Primary malignant lymphoma in the sphenoid sinus with orbital apex syndrome; a case report]

Only 2.0-6.8% of extranodal malignant lymphomas are found in the nasal region and paranasal sinuses. Primary malignant lymphoma of the paranasal sinuses usually occurs in the maxillary or ethmoid sinuses, and is very rare in the sphenoid sinus. Here we report a rare case of primary malignant lymphoma of the sphenoid sinus that was found accompanying orbital apex syndrome. The patient's progressively deteriorating neurological condition was improved after surgery via the transsphenoidal approach. A 52-year-old man was admitted with reduced left visual acuity, diplopia, and retroorbital pain. CT showed an isodense mass in the sphenoid sinus with slight enhancement, and MRI showed that the lesion was slightly hypointense on T1-weighted images, hypointense on T2-weighted images, and slightly enhanced by Gd-DTPA. On January 19, 1989, the patient suddenly became blind. An operation via the transsphenoidal approach was done as an emergency procedure to decompress the sphenoid sinus and the left optic canal. The histological diagnosis was non-Hodgkin's lymphoma of the diffuse large cell type (B cell lymphoma). Malignant lymphoma in the paranasal sinuses is usually biopsied and treated by chemotherapy and/or radiotherapy without surgical resection. In this rare case, an operation via the transsphenoidal approach was effective in improving the patient's visual acuity.     

Carcinosarcoma of the nasal cavity and paranasal sinuses

Malignant neoplasms consisting of an epithelial element and 1 or more nesenchymal components are variously termed teratocarcinosarcoma, carcinosarcoma, malignant teratoma, spindle cell carcinoma, and pseudosarcomatous squamous cell carcinoma. Carcinosarcoma, consisting of a malignant epithelial element and a single malignant mesenchymal component, is extremely rare in the sinonasal tract. We report a case of carcinosarcoma involving the nasal cavity, paranasal sinuses, and anterior cranial fossa. Rapid growth and extensive local destruction are prominent features of this tumor, emphasizing the need for early diagnosis and prompt institution of aggressive therapy. The clinical presentation, pathologic features, and clinical course are detailed.     

Cavernous hemangioma of the sphenoid sinus: case report and review of the literature

BACKGROUND: Few cases of paranasal sinus cavernous hemangiomas have been reported in the literature. We report the first case of cavernous hemangioma of the sphenoid sinus and discuss therapeutic considerations and differential diagnosis. CASE DESCRIPTION: A case of sphenoid sinus tumor in a 67-year-old woman is reported. The initial symptoms were a horizontal diplopia, a left facial dysesthesia, and a recent history of unusual headache. Physical examination revealed a left VIth nerve paresis. A CT scan was performed showing a hypodense homogeneous mass in the sphenoid sinus that was not enhanced after administration of contrast medium. MR study demonstrated on T1-weighted image an isointense nonenhancing homogeneous mass filling the sphenoid sinus. On T2-weighted images the tumor mass displayed a mild hyperintense signal. The patient was operated on via a transsphenoidal approach with total removal of the tumor. Pathological findings were consistent with a nonosseous cavernous hemangioma. MR imaging performed 5 years later was still normal. CONCLUSION: The clinicoradiological and pathological features of this entity are described, and the literature reviewed.     

Acantholytic Squamous Cell Carcinoma in Upper Aerodigestive Tract: Histopathology, Immunohistochemical Profile and Epithelial Mesenchymal Transition Phenotype Change

