Adénocarcinome de bas grade des fosses nasales : à propos d’un cas

Low-grade sinonasal adenocarcinomas are uncommon and recently described entities. Its histologic diagnosis is challenging. This tumour is characterized by a tendency to local invasion, and rare distant metastases. Well treated, the prognosis is excellent. We describe a case of low-grade nasal cavity adenocarcinoma and discuss the anatomoclinical, therapeutic and evolutionary characteristics of this malignant tumour. A 54-year-old female patient presented with a 10 years history of right-sided nasal obstruction and recurrent epistaxis. On examination the patient had a large, firm mass in the right nasal cavity. Endoscopic sinonasal surgery was performed. The lesion was found to originate from the posteriolateral wall of the right nasal cavity. Histopathology analysis identified a low-grade sinonasal adenocarcinoma. Upon follow-up 4 years after surgery, the patient exhibited no clinical evidence of recurrence. Low-grade sinonasal adenocarcinomas are poorly defined neoplasms, accounting for 4 to 20% of all sinonasal malignancies. The nasal cavity is the most frequently involved site. Low-grade sinonasal adenocarcinomas pose a diagnostic challenge for the pathologist because they must be distinguished from benign tumours, especially adenomas. The primary treatment of sinonasal adenocarcinoma is complete surgical excision.     

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.     

Management of a Sporadic Malignant Subfrontal Peripheral Nerve Sheath Tumor

Summary Malignant subfrontal (olfactory) peripheral nerve sheath tumors (MPNSTs) are exceedingly rare. Although meningiomas are the most common subfrontal extra-axial lesions, it is important to recognize that MPNSTs, which are radiographically similar to meningiomas, can also be present in this location. MPNSTs require more aggressive surgical and postoperative management than meningiomas. In this paper, we describe a patient with a subfrontal MPNST with unusual histological characteristics and present a review of the literature. A 49-year-old woman presented with chronic sinusitis and progressive headaches. A neurological examination revealed left-sided anosmia. Brain-imaging studies revealed a large left subfrontal mass with extension into the frontal and ethmoid sinuses and the nasal cavity. The patient underwent both a bifrontal transbasal craniotomy and a transnasal approach for an attempt at total resection of both the intradural and extradural components of the MPNST. The patient was treated postoperatively with radiation therapy, and had no evidence of recurrence at her follow-up examination 1-year after treatment. Subfrontal PNSTs are extremely rare and usually benign. The specific cell and nerve of origin for these tumors remains unknown. Our case shows that these rare lesions can present as a malignant variant and thus require aggressive surgical and postoperative management to provide long-term tumor control.     

A case report of esthesioneuroblastoma

Esthesioneuroblastoma (Olfactory Neuroblastoma) arises from the olfactory placode in the olfactory area of the nasal cavity. The age incidence ranges from 3 years to 79 years. It usually presents with nasal symptoms like obstructed nasal breathing and epistaxis. The diagnosis may be delayed for several months due to its slow growing nature. It may be misdiagnosed with other small round cell tumors. One such case is reported here due to its rare incidence, difficulty in early diagnosis and very aggressive behaviour compared to the reported series inspite of recommended treatment by different modalities.     

Serum squamous cell carcinoma antigen is a useful biologic marker in patients with inverted papillomas of the sinonasal tract

BACKGROUND: Inverted papilloma (IP) is a frequent benign sinonasal tumor that is characterized histologically by squamous metaplasia, epithelial acanthosis, and hyperplasia of the nasal epithelium. Because of its high recurrence rate and malignant transformation potential, careful long-term follow up is necessary. METHODS: The purpose of the current report was to study the expression of squamous cell carcinoma (SCC) antigen in sinonasal IPs and to evaluate the usefulness of SCC antigen as a biologic marker for the follow-up of patients with sinonasal IP. The expression of SCCA1 in three sinonasal IP cases, three sinonasal SCC cases, and cases of normal nasal epithelium were examined by Western blot analysis, and the SCCA1 expression pattern in 31 IP specimens and 4 carcinoma in IP specimens were evaluated immunohistochemically. The serum levels of SCC antigen in 11 patients with sinonasal IP also were analyzed. RESULTS: SCCA1 was overexpressed in all three sinonasal IP tissues compared with sinonasal SCC tissues or normal nasal epithelium. SCCA1 cytoplasmic immunoreactivity was detected in the suprabasal epidermal keratinocytes of all 31 sinonasal IP cases. In the four carcinoma in IP specimens, SCCA1 expression in the papillomatous lesion was more intense than in the cancerous lesion. The serum SCC antigen level was high in 10 of 11 patients with IP (91%) and significantly decreased after surgical resection of the tumors. CONCLUSIONS: The results of the current study indicate that SCCA1 frequently is overexpressed and may play a biologic role in the development of sinonasal IPs. Serum SCC antigen may be a useful biologic marker in patients with sinonasal IP.     

