Complete response to immunotherapy in sinonasal undifferentiated carcinoma.

Sinonasal undifferentiated carcinoma (SNUC) is an uncommon aggressive tumor. Locally advanced disease is usually diagnosed at presentation. Multidisciplinary approach is essential and aims to ensure optimal trimodal strategy. Induction chemotherapy is preferred in order to select patients who will benefit from chemoradiotherapy or surgery. Immunotherapy is not indicated in patients with recurrent SNUC. We describe an impressive response in a young man previously treated with radiotherapy and chemotherapy and demolitive surgery who had metastatic bone and lung disease. We also report data on PD-L1, next-generation sequencing, and neutrophil/platelets ratio.

A case report of sinusoidal diffuse large B-cell lymphoma in a STK4 deficient patient.

INTRODUCTION: Primary immunodeficiency diseases (PIDs), a rare group of gene defects with different manifestations, are at great risk of malignancy. The incidence of diffuse large B-cell lymphoma in the sinusoidal tract is quite rare with nasal congestion, stuffiness, and pain in maxillary sinus manifestation. Human serine-threonine kinase 4 (STK4) deficiency affects the immune system with recurrent bacterial and viral infections, mucocutaneous candidiasis, cutaneous warts, skin abscesses, T- and B-cell lymphopenia, and neutropenia. PATIENT CONCERN: In this study we describe the infrequent incidence and successful treatment of sinusoidal diffuse large B-cell lymphoma in a STK4 deficient patient with clinical manifestation of severe intractable headaches, unilateral swelling of her face, nasal congestion, stuffiness, and pain in maxillary. DIAGNOSIS: Clinical data including headaches, unilateral swelling of face, nasal congestion, stuffiness and pain in maxillary sinus with confirmed histopathology and magnetic resonance imaging finding confirmed sinusoidal diffuse large B cell lymphoma in a STK4 deficient patient. INTERVENTION: Six cycles of R-CHOP (rituximab with cyclophosphamide, doxorubicin, vincristine, and prednisolone) were administered and after each cycle, G-CSF support was used. Chemotherapeutic drugs were administered with standard dose and no dose reduction was done during the treatment. IVIG treatment continued during the courses of chemotherapy. OUTCOME: The index patient achieved complete response at the end of chemotherapy courses and was in remission for about 8 months afterward, prior to the date of the present report. CONCLUSION: PID patient are often at increased risk of malignancies. Sinusoidal diffuse large B-cell lymphoma is quite rare and prognosis is variable. Early attention to patient's manifestation, suitable treatment, and monitoring manifestations caused by PID are critical.

Sinonasal carcinomas: recent advances in molecular and phenotypic characterization and their clinical implications.

Sinonasal carcinomas are rare tumors with an aggressive clinical behaviour which frequently pose a number of problems regarding the interpretation of diagnostic findings and the treatment. In addition, in comparison with other malignancies of the head and neck region, an elevated fraction of sinonasal carcinomas can be attributed to occupational exposure. This review is focused on the recent advances in the molecular and phenotypic characterization of sinonasal carcinomas, and their possible implications for the interpretation of epidemiological data, as well as for the diagnosis and treatment of these rare malignancies. The increasing knowledge on their phenotypic and genotypic features is progressively leading to a refinement in diagnosis, especially for poorly differentiated and undifferentiated lesions, as well as to the identification of markers which can be potentially useful to identify the early phases of carcinogenesis, to detect subclinical disease, to predict the response to therapy, and finally, that may represent potential targets for alternative treatments.

Postradiation malignant fibrous histiocytoma of the maxillary sinus.

Malignant fibrous histiocytoma is the most common soft tissue sarcoma in adults. The common sites of involvement are the retroperitoneal space and limbs. The occurrence of this tumor in the head and neck is rare. Recent studies have shown that the occurrence of malignant fibrous histiocytoma in the head and neck can follow radiotherapy for the treatment of other tumors. We, herein, report a case of malignant fibrous histiocytoma, which developed 17 years after radiotherapy of a carcinoma in the maxillary sinus.

Carcinoma de seio maxilar: análise de dez casos / Maxillary sinus carcinoma: an analysis of ten cases

OBJETIVO: Avaliar o papel, principalmente da tomografia computadorizada, no estadiamento dos carcinomas dos seios maxilares. MATERIAIS E MÉTODOS: Foram analisados dez casos de carcinoma diagnosticados e tratados pelos Departamentos de Diagnóstico por Imagem e Cirurgia de Cabeça e Pescoço do Hospital Heliópolis, São Paulo, SP, entre 1988 e 2002. RESULTADOS: Nove pacientes tiveram extensão tumoral para a bochecha, oito para o espaço mastigador, sete para o assoalho da boca e palato duro, cinco para a fossa pterigóide, cinco para a órbita, três para o etmóide e um para a base do crânio. Três pacientes foram classificados como T3 e sete, como T4. Dois tinham metástases linfonodais no momento da apresentação inicial, os quais pertenciam ao estágio T4. Todos os casos foram confirmados com exame histopatológico. CONCLUSÃO: A análise precisa da extensão local e disseminação tumoral fornecida pela tomografia computadorizada e ressonância magnética desempenha papel importante no planejamento cirúrgico, influenciando, também, na conduta terapêutica e prognóstico.

OBJECTIVE: To evaluate the role, especially of computed tomography, in the staging of maxillary sinus carcinomas. MATERIALS AND METHODS: Ten cases of carcinoma treated in Hospital Heliópolis Department of Diagnostic Imaging and Head and Neck Surgery, São Paulo, SP, Brazil, in the period between 1988 and 2002, were evaluated. RESULTS: Nine patients presented with tumor extension to the cheek, eight to the masticator space, seven to the mouth floor and hard palate, five to the pterygoid fossa, five to the orbit, three to the ethmoid bone, and one to the skull base. Three of the patients were staged T3, and seven T4. Two patients had lymph nodes metastases at their initial presentation, and were staged T4. All of the cases were histopathologically confirmed. CONCLUSION: The accurate analysis of the tumor local extent and dissemination by means of computed tomography and magnetic resonance imaging plays a relevant role in the surgical planning, besides influencing the therapeutic conduct and prognosis.

Osteogenic sarcoma after orbital radiation rhabdomyosarcoma.

PURPOSE: We describe the occurrence of maxillary and orbital osteogenic sarcoma in a child after treatment of contralateral orbital rhabdomyosarcoma with external beam radiation and chemotherapy. DESIGN: Interventional case report. INTERVENTION: Treatment of a maxillary and orbital rhabdomyosarcoma with surgical resection, systemic chemotherapy, and local radiation. MAIN OUTCOME MEASURES: Occurrence and histology of secondary malignancy after orbital radiation. RESULTS: An eleven year-old male presented for evaluation of left facial swelling, occurring ten years after successful treatment of a right orbital embryonal rhabdomyosarcoma with chemotherapy and 5040 cGY of external beam radiation. Computerized tomography demonstrated a mass involving the left maxillary sinus and orbital floor. Biopsy demonstrated osteogenic sarcoma. Despite attempted total excision with radical maxillectomy, resection margins were found to have microscopic extension of the tumor. Postoperatively he was treated with systemic chemotherapy and local radiation. Eight months postoperatively he remains alive despite local progression. CONCLUSIONS: Osteogenic sarcoma can occur as a secondary malignancy years after the successful treatment of orbital rhabdomyosarcoma with external beam radiation and chemotherapy. After orbital radiation, subjects should undergo routine lifelong examinations.

Factors in the pathogenesis of tumors of the sphenoid and maxillary sinuses: a comparative study.

OBJECTIVES/HYPOTHESIS: To explain the processes that lead to the development of tumors in the maxillary and sphenoid sinuses. STUDY DESIGN: A 32-year review of the world's literature on neoplasms of these two sinuses and a randomized case-controlled study comparing the normal mucosal architecture of the maxillary to the sphenoid sinus. METHODS: Analysis of a 32-year world literature review reporting series of cases of maxillary and sphenoid sinus tumors. Tumors were classified by histological type and separated into subgroups if an individual incidence rate was reported. Histomorphometry of normal maxillary and sphenoid sinus mucosa was performed in 14 randomly selected patients (10 sphenoid and 4 maxillary specimens). Specimens were fixed in 10% formalin, embedded in paraffin, and stained with periodic acid-Schiff (PAS) and hematoxylin. Histomorphometric analysis was performed with a Zeiss Axioscope light microscope (Carl Zeiss Inc., Thornwood, NY) mounted with a Hamamatsu (Hamamatsu Photonics, Tokyo, Japan) color-chilled 3 charge coupled device digital camera. The images were captured on a 17-inch Sony (Sony Corp., Tokyo, Japan) multiscan monitor and analyzed with a Samba 4000 Image Analysis Program (Samba Corp., Los Angeles, CA). Five random areas were selected from strips of epithelium removed from each sinus, and goblet and basal cell measurements were made at magnifications x 100 and x 400. RESULTS: The literature review revealed that the number and variety of tumors in the maxillary sinus are much greater than those in the sphenoid. The incidence of metastatic lesions to each sinus is approximately equal. No recognized pattern of spread from any particular organ system could be determined. On histomorphometric study there were no statistically significant differences between the sinuses in the concentration of goblet cells, basal cells, or seromucinous glands. CONCLUSIONS: Factors involved in the pathogenesis of tumors of the maxillary and sphenoid sinuses include differences in nasal physiology, embryology, morphology, and topography. There are no significant histological differences in the epithelium and submucous glands between the two sinuses to explain the dissimilar formation of neoplasms.

Fibrosarcoma del seno maxilar izquierdo / Fibrosarcoma of the left maxilary sinus

Se presenta el caso de una paciente de 80 años con un fibrosarcoma del seno maxilar, se destacan sus características clínicas, diagnósticas y terapéuticas, asimismo reseñamos algunos aspectos de frecuencia, etiología e histopatología. Se destaca la importancia del diagnóstico precoz en esta enfermedad de comienzo lento, pero con diagnósticos diferenciales comúnes(AU)

Malignant neoplasms of the maxillary antrum in Nigerians.

OBJECTIVE: To highlight the presentation of antral cancers in Nigerians. PATIENTS AND METHODS: Hospital records of all patients with a histologically diagnosed antral cancer who presented at the Lagos University Teaching Hospital (a tertiary referral centre) over a 16 year period were analysed. RESULTS: 28.7% of orofacial cancers and 12.6% of head and neck cancers were located in the antrum. 54.1% were squamous cell carcinoma, 27.4% were minor mucus gland carcinoma and 14.8% were mesenchymal cancers. Males were more frequently affected (ratio 1.7 to 1) and were relatively younger (peak age = 4th decade) than females (peak age = 6th decade). Males who had squamous cell carcinoma (x = 47.5 +/- 18.8 years) were relatively younger than their female counterparts (x = 57.2 +/- 13.8 years). But females with minor mucus gland carcinoma were relatively younger (x = 34.2 +/- 19.5 years) than males (x = 49.1 +/- 17.9 years). All patients had facial swelling at presentation and the most frequent initial symptom was toothache (36.2%). Fifty-two percent had prior treatment and the most common