Giant supraglottic schwannoma

Schwannomas arising from the supraglottis are uncommon, if not rare. They are known to arise from the head and neck region but oropharyngeal presentation is not often seen. Few cases of pedunculated schwannoma arising from the aryepiglottic fold presenting as giant tumours in the oropharynx have been reported so far. The tumour in this case has been excised via intraoral approach, with nil residual morbidity. A follow up period of one year was uneventful without any recurrence.     

浅表神经鞘瘤的超声诊断价值

目的 探讨浅表神经鞘瘤的超声诊断特点。方法 回顾性分析2014年1月至2015年6月超声检查初步诊断为神经鞘瘤36例患者的超声影像学特征。结果 超声肿块的检出率为1000%。36例患者中手术及病理证实为神经鞘瘤24例,超声诊断与病理诊断符合率为667%（24/36）。瘤体多为圆形或椭圆形,边界清;1例为多发病灶,其余均为单发;内部回声为低回声18例,囊实混合性回声6例;18例探测到鼠尾征;Adler分级法：0级4例,Ⅰ级7例,Ⅱ级10例,Ⅲ级3例。结论 超声诊断浅表神经鞘瘤具有较高的诊断价值,且操作简便,无创伤,值得临床推广应用。     

腹膜后神经鞘瘤82例临床分析

目的通过临床病例分析,了解腹膜后神经鞘瘤的临床诊治状况,提高鉴别诊断能力.方法对本组病例行临床分析.结果本组82例均行手术治疗,肿瘤全切除60例(占73.2%),次全和大部分切除13例(占15.9%),无法切除9例(占11.0%);术中探查单发肿瘤78例(占95.1%),多发4例(占4.9%).肿瘤多位于脊柱旁,呈囊实性,有包膜,表面光滑,血管丰富,界清,包膜表面可与周围组织粘连.病理结果诊为神经鞘瘤81例(占98.8%),恶性神经鞘瘤1例(占1.2%);在多发肿瘤4例中,2例(占2.4%)诊为双原发,伴随病灶为升结肠腺癌及左上肺鳞癌,术后复发1例.结论腹膜后神经鞘瘤发病率低,术前诊断困难,绝大部分为良性,恶性少见,主要治疗方法为手术治疗,恶性神经鞘瘤对放化疗不敏感,预后差.     

脑回状神经鞘瘤的临床病理学研究

目的探讨脑回状神经鞘瘤(GSW)的临床病理学特征。方法对4例GSW进行临床特征、光镜表现、免疫组化情况及超微结构进行观察。结果 4例患者中男性、女性各2例,均位于头颈部(颈部及腮腺各2例),为缓慢生长的无痛性或轻微触痛,境界清楚的包块。大体标本肿瘤包膜完整,直径2~4 cm,切面呈灰黄色。镜下肿瘤组织呈实性片状分布,由圆形或卵圆形细胞构成,胞浆丰富、红染,胞核排列紧密,肿瘤细胞形成不规则梁索状或分枝状并相互吻合,极性明显且富于胞浆的突起分布于梁索状或分枝状胞核两侧,形成脑回状外观。免疫组化显示GSW表达Vimentin、S-100蛋白、CD56、Bcl-2、PGP9.5,弱阳性表达CD99,不表达calponin、β-catenin、desmin、CD34、MSA、SMA、GFAP、collagen IV、Cyclin D1、laminin、AE1/AE3及p63。电镜下显示GSW细胞具有神经鞘细胞特征。结论 GSW具有独特的形态学表现,其免疫表型及超微结构特征均具有神经鞘细胞分化的特点,因此推测该肿瘤可能为神经鞘瘤的一种新亚型。     