Acantholytic squamous cell carcinoma is a rare variant of squamous cell carcinoma in the mucosa of upper aerodigestive tract. Histomorphologically, acantholytic squamous cell carcinoma may lose the typical features of conventional squamous cell carcinoma and mimic other epithelial or mesenchymal malignancies due to advanced acantholysis and dyskeratosis. Because of its rarity, information of prognosis, pathologic features and immunohistochemical profiles is limited. We have studied clinicopathologic features and immunohistochemical profiles of four acantholytic squamous cell carcinoma cases arising from upper aerodigestive tract. Clinical results indicate an aggressive biologic behavior. Morphologically, all tumors revealed significant acantholysis with separation of tumor cells and intratumoral spaces. The tumor cells were highly pleomorphic and growth patterns were variable. In immunohistochemical studies, all tumor cells revealed positive reactions for AE1/AE3 and p63 supporting a squamous epithelial origin. In contrast to conventional aerodigestive squamous cell carcinoma, acantholytic squamous cell carcinoma showed significant reductions of cytokeratin19, E-cadherin and concomitant up-regulation of vimentin expression. Both morphologic features and immunohistochemical profiles indicate that acantholytic squamous cell carcinoma has acquired an epithelial mesenchymal transition phenotype. However, in contrast to other solid malignant tumors, the epithelial mesenchymal transition phenotype change in acantholytic squamous cell carcinoma is not limited to the invasive front of the peripheral tumor but, rather, diffusely involves entire neoplastic lesion. In addition, because cytokeratin 19 staining is attenuated, this would be an insensitive marker for following up and/or in detecting disseminated tumor cells in cases of acantholytic squamous cell carcinoma in upper aerodigestive tract.     

Basaloid-squamous carcinoma of the esophagus: report of a case

We report herein the case of a 66-year-old man with basaloid squamous cell carcinoma (BSCC) of the esophagus. There are only 60 cases of BSCC of the esophagus previously reported in Japan. In our patient, endoscopic findings revealed a type 2 in the lower intrathoracic esophagus (Lt), and the tumor was intact with the aorta (T2). A biopsy suggested that it was adenosquamous cell carcinoma. An operation was done on June 11, 1996. With open thoracotomy, esophagectomy was performed with mediastinal lymphnode dissection by posterior mediastinal esophagogastrostomy. Histologically, the lesion of the tumor with ulceration was composed of BSCC, and other lesion was composed of typical squamous cell carcinoma (SCC). The immunohistochemical findings of the respected specimens led us to suspect that the basal-layer-type SCC had transformed into BSCC by undergoing differentiation and expansive proliferation. According to the prognosis, eventually the patient died of pneumonia due to methicillin resistant Staphylococcus aureus (MRSA) and candida 4 years and 9 months after the surgery.     

Abducens nerve paralysis: a potential presentation of sphenoid sinus cancer

The sphenoid sinus is the paranasal sinus most commonly implicated when cranial neuropathies are present. Two patients presenting with sixth nerve paralysis secondary to sphenoid sinus involvement are presented. One patient had a primary sphenoid sinus tumor, and the other a metastasis from a bronchogenic carcinoma. Of the two patients, one carried the diagnosis of idiopathic sixth nerve paresis and had had a normal sinus x-ray film and CAT scan done previously. Even in the absence of positive radiographic findings, the high clinical suspicion of sinus malignancy must be maintained in patients manifesting abducens nerve paralysis. In these patients, the petrous apex and cavernous sinus "silent area" must be diligently evaluated. For the patient to have any chance for palliation or potential cure, the tumor must be diagnosed as soon as possible.     

Unique characteristics of malignant schneiderian papilloma

PURPOSE: To ascertain the characteristics unique to malignant schneiderian papilloma (MSP). METHODS: A case-control study of all schneiderian papilloma (SP) patients treated between 1978 and 1997 was conducted. Comparison was made between patients with MSP and patients with benign SP (BSP). RESULTS: A diagnosis of SP was made in 72 patients. Malignant changes, all of them the inverted papilloma subtype, were found in 8 of these patients. Three were diagnosed carcinoma in situ, and 5 were defined as invasive squamous cell carcinoma. At presentation, the MSP patients had significantly larger tumor spread into the ethmoid and sphenoid sinuses. The recurrence rate was significantly lower in SP patients treated with extensive surgical procedures. An association was found between the presence of malignant lesions and positive smoking history, subjective awareness of a nasal mass, and ethmoid and sphenoid sinus involvement. Also, histologic multicentricity was a feature more often seen in MSP than BSP and was a significant correlate with malignancy. CONCLUSION: The physician evaluating a patient with SP should be aware of the features described and of their possible association with a malignant lesion.     

