Figure 3a: Presence of right thick capsule seen in Schwannoma (HEX100). Figure 3d: CD34 positivity in peripheral nerve sheath tumor (X400). Chest wall shwannoma originating from the intercostal nerves is very rare [2]. It is seen equally among women and men. Neurofibromas are mostly associated with neurofibromatosis. Our patient was a tumor originating from the intercostal nerve. They are usually asymptomatic, benign and very rarely malignant [3]. It may occur as a solid mass on the chest wall [4]. Symptomat- ic ones often present as compression-related pain or slow-growing, painless, prominent masses [1]. Schwannomas are divided into four groups as conventional, cellular, plexiform and melanotic schwanno- mas. Classical schwannoma is associated with neurofibromatosis type 2. It is also called neurilemmoma and neurinoma. It can be seen at any age, but peaks in the third and sixth decades. It occurs equally in both sexes. central nervous system schwannomas are more common in women [5]. Schwannomas originating from larger nerves are seen especially in cranial sensory nerves and spinal nerve roots. Patients Figure 3b: Neurofibroma areas consisting of spindle-shaped and wavy nu- cleus cells and collagen (HEX400). are usually asymptomatic. Cutaneous scwannomas are usually small in diameter. Mediastinal, retroperitoneal and sacral localizations may be large. Therefore, symptoms may occur due to compression of the mass to the surrounding tissue. Schwannomas are usually solitary le- sions and grow slowly over the years. Palpable lesions are detected mobile on physical examination. Multiple schwannomas are encoun- tered in two cases. The most common of these is NF2-related bilateral 8th nerve schwannomas. Rarely encountered are multiple schwanno- mas associated with somatic mutations in the NF2 gene and are called “schwannomatosis [3-5]. These patients do not have a family history and do not have bilat- eral 8th nerve schwannomas. Instead, most cutaneous or subcutane- ous, larger spinal or cranial nerves are involved. Diagnosis is made by the presence of both Antoni A/Antoni B and verocay bodies with histochemical stains. As they are benign and well-limited, total exci- sion is sufficient for treatment. However, when they are incompletely removed, they rarely recur. Malignant transformation is the only type [3-5]. Figure 3c: S-100 positivity in peripheral nerve sheath tumor (X400). Radiological data are helpful in the diagnosis. Approximately 5% intercostal nerves have been reported rarely, schwannoma-neurofibro- of the spinal origin is seen in the form of an hourglass neural fora- ma hybrid tumor has not been reported [2]. Thoracic neurogenic tu- men extension. Computed tomography or oblique chest X-rays may mors occur in the posterior mediastinum because intrathoracic nerves show notches in the ribs and enlargement of the vertebrae foramen. are mostly concentrated in the paravertebral sulcus region (75-95%). In non-contrast CT, paraspinal, well-circumscribed homogeneous Volume 6 • Issue 3 • 100047 J Pulm Med Respir Res ISSN: 2573-0177, Open Access Journal DOI: 10.24966/PMRR-0177/100047 Citation: Muharrem C, Ferda DA (2020) Neurofibrom-Schwannom Hybrid Tumor in the Chest Wall. J Pulm Med Respir Res 6: 047. • Page 3 of 3 • hypodense in the posterior mediastinum, areas with fluid density in Conflicts of İnterest places, and post-contrast muscle and iso-hyperdense lesions appear. Magnetic resonance imaging shows iso-hypointense on T1-weighted There are no conflicts of interest. images and homogeneous and heterogeneous apparent hyperintense References on T2-weighted images [6]. Treatment is complete surgical excision, but long-term follow-up is required for recurrence. Standard thora- 1. Albert P, Patel J, Badawy K, Weissinger W, Brenner M, et al. (2017) Pe- ripheral nerve schwannoma: A review of varying clinical presentations and cotomy often requires a large incision and causes postoperative pain. ımaging findings. The Journal of Foot and Ankle Surgery 56: 632-637. Thus, VATS may be preferred to minimize trauma in posterior thorac- ic cavity located masses [4]. 2. Galukande M, Khingi A (2016) Chest wall schwannoma presenting as a solitary malignant lesion: A case report. Springer Plus 1: 1549. In conclusion, schawannoma-neurofibroma hybrid tumor are not common. Additionally, chest wall placement is very rare. These tu- 3. Moon H, Park SJ, Kim SR, Park HS, Lee YC (2010) Benign intercostal mors should be considered in the differential diagnosis of chest wall schwannoma mimicking a solitary metastasis from lung cancer. Thorax masses in elderly patients. 65: 753-754. Declaration of Patient Consent 4. Chen F, Nakayama E, Okubo K, Date H (2008) Intrathoracic multiple schwannomas of a single intercostal nerve. Ann Thorac Surg 86: 660-661. The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their 5. Ida CM, Scheithauer BW, Yapicier Ö, Carney JA, Wenger DE, et al. (2011) consent for his/her/their images and other clinical information to be Primary schwannoma of the bone: A clinicopathologic and radiologic reported in the journal. The patients understand that their names and study of 17 cases. Am J Surg Pathol 35: 989-997. initials will not be published and due efforts will be made to conceal 6. Kivrak AS, Koc O, Emlik D, Kiresi D, Odev K, et al. (2009) Differential their identity, but anonymity cannot be guaranteed. diagnosis of dumbbell lesions associated with spinal neural foraminal wi- Financial Support and Sponsorship dening: Imaging features. Eur J Radiol 71: 29-41. The authors
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