Sinonasal Masses: A Retrospective Analysis

To study clinico-pathological profile of sinonasal masses (SNM). This is a retrospective analytical review of 92 patients of SNM who presented to Department of ENT, S.S. Medical college & Associated Hospital, Rewa from January 2008 to August 2012. Their biodata, clinical profile and histolopathological diagnosis were analyzed. The study design was retrospective done in tertiary referral hospital setting. In the present study total 92 patients with age ranging from 7 to >70 years were analysed. The overall M:F ratio was 1.35:1. Most of the patients (35.86 %) belong to 11–20 year age group. The main presenting symptoms were nasal blockage (94 %) and rhinorrhoea (90 %). About 48.9 % cases showed mass on left side, 34.78 % on right side and 16.3 % bilateral. Maximum cases 42.39 % were reported to be antrochoanal polyp followed by ethmoidal polyp and angiomatous polyp. About 6.52 % cases were histopathologically reported to be malignant. This article gives an insight into prevalence of SNM in this region of North India thus advocating early recognition, proper diagnosis and treatment of SNM.     

Silent intratemporal facial nerve schwannoma associated with Chronic Suppurative Otitis Media: A rare presentation

Facial palsy is a common manifestation of intratemporal facial nerve schwannoma. Review of English literature describes intratemporal facial nerve schwannoma presenting as vertigo, tinnitus (without facial palsy) which were diagnosed on CT scan or MRI of temporal bone. We are presenting two cases of asymptomatic facial nerve schwannoma without facial palsy presenting only as Chronic Suppurative Otitis Media (CSOM), which were diagnosed incidentally during surgery.     

Versatility of Pectoralis Major Myocutaneous Flap in Oncosurgery and Its Role in Developing Countries

Schwannoma of nasal septum presentation is exceedingly rare in the literature, previously reported multiple lesions involving the paranasal sinuses or nasal mucosa. We report the case of a 20 year-old yellow male with a tiny schwannoma arising from the nasal septum, complaining of lightly alternate nasal obstruction. Both anterior and endo-rhinoscopy didn’t detect this tiny smooth-surfaced mass and other abnormality, except a little septal deviation. After final clinical diagnosis, we completely removed this tumor and corrected the deviation by an endoscopic approach. We discuss the clinical presentation, differential diagnosis, pathologic diagnosis factor and therapy of this rarely encountered neoplasm.     

The bony crescent sign -a new sign of facial nerve schwannoma

Schwannomas of the facial nerve are rare slowly growing lesions that have a predilection for the geniculate ganglion. Radiological evaluation is important in their diagnosis and in the assessment of their extent. In our series of 4 cases the facial nerve schwannoma was seen on high resolution CT as a soft tissue mass bounded anteriorly by a thin rim of bone. This bony crescent sign is a previously undescribed sign of facial nerve schwannoma which appears on the basis of this small series to be strongly indicative of the presence of this tumour. Schwannomas are relatively uncommon intracranial tumours. They most commonly involve the acoustic nerve followed in frequency by the trigeminal nerve. Other cranial nerves are rarely involved. Facial nerve schwannomas occur ring within the petrous temporal bone are very rare. Their diagnosis may be missed prospectively even when appropriate CT scans are per formed. Even in retrospect the site of abnormality may be difficult to identify, especially if there is an associated middle ear mass such as a cholesteatoma. Lesions occurring in the petrous area are all rare. The differential diagnosis includes cholesterol granuloma, epidermoid, carotid aneurysm and, very rarely, primary and secondary bone tumours. We describe a new sign associated with facial nerve schwannoma on CT, that of a bony crescent. Recognition of this sign makes those tumours arising in the region of the geniculate ganglion easy to diagnose prospectively.     