Thickening of sphenoid sinus mucosa during the acute stage of pituitary apoplexy.

The authors treated two patients with pituitary apoplexy in whom magnetic resonance (MR) images were obtained before and after the episode. Two days after the apoplectic episodes, MR imaging demonstrated marked thickening of the mucosa of the sphenoid sinus that was absent in the previous studies. The relevance of this change in the sphenoid sinus was investigated. Retrospective evaluations were performed using MR images obtained in 14 consecutive patients with classic pituitary apoplexy characterized by acute onset of severe headache. The mucosa of the sphenoid sinus had thickened predominantly in the compartment just beneath the sella turcica, in nine of 11 patients, as ascertained on MR images obtained within 7 days after the onset of apoplectic symptoms. This condition improved spontaneously in all four patients who did not undergo transsphenoidal surgery. The sphenoid sinus mucosa appeared to be normal on MR images obtained from three patients at the chronic stage (> 3 months after onset). The incidence of sphenoid sinus mucosal thickening during the acute stage was significantly higher in the patients with apoplexy than that in the 100 patients without apoplexy. A histological study conducted in four patients who underwent transsphenoidal surgery during the early stage showed that the subepithelial layer of the sphenoid sinus mucous membrane was obviously swollen. The sphenoid sinus mucosa thickens during the acute stage of pituitary apoplexy. This thickening neither indicates infectious sinusitis nor rules out the choice of the transsphenoidal route for surgery.     

Squamous cell carcinoma in chronic ulcerative lesions: a case report and literature review

A case report and literature review are presented involving a fatal case of squamous cell carcinoma of the lower extremity. The unique aspects of this patient include the young age at presentation (35 years old), and the association with both a burn scar (Marjolin's) ulcer and a draining osteomyelitis fistulus tract. Epidemiologic data for Marjolin's ulcers as well as squamous cell carcinoma associated with draining sinus tracts of osteomyelitis are reviewed, in addition to the recommended management of such patients. The case presented is a reminder of the need to maintain a high index of suspicion for malignant transformation within ulcerative lesions.     

Primary pituitary carcinoma: a clinical pathological study

A 60-year-old woman presented with a history of headache and rapidly progressive complete bilateral ophthalmoplegia. Her endocrine function was normal. Radiographic studies revealed signs of diffuse destruction of the sella turcica by a tumor without suprasellar extension. Biopsies of the lesion within the sphenoid sinus demonstrated the presence of a primary pituitary carcinoma; autopsy findings revealed metastatic deposits in the liver, kidney, and lung. The histological characteristics of these cells and the electron microscopic findings confirmed that the tumor can first manifest themselves by the rapid development of unilateral or bilateral ophthalmoplegia; this diagnosis should be considered in all individuals with such a syndrome.     

Verrucous carcinoma (Buschke–Lowenstein) arising in a sacrococcygeal pilonidal sinus tract: report of a case

Purpose Pilonidal sinus disease is usually located in the sacrococcygeal region. Verrucous carcinoma (Buschke–Lowenstein) is a rare variant of squamous cell carcinoma with distinct clinical and histopathologic features, with a favorable prognosis. Malignant degeneration is an infrequent complication, which is associated with a high recurrence rate and poor prognosis. Materials and methods A case report is presented. Results We report a 48-year-old man with a 10-year history of recurrent pilonidal sinus disease presented with a sacrococcygeal verrucous carcinoma arising in the pilonidal sinus tract and associated with good prognosis. After operation, a 5-year disease-free follow-up was observed. Conclusion Careful inspection of the pilonidal area in all chronic and long-standing inflammatory processes is important and should be evaluated for malignant transformation.     

Fungal granuloma of the sphenoid sinus and clivus in a patient presenting with cranial nerve III paresis: case report rand review of the literature

OBJECTIVE AND IMPORTANCE: Isolated fungal granulomas originating within the sphenoid sinus are extremely rare in immunocompetent patients. In their symptoms and morphological appearance, these lesions may be mistaken for pituitary tumors. We report such a case and review the literature. CLINICAL PRESENTATION: A 74-year-old man presented with a 3-week history of Cranial Nerve III paresis. The patient had a long-term history of snuff abuse. Computed tomography demonstrated a space-occupying lesion of the sellar and sphenoid sinus region with displacement of the cavernous sinus. INTERVENTION: The lesion was operated on via a transnasal-transsphenoidal approach. After the sphenoid sinus was opened, mucus extruded spontaneously, and a brownish, crumbly mass was found and removed. The lesion had completely eroded the sella and clivus. Histological analysis revealed numerous Aspergillus hyphae. Postoperatively, the IIIrd cranial nerve paresis resolved completely within a few days. No systemic fungal infection was found in extensive serological studies. There was no evidence of immunosuppression. CONCLUSION: Fungal granuloma must be included in the differential diagnosis of lesions in the sellar region, even in nonimmunosuppressed patients. Early diagnosis and transsphenoidal extirpation is crucial with this potentially life-threatening disease.     

Diplopia - an unusual primary manifestation of metastatic renal cell carcinoma

It is relatively unusual that initial symptoms of renal cell cancer begin with the metastatic involvement of other sites. Intracranial metastases especially in the paranasal sinuses may be unusual not only to surgeons but also to physicians. In this report, we present a case where a metastasis was first manifest as a sphenoid sinus secondary with ocular and visual disturbances prior to the demonstration of the primary tumor site. It turned out to be a case of diplopia masquerading as a metastatic renal cell cancer.     

Squamous carcinoma of the nasal cavity and paranasal sinuses

This study retrospectively analyzed 105 patients with squamous carcinoma of the nasal cavity and paranasal sinuses. The primary tumor was located in the maxillary sinus in 65 patients (62 percent), the nasal cavity in 27 (26 percent), the ethmoid sinus in 11 (10 percent), and the sphenoid sinus in 2 (2 percent). Over half of the patients with antral cancer were treated with surgery and radiotherapy, whereas one-third of the remaining patients received combination therapy. Most procedures were radical, including sacrifice of the orbital contents in half of the surgically treated patients. The 5-year determinate cure rate was 45 percent for patients with nasal cavity tumors, 38 percent for those with maxillary sinus lesions, and 13 percent for those with ethmoid tumors. Local recurrence remains a major problem despite aggressive surgery and increased use of adjunctive radiotherapy.     

Malignant melanoma arising from the sphenoidal sinus--case report

Malignant melanomas arising from the sella turcica or sphenoidal sinus with bilateral invasion of the base of the skull or cavernous sinus are extremely rare. Whether the sella turcica or sphenoidal sinus is the site of origin is difficult to determine based on neuroradiological findings. An 83-year-old Japanese female presented with headache as the initial symptom. She suffered rapid progression of bilateral obstruction of the nasal cavity, left nasal bleeding, and bilateral visual field defects. The preoperative diagnosis was pituitary adenoma, metastatic tumor, or malignant paranasal tumor. Biopsy was performed. The histological diagnosis was malignant melanoma. Postoperatively, the tumor progressed rapidly. She suffered several cranial nerve pareses and hypopituitarism. She died within 6 months. Tumors arising from the sphenoidal sinus cause obstruction of the nasal cavity or nasal bleeding first, and then cause cranial nerve pareses by invasion of the cavernous sinus. This sequence of clinical manifestations can be attributed to the anatomical relationships between the sphenoidal sinus, nasal cavity, and cavernous sinus. Differential diagnosis of the origin in the sella turcica or sphenoidal sinus appears to be relatively easy based on further observation of the clinical course and symptoms.     

Epidermoid cyst of the sphenoid sinus with extension into the sella turcica presenting as pituitary apoplexy: case report

BACKGROUND: Epidermoids of the central nervous system are rare tumors. They are usually found in the fourth decade of life and most commonly off midline in the cerebellopontine angle. We report here a rare case of an epidermoid arising from the sphenoid sinus with extension into the sella and adjacent structures with acute onset of neurological deficit. The significance of the clinical presentation resembling pituitary apoplexy and magnetic resonance imaging (MRI) findings is noted. CASE DESCRIPTION: A 25-year-old man presented with acute severe headache, diplopia, and decreased visual acuity. Examination revealed right-sided ptosis and paresis of the third cranial nerve on the right side. Computed tomography and MRI were suggestive of a slow-growing sphenoid sinus mass with extension into the sella. The sublabial transsphenoidal approach was used to remove the mass under direct visualization. The patient's neurological status improved to baseline both subjectively and objectively after the operation. CONCLUSION: Epidermoids, although rare, should be considered as part of the differential diagnosis when evaluating lesions of sphenoid sinus or sellar origin.     

Expression of PCNA, p53, Bax, and Bcl-X in oral poorly differentiated and basaloid squamous cell carcinoma: relationships with prognosis

BACKGROUND: The aim of this study was to compare the clinical features and proliferating cell nuclear antigen (PCNA), p53, Bcl-X, and Bax expression in primary oral basaloid squamous cell carcinoma (BSCC) and poorly differentiated squamous cell carcinoma (PDSCC) matched by stage and site and to assess the possible prognostic significance of these variables. METHODS: Seventeen cases of oral BSCC were compared with 27 PDSCCs matched by stage and tumor site. In addition, PCNA, p53, Bax, and Bcl-X expression in both carcinomas were evaluated in relation to their clinicopathologic features and prognostic values using the Kaplan-Meier method and Cox regression models. RESULTS: No statistically significant differences were found between the groups (BSCC and PDSCC) in regard to clinical features and immunohistochemical reactivity for antibodies PCNA, p53, and Bcl-X. In comparison with PDSCC, the BSCC group exhibited a higher Bax score (p = .031). The 5-year and 10-year overall survival, cancer-specific survival, and disease-free survival rates demonstrated no significant differences between the BSCC and PDSCC groups, and the PCNA, p53, Bax, and Bcl-X also showed no prognostic value. CONCLUSIONS: These results suggest that the clinical and biologic course of BSCC is similar to PDSCC in the oral cavity when clinical stage and site are matched.     

Rhabdomyosarcoma in the region of the sella turcica

Summary Intracranial extension of rhabdomyosarcoma from the face, nasopharynx or middle ear is rare.A 16-year-old boy presented with deterioration of vision and headache. CT scan revealed a soft tissue mass occupying the sphenoid and ethmoid sinuses, extending to the suprasellar fossa and impinging on the optic chiasm. The tumour, mimicking pituitary carcinoma, was removed by transsphenoidal craniotomy. Morphologic studies, including immunohistochemistry and electron microscopy, revealed that the tumour was a rhabdomyosarcoma. This case stresses the value of immunohistochemical and ultrastructural studies in the diagnosis of tumours occurring in the region of the sella turcica. The origin of this tumour was thought to be the sphenoid or ethmoid sinus. The pituitary gland appeared intact.     

Case of frontal sinus carcinoma for which anterior skull base surgery was performed: application for resection of orbita

Carcinoma of the nasal cavity and paranasal sinuses occupies only 3% of head and neck malignant tumors in the West, but 10% in Japan. Frontal sinus carcinoma is much rarer, occupying less than 1% of carcinoma of the nasal cavity and paranasal sinuses. In Japan, only 38 cases have been reported for 40 years, from 1924 to 1964. Almost all patients died within one year because only facial resection was performed. We report a case of frontal sinus carcinoma (squamous cell carcinoma) invasive the to skull base for which we performed anterior skull base surgery and can confirm two years survival in our institution. In this case, resection of the right orbit contents was performed, but careful consideration must be given to resection and repair of orbit contents for maintaining a patient's quality of life. Not only computed tomography scan and magnetic resonance imaging but also angiography is thought to be useful, especially for the ophthalmic artery (in this case, the lacrimal artery) is important.