Polymorphic reticulosis: A case report

A 32 year old male came to us with right sided nasal obstruction, epistaxis and nasal mass. Biopsy confirmed the diagnosis of polymorphic reticulasis. which is infact malignant T cell lymphoma of the sinonasal region. The nasal T cell lymphoma causes slow progressive destructian of nose and midfacial region and still presents a diagnostic problem. The clinical and pathological characteristics and management of sinonasal lymphomas are discussed.     

头颈部黏膜恶性黑色素瘤的临床诊治现状及进展

头颈部黏膜恶性黑色素瘤是一类少见的恶性肿瘤,主要发生在鼻窦和口腔,5年生存率不到30%。目前治疗模式尚存争议。多数回顾性研究结果显示术后放疗可以提高LC,但OS率未见明显获益。本文旨在加强对头颈部黏膜恶性黑色素瘤的认识,包括其临床特点、诊断、分期、治疗模式和疗效等。     

Malignant schwannoma of the sinonasal tract

Malignant Schwannoma of the sinonasal tract is an extremely rare tumour. So far, only 19 cases have been reported in the literature. We report herein a case of malignant schwannoma involving nasal cavity, maxillary antrum and ethmoid sinus in a 60-year-old male patient. The patient was treated with surgery and post-operative radiotherapy. He currently remains free of the disease 15 months after the diagnosis and twelve months after completing therapy.     

Metastatic meningiomas: an unusual clinical and pathological diagnosis with highly variable outcome

Metastatic meningioma is a rare situation. We conducted a retrospective study from our databases and identified cases of metastatic meningioma. We report three presentations of patients with medical history of surgical removal of meningioma presenting several years later a liver tumor with bone metastasis or multiple lung tumors. These observations highlight the difficulty of the clinical and pathological diagnosis and the absence of consideration of metastatic state for histologically “benign” but clinically aggressive meningiomas in the current WHO 2007 classification of meningiomas. We also reviewed published cases of metastatic meningiomas since they are clearly distinguished from haemangiopericytoma.     

Papillary meningioma: a malignant variant of meningioma.

A series of 17 meningiomas histologically characterized by a papillary pattern is reported. This pattern was invariably associated with other histologic features of malignancy. The tumors often displayed aggressive clinical behavior marked by a high rate of local recurrence or the development of distant metastases. A relatively large proportion occurred in children. It is suggested that this variant of meningioma is sufficiently characteristic to justify its separation as a distinct clinicopathologic entity.     

Rare case of extraskeletal Ewings sarcoma of the sinonasal tract

Ewings sarcoma (ES) and primitive neuroectodermal tumor are closely related family of small round cell tumors seen in childhood and adolescence. The incidence of these tumors occurring in the head and neck region is just 2-7%. Mandible and maxilla are the most common sites, whereas involvement of the sinonasal tract is very rare. We report a case of extraskeletal ES of the sinonasal tract in a 29-year-old female who presented with nasal obstruction and epistaxis. The patient was treated with 14 cycles of chemotherapy, combined with surgery and radiotherapy with complete recovery. We present this case due to its rarity, to analyze the clinical, histopathological and immunohistochemical findings, so as to differentiate from other small round cell tumors of the sinonasal tract for appropriate treatment.     

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。     

鼻腔鼻窦肌上皮癌的生物学行为及诊治

目的 提高临床医生对鼻腔鼻窦肌上皮癌的认识。方法 回顾性分析我科近期收治的两例鼻腔鼻窦肌上皮癌临床资料，并复习文献。结果 1例为女性，47岁；1例为男性，54岁。临床表现为鼻塞、鼻腔暗红色肿物。鼻窦CT示鼻腔占位性病变。术前病理及免疫组化(S-100蛋白、平滑肌动蛋白、细胞角蛋白、波形蛋白等呈阳性染色)确诊“肌上皮癌”。两例均行鼻侧切开鼻腔肿瘤切除术，例1术后5月出现颈淋巴结转移，术后11月死于全身骨及肝转移。例2术后8月死于肺转移。结论 鼻腔鼻窦肌上皮癌极为罕见，迄今为止，仅8例报道。综合文献，该瘤具有生长迅速、广泛侵犯周围组织、颈部淋巴结转移率高、血行转移率高、治疗后易复发、预后差等特点。诊断依赖病理及免疫组化。治疗以根治性手术为主，对于cNO的患者,应行分区性颈淋巴结清扫术。     

Inverted papilloma involving the temporal bone and its association with squamous cell carcinoma: critical analysis of the literature

Inverted papilloma is a rare, benign neoplasm, which usually originates from the lateral nasal wall and can be locally aggressive, extending into surrounding structures. Unusually, this disease involves temporal bone. There have been only 18 reported cases in the literature. The available data regarding inverted papilloma of the temporal bone are reviewed, and its etiopathogenesis, recurrence rate and association with squamous cell carcinoma are discussed.     

Inverted papilloma involving the temporal bone and its association with squamous cell carcinoma: critical analysis of the literature

Inverted papilloma is a rare, benign neoplasm, which usually originates from the lateral nasal wall and can be locally aggressive, extending into surrounding structures. Unusually, this disease involves temporal bone. There have been only 18 reported cases in the literature. The available data regarding inverted papilloma of the temporal bone are reviewed, and its etiopathogenesis, recurrence rate and association with squamous cell carcinoma are discussed.     

A review of malignant meningiomas: diagnosis,characteristics, and treatment

Anaplastic or malignant meningiomas (WHO Grade III) represent the most rare but aggressive subtype, accounting for 1–3% of all intracranial meningiomas. Due in large part to their scarcity, malignant meningiomas have been understudied and therefore represent an area where significant clinical advances may be made. To this point, our understanding of the genetic and histologic attributes of these lesions has grown, though the management and treatment of these aggressive tumors is less well elucidated and thus has room for further study. In this review, we describe the current understanding of malignant meningiomas in terms of their genetic alterations and unique histologic markers. Using this as a foundation, we will then discuss the current therapeutic strategies for managing these lesions and the future direction that such interventions may take.     

Recurrent meningioma

Single meningiomas are histologically classified as benign tumors, but clearly malignant types have been encountered. The standard mode of management is total macroscopic removal with excision of the dural attachment and abnormal bone, if there is any. Despite this aggressive surgery, recurrence rates of approximately 9% have been reported with the removal of benign tumors, and the rate is much higher with the removal of malignant meningiomas. Recurrence most frequently occurs at the original tumor site and is most often explained by incomplete removal, which, in turn, is a function of the anatomic location of the tumor. Less common are regional recurrences, which may be explained on the basis of the multicentric origin of meningiomas. This theory may also explain the rare entity, "multiple meningioma." This article documents an unexpected regional recurrence of meningioma. The pertinent literature is reviewed.     

Sinonasal T Cell Lymphoma: A Case Report

Sinonasal lymphomas are aggressive locally destructive midfacial necrotizing lesions. Most of them initially diagnosed as lethal midline granulomas, a term which is slowly replaced by sinonasal lymphoma. Here is one such case report of sinonasal T cell lymphoma where there was a difficulty in diagnosis and required an incisional biopsy.     

Fibrosarcoma of the maxillary sinus

Fibrosarcoma arising in the sinonasal cavities are very rare. By the time of clinical diagnosis, they are usually advanced. Lack of adequate surgical margins predisposes these patients to tumor recurrences. Most common sites are the extremities, with only one percent of fibrosarcoma arising in the head and neck area. The imaging features of these tumors reflect their aggressive behavior. We report the case of a maxillary sinus fibrosarcoma in a 16 year-old male patient. Neoadjuvant chemotherapy, medial maxillectomy, adjuvant chemotherapy and subsequent external irradiation was perfomed.     

Unusual presentation of metastatic hepatocellular carcinoma in the nasal septum: a case report and review of the literature

Hepatocellular carcinoma with sinonasal metastasis is extremely rare. We report a case of a 49-year-old man who had a history of synchronous hepatocellular carcinoma and verrucous carcinoma of tongue. A painless and non-bleeding mass was found in the left nasal septum 16 months after hepatocellular carcinoma was diagnosed. On computed tomography, the mass was enhanced with contrast. It was resected and proved to be metastatic hepatocellular carcinoma. The patient was treated with radiotherapy to the nasal area and then with chemotherapy. He was still alive, 15 months after the appearance of the nasal metastasis.