鼻内镜下手术治疗侵及眼眶的鼻腔鼻窦肿瘤

目的： 探讨鼻内镜下手术治疗侵及眼眶的鼻腔鼻窦肿瘤的安全性、临床效果和术式的选择。方法:回顾性分析15例在鼻内镜下行切除手术治疗的侵及眼眶的良性和早期恶性鼻腔鼻窦肿瘤患者的临床资料、手术方法、并发症及治疗预后。结果:全部病例中,11例单纯经鼻内镜下切除肿瘤,4例经鼻腔和鼻外切口双径路手术治疗,其中 1例侵袭性大B细胞淋巴瘤患者术后出现下睑外翻,经治疗后好转;1例鼻腔鼻窦骨瘤患者于鼻内镜下全切骨瘤,术后出现患侧内眦外移;4例良性骨源性病变患者行鼻内镜下病变部分切除术,其余病例均彻底切除肿瘤而无明显并发症,随访6~36月未见复发。结论:鼻内镜下手术治疗侵及眼眶的鼻腔鼻窦肿瘤有其独特的优势,在鼻内镜下单纯经鼻腔或内镜辅助经鼻腔和鼻外切口双径路手术治疗可以取得比较好的临床预后。     

Sinonasal Malignant Lymphoma of Natural Killer Cell Phenotype Associated with Diffuse Pancreatic Involvement

We report a case of sinonasal lymphoma with a natural killer (NK) phenotype. This 40-year-old man was admitted to our hospital because of left nasal obstruction. Physical examination and computed tomography of the skull revealed a tumor in the left nasal cavity and maxillary sinus. Histopathological examination revealed a diffuse proliferation of pleomorphic lymphoid cells. Imprint cytology showed that tumor cells contained some azurophilic granules, and expressed CD2, CD8, CD16, CD56 and HLA-DR antigens with little expression of other lymphoid or myeloid markers. Southern blot analysis revealed germline configuration for immunoglobulin heavy chain and T-cell receptor genes. These findings indicated that these cells were in fact NK cells. The patient's enlarged pancreas was also involved by lymphoma and the pattern of involvement simulated that seen in primary pancreatic lymphoma. Ulcerative colitis (UC) was also present, a rare finding in this disorder.     

Sinonasal inverted papilloma associated with squamous cell carcinoma

Background

The aims of the study were to review single-institution experiences with sinonasal inverted papilloma associated with squamous cell carcinoma (IP/SCC), to analyze the presence of human papillomavirus (HPV) and to evaluate the role of radiotherapy.

Patients and methods

Five patients with IP/SCC were identified in the prospective institutional databases (1995–2005) and HPV status was determined in all five tumors.

Results

Four out of five patients had T3-4 tumors; no nodal involvement was seen in any of them. Four patients had curative surgery, supplemented in three of them with radiotherapy. Debulking surgery was performed in the patient with a non-resectable tumor followed by radical radiotherapy. Tumor was controlled locally in three patients at 8, 46 and 58 months post-surgery. Local failure occurred in two patients: after endoscopic resection of a T1 tumor (the recurrent tumor was successfully salvaged with additional surgery) and in a patient with an inoperable tumor. No regional or distant metastases occurred. HPV status was determined in all five tumors and three of them were found positive for HPV type 11.

Conclusions

In operable sinonasal IP/SCC, upfront surgery and postoperative radiotherapy to the tumor bed with dose levels comparable to those used for invasive SCC are recommended. For non-resectable disease, radical radiotherapy to a dose of 66–70 Gy could be of benefit.     

小儿恶性雪旺氏细胞瘤11例

（目的）探讨小儿恶性雪旺氏细胞瘤的发病特点及诊治方法。（方法）分析1988年6月至1997年10月收治的11例小儿恶性雪旺氏细胞瘤病例的临床病理特征、诊治方法及预后。（结果）颈部3例，腹部6例，纵隔及肩胛后各1例。其中3例为神经纤维瘤病变。均手术治疗，部分病例会并化、放疗。仅1例术后无瘤生存14个月，其余均在术后1-15个月死于肿瘤转移、局部浸润及相关并发症。（结论）小儿恶性雪旺氏细胞瘤发病不同于成人。辅助化、放疗有一定疗效，但预后不佳，迫切需要寻找新的综合治疗方法。     

Sinonasal teratocarcinosarcoma-A case report

Sinonasal teratocarcinosarcoma is a very rare tumor of ambiguous origin. Detailed examination of the biopsy material is essential for diagnosis. It is a very aggressive tumor with a five-year survival rate of 45%. Surgery with radiotherapy has been shown to be effective. We report a case of a 38-year old Indian gentleman with left nasoethmoidal mass eroding the cribriform plate with intracranial extension. Subtotal endoscopic excision of the mass revealed features of a sinonasal teratocarcinosarcoma. Immunohistochemistry confirmed the same. The patient was subsequently treated with radiotherapy. The clinical features, histopathological and immunohistochemistry findings and management of sinonasal teratocarcinosarcoma are discussed.     

A case of nodular pseudoangiomatous stromal hyperplasia (pash)

Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a common microscopic lesion that is found at breast biopsy, and presents with proliferation of the stromal cells and slit-like pseudovascular spaces with endothelial-like spindle cells. In contrast, nodular PASH is relatively rare. We report here a case of nodular PASH with multiple palpable masses. A 49-year-old woman who had experienced gradual enlargement of her breasts for 13 years noticed an elastic but firm palpable mass in her breast. We were able to detect 7 masses in her right breast and 2 in the left. Ultrasonography and mammography demonstrated nonspecific findings, and FNA and CNB did not establish a diagnosis. An excisional biopsy was performed, and the pathological findings revealed nodular PASH. Eighteen months after the excisional biopsy, the size of the nodules and the whole breast had decreased remarkably. While the possibility of a change in the hormonal background or the influence of drugs was considered, we were not able to reach a single specific conclusion regarding the pathogenesis.     

Hypoglossal nerve schwannoma with intratumoral hemorrhage

Intratumoral hemorrhage of hypoglossal schwannoma is very rare. A 37-year-old man was admitted to our hospital with severe headache and gait disturbance. Radiologic examination revealed subarachnoid hemorrhage and a well-circumscribed enhanced lesion compressing the medulla oblongata. Gross total removal of the intracranial tumor was performed. Histopathological examination revealed the characteristic features of a schwannoma. The tumor had several hemorrhagic areas and numerous hyalinized blood vessels. Although the mechanism of hemorrhage from schwannomas is unclear, it is hypothesized that tumor vessels showing focal sinusoidal dilatation and hyaline thickening of the walls lead to spontaneous thrombosis with consequent necrosis, and often hemorrhage.     

Malignant schwannoma in siblings with neurofibromatosis

Three brothers with neurofibromatosis (NF) died of sarcoma. The proband tumor had malignant schwannoma. The histologic nature of the sarcoma in the other two brothers was not known, but it seemed likely to be similar to that of the proband. However, the sarcomatous lesions in these two siblings developed in the patients' right lower limbs which showed clinical and radiological evidence of anterior bowing of the tibia. Their mother and two other siblings have neurofibromatosis, but have not developed malignant tumors. The unaffected father died at age 61 of carcinoma of the common bile duct. An unaffected sister developed adenocarcinoma of both colon and lung. Although it is known that patients with NF are at a high risk of developing malignant sarcoma and second primary malignancies, it is not clear whether this association is genetically determined. The family we report has some of the features of a "cancer family" and suggests that sarcomatous transformation in patients with NF may be genetically influenced. This report raises the possibility that the presence of congenital malformation and exposure to irradiation may predispose patients with neurofibromatosis to develop malignant schwannoma.     

Schwannoma of larynx — a rare presentation

Neurogenous tumours of the larynx are extremely uncommon. Schwannoma of larynx is a variant of neurogenous tumour. The patient underwent microlaryngoscopic excision of that tumour. We present here the clinical findings of the case, along with direct laryngoscopic view, the photograph of the mass after removal and histological slide compatible with the diagnosis of schwannoma.