Sinonasal amelanotic melanoma with neuroendocrine differentiation: a diagnostic conundrum.

Mucosal malignant melanoma of the head and neck (HN) is a rare and aggressive neoplasm which constitutes only 1% of all melanomas. Neuroendocrine differentiation is an extremely unusual phenomenon in mucosal melanomas, of which five cases have been reported. We report a rare case of a 63-year-old female who developed sinonasal amelanotic melanoma with immunohistochemical expression of neuroendocrine markers, presenting a diagnostic dilemma. Ultrastructural evidence of melanosomes and neurosecretory granules aided in arriving at the diagnosis. Aberrant immunoexpression of neuroendocrine markers, particularly in an amelanotic melanoma, has critical diagnostic implications, as various malignancies with undifferentiated histomorphology occur at this site, many of which stain positively with neuroendocrine markers. We discuss the differential diagnoses and recommend a high index of suspicion so as not to miss the diagnosis of mucosal melanoma at this location.

Alveolar soft part sarcoma of the paranasal sinuses masquerading as a giant invasive pituitary adenoma.

Alveolar soft part sarcoma (ASPS) is a relatively rare tumor that mostly presents as a slow growing mass in the deep soft tissue of the extremities. A substantial number of cases in children occur in the head and neck region; however, in any age group, it is very rarely reported in the sinonasal region. We report a case of ASPS of the paranasal sinuses with sellar extension in a 25-year-old man that masqueraded as a giant invasive pituitary adenoma. This is only the fifth case of sinonasal ASPS in literature. The clinical and radiological diagnoses were misleading, but an extensive pathology workup including electron microscopy helped reach an accurate diagnosis in this unusual case.

[Clinicopathologic study of sinonasal inflammatory myofibroblastic tumor].

OBJECTIVE: To study the clinicopathologic features, immunophenotype and ultrastructural features of sinonasal inflammatory myofibroblastic tumors (IMT). METHODS: The clinical and histologic features of 5 cases of sinonasal IMT were reviewed. Immunohistochemical study for vimentin, MSA, SMA, calponin, h-caldesmon, desmin, ALK, fibronectin, CK, S-100 and Ki-67 was carried out. Ultrastructural examination was also performed in two of the cases. RESULTS: The patients age ranged from 28 to 62 years (mean = 43 years). The male-to-female ratio was 2:3. The clinical presentation included nasal obstruction, nasal discharge, nasal bleeding, facial pain, facial swelling, toothache and tear overflow. All of the 5 patients suffered from disease relapses; and 4 of them had recurrences for more than 5 times. One patient had lymph node metastasis and 3 patients died of the disease. Histologically, the tumor cells were arranged in interlacing fascicles and sometimes haphazard in fashion. They were spindly in shape, cytoplasm eosinophilic with mild nuclear atypia and a low mitotic activity. The intervening stroma was myxoid in appearance accompanied by lymphocyte and plasma cell infiltration, abundant blood vessels and focal collagenized areas. In 3 of the recurrent cases, the tumor cells displayed increased nuclear atypia and mitotic activity (average about 5 to 6 per 10 high-power fields), accompanied by patchy necrosis, less inflammatory cell infiltration and focal sarcomatous changes. Immunohistochemical study showed that the tumor cells were diffusely positive for vimentin. SMA, MSA, calponin and fibronectin were variably expressed. Desmin was weakly positive in 1 case. The staining for h-caldesmon, ALK, S-100 and CK was negative. The Ki-67 proliferation index increased with tumor recurrences. Electron microscopy revealed abundant rough endoplasmic reticulum and dense body formation in the cytoplasm. There were an increased amount of collagen fibers in the stroma. CONCLUSIONS: IMT rarely occurs in nasal cavity and paranasal sinuses. The tumor is prone to local invasion and recurrences, with subsequent progression to frank malignancy and distant metastasis, resulting in high mortality and poor prognosis. Complete surgical resection remains the main modality of treatment.

Sinonasal clear cell adenocarcinoma: a case report.

BACKGROUND: Nonsalivary adenocarcinomas are the most interesting tumors found in the sinonasal area. They are rare tumors arising from surface epithelium. The clear cell type of this tumor is even more rare. We present cytologic findings of clear cell sinonasal adenocarcinoma and related pitfalls. CASE: A 52-year-old woman presented with a left-cheek facial mass of 3-4 years' duration, with progressive enlargement, nasal discharge and discoloration of the lateral side of her left eye. Computed tomography was performed, revealing an expansile mass involving the nasal cavity, left maxillary sinus, ethmoid sinus with extension to sphenoid sinus, left side of oral cavity and left orbit. Fine needle aspiration performed through the upper buccogingival canine fossa showed clusters of epithelial cells with clear cytoplasm, round nuclei, inconspicuous nucleoli and slight pleomorphism. Some normal ciliated columnar epithelial cells are identified in the vicinity of neoplastic cells. The mass was reported to be a clear cell neoplasm, and excision of the whole mass was performed. CONCLUSION: Cytologic findings of this rare tumor overlap with those of salivary gland-type tumors with clear cell change and should be added to the list of head and neck tumors with clear cell change.

Myofibroblastic sarcoma of the nasal cavity and paranasal sinus: a clinicopathologic study of 6 cases and review of the literature.

OBJECTIVE: We describe the clinicopathologic features of 6 cases of myofibroblastic sarcoma (MS) occurring in the nasal cavity and paranasal sinus. STUDY DESIGN: The paraffin-embedded tissues of 6 cases of MS were stained immunohistochemically and examined by electron microscopy. RESULTS: Clinically, a painless enlarging mass was the most common symptom, followed by the nasal obstruction, epistaxis, copious rhinorrhea, and proptosis. Histologically, the tumors showed a diffusely infiltrative growth pattern and consisted mainly of spindle cells with abundant eosinophilic cytoplasm. The hypocellular myxoid areas and the hypercellular fibrous areas were identified. Immunohistochemically, all 6 tumors were positive for vimentin, alpha-smooth muscle actin, calponin, and fibronectin. Ultrastructural examination in 3 cases showed characteristic features of myofibroblast. Follow-up in 6 patients revealed high local recurrence rate (6 out of 6). CONCLUSION: Myofibroblastic sarcoma of the nasal cavity and paranasal sinus exhibit diverse histologic appearances and a strong aggressive behavior.

[Sinonasal-type hemangiopericytoma: a clinicopathologic analysis of 6 cases].

OBJECTIVE: To study the clinicopathologic features, histologic diagnosis and differential diagnosis of sinonasal-type of hemangiopericytoma (SNTHPC). METHODS: The clinical, radiographic and pathologic findings of 6 cases of SNTHPC were analyzed. Immunohistochemistry and electron microscopy were performed on selected examples. RESULTS: Amongst the 6 patients studied, 4 were males and 2 were females. The age of patients ranged from 56 to 71 years (mean = 60.5 years old). The commonest clinical presentation was nasal obstruction and/or epistaxis. Other symptoms could include increased nasal secretion, eyeball pain, decreased visual acuity, increased tear secretion and headache. The tumor involved nasal cavity and/or paranasal sinuses. Gross examination showed polypoid tumor masses, brownish fleshy tissue or whitish tumor tissue fragments. Histologically, the tumor showed a mixture of diffuse, fascicular, storiform, reticulated and whorled growth patterns. The tumor cells were spindle-shaped and possessed clear to eosinophilic cytoplasm. Mitotic figures were rarely seen. The intervening vasculature was characteristically thin-walled, with focal hyalinization changes and rarely the staghorn pattern. Immunohistochemical study showed that the tumor cells expressed vimentin (6/6), smooth muscle actin (5/6) and CD34 (3/6). Electron microscopy demonstrated the presence of intracytoplasmic myofilaments. The tumor cells were linked together by primitive cell junctions. In general, the histologic diagnosis of SNTHPC was difficult, and only 1 case had the correct initial pathologic diagnosis made. Follow-up data were available in 5 patients and 2 of them had local recurrences. CONCLUSIONS: SNTHPC is a low to intermediate grade soft tissue tumor with pericytes differentiation. Correct diagnosis relies on detailed pathologic assessment and application of ancillary investigations.

Extramedullary plasmacytoma extensively affecting the sella turcica and paranasal sinuses.

We report a 62-year-old Japanese male who complained of double vision and showed clear boundary mass extending to the clivus, intrasella, suprasella, ethmoidal sinus and sphenoid sinus on neuroimaging. The tumor mass was partially resected via transsphenoidal approach and was diagnosed as the extramedullary plasmacytoma by IgA immunostaining and electron microscopy. Making diagnosis from the imaging findings was difficult in this rare case, but immunohistological and electron microscopic examinations were useful for pathological diagnosis.

HIV and plasmablastic lymphoma manifesting in sinus, testicles, and bones: a further expansion of the disease spectrum.

Patients with HIV infection are at increased risk for B-cell neoplasms and plasma cell dyscrasias. Both B cell and plasma cell tumors tend to be intermediate or high grade and are frequently associated with Epstein-Barr virus. Patients with HIV infection are also at higher risk of acquiring plasmablastic lymphoma. Until this time, only sinus, oral gastrointestinal, and lung manifestation have been noted. In this report we describe a 41-year-old male with HIV infection who developed multiple pleomorphic, extramedullary plasmablastic lymphomas associated with Epstein-Barr virus. We review the clinical and immunological features of his malignancy and thereby expand the spectrum of disease to include additional sites (bones, testicles) not previously reported.

Sinonasal teratocarcinosarcoma: ultrastructural and immunohistochemical evidence of neuroectodermal origin.

The authors report a case of sinonasal teratocarcinosarcoma in a 37-year-old man, which was located in the anterior skull base and extended to the right nasal cavity and paranasal sinuses. The tumor was surgically resected twice, but it could not be removed completely. Microscopically, it was mainly composed of primitive cell nests within a moderately cellular stroma. The components of squamous cell epithelia with focal teratoid appearance and adenocarcinomatous differentiation were observed. There were many rhabdomyoblasts scattered in the nests and stroma. Ultrastructurally, the primitive cells had many neural processes with parallel microtubules, resembling olfactory neuroblastoma. Rhabdomyoblasts showed various degrees of skeletal muscle differentiation. Some of the stromal spindle cells had actin filaments with dense patches and dense core granules. Immunohistochemically, the primitive cells were positive for epithelial markers, neuron-specific enolase, synaptophysin, and myogenic regulatory proteins. The rhabdomyoblasts showed immunoreactivity for myoid markers, cytokeratin, epithelial membrane antigen, and synaptophysin. Most of the stromal spindle cells were positive for smooth muscle actin, neuron-specific enolase and synaptophysin. The immunohistochemical and ultrastructural findings suggest that primitive cells had the most primitive phenotype of placodes, and support the possibility that sinonasal teratocarcinosarcoma is essentially a neuroectodermal tumor with divergent differentiation.

Tumores de las fosas nasales y senos paranasales. Tumores no epiteliales / Tumors of the nasal fossae and paranasal sinuses. Non epithelial tumors

Los tumores no epiteliales de las fosas nasales incluyen tumores de origen mesectodérmicos y neuroectodérmicos. Las neoplasias mesectodérmicas mas frecuentes son los vasculares y de estos los angiofibromas son los mas frecuentes y le siguen los hemangiomas, los angiosarcomas son excepcionales, Los linfomas son raros no obstante el anillo de Waldeyer. Los mixomas son frecuentes y por su vecindad con la órbita y la cavidad craneana aunque su comportamiento es benigno son muy destructivos y pueden llevar a la muerte. Dentro de los sarcomas lo rabdomiosarcomas son los mas frecuentes

Amphicrine tumor.

The term amphicrine refers to cells, and tumors, which show both exocrine and endocrine features. Author s aim was to analyse the characteristics of these neoplasms. 40 suspicious cases were reviewed. Mucin-stains (PAS, diastase-PAS, Stains-all, Alcian-blue), immunohistochemistry (antibodies against Neuron-Specific Enolase (NSE), and Chromogranin A (CGA), and electronmicroscopic studies were performed to demonstrate exocrine and/or endocrine features of the tumor cells. By means of these methods, 16 cases turned out to be amphicrine tumors. Among them, there were 4 sinonasal, 1 bronchial, 1 mediastinal, 8 gastrointestinal and 2 suprarenal gland neoplasms. In connection to the subject, a brief review is given of amphicrine tumor, regarding its etiological and pathological aspects. These tumors form a distinct clinicopathological entity and should be separated from both neuroendocrine tumors and adenocarcinomas.

Epithelial-myoepithelial carcinoma: An unusual tumor of the paranasal sinus.

A 65-year old woman presented with nasal obstruction and on examination was found to have a huge mass in the maxillary sinus. This was removed, and histological examination revealed a mixture of trabecular structures consisting of inner dark cells, outer clear cells and solid structures consisting of only clear cells. Immunohistochemical examination showed the clear cells to be positive for alpha-smooth muscle actin. Ultrastructural examination confirmed the myoepithelial cell origin. The characteristic morphological, immunohistochemical and ultrastructural features aided in the diagnosis of epithelial-myoepithelial carcinoma.

[Primary nasal sinus malignant melanomas]. / Melanomas malignos primarios nasosinusales.

Primary malignant melanoma of the mucosa of the nasal and paranasal sinuses is rare, difficult to treat, and usually has a poor prognosis. Seven cases, six women and one man (mean age 76.5 years), were treated at the Hospital 12 de Octubre (Madrid, Spain) during the period 1982-1994. The presenting symptoms, diagnostic evaluation, morphology, therapeutic modalities, histological evaluation, and clinical course were reviewed. Three patients were treated surgically and three received palliative irradiation. All had local recurrence or distant metasfases within 15 months after concluding treatment. The seventh patient refused treatment.

[Primary malignant melanoma of the mucosa of the nasal septum. A case report]. / Melanoma maligno primario de mucosa del tabique nasal. A propósito de un caso.

Mucosal melanomas are rare, representing 3% of malignant nasosinusal tumors. Their prognosis is poor. A case of malignant melanoma of the nasal septum is presented. A bibliographic review was made and the incidence, clinical features, diagnosis, treatment, and prognosis of these tumors are discussed.

Pharyngeal hypophysis.

Derived from embryologic remnants of Rathke's pouch and its craniopharyngeal duct, the pharyngeal hypophysis has long been considered to be present in all humans. This thesis has been recently challenged, but the weight of investigative evidence still favors the original premise. The pharyngeal hypophysis is just one product of Rathke's pouch. Others are extracranial pituitary tumors, the very rare craniopharyngioma, pharyngeal hypopharyngeal cysts, and cerebrospinal fluid rhinorrhea.

[Hemangiosarcoma of the nasal cavity]. / Hemangiosarcoma de fosas nasales.

Hemangiosarcoma is a rarely diagnosed in the nasal cavity and there are few reports in the ENT literature. The most interesting aspect of our 82-year-old male's case was that he presented all the findings characteristic of angiosarcoma, but the final post-mortem diagnosis was unexpected because of the poor information available until that moment.

Fine-needle aspiration of chondroblastic osteosarcoma of the skull: report of a case in an 11-year-old girl.

We describe an unusual case of chondroblastic osteosarcoma of the skull in an 11-yr-old girl in whom a preoperative diagnosis was made by fine-needle aspiration (FNA) biopsy, followed by histologic confirmation of tissue biopsy and the surgically resected specimen. FNA cytology revealed pleomorphic oval cells with prominent nucleoli along with spindle cells, tumor giant cells, and a chondromyxoid background. The cell block of the aspirated material showed osteoid associated with the malignant cells. Immunocytochemical stains revealed S-100 and vimentin positivity; actin, myoglobin, and cytokeratin stains were negative. Electron microscopy revealed neoplastic cells with chondrocytic differentiation. This case demonstrates the value of FNA biopsy combined with immunocytochemical and ultrastructural studies performed on the aspirated material in diagnosing osteosarcoma from an unusual location such as the base of the skull.

Ethmoidal myofibroblastoma.

Myofibroblastoma is an uncommon mesenchymal tumour characterized by spindle cells exhibiting the ultrastructural and immunohistochemical features of myofibroblasts. We report a case of a myofibroblastoma occurring in the ethmoid sinus, which to our knowledge is the first case reported in the literature. The clinical presentation, diagnosis, histology, and management of this lesion is discussed with a review of the relevant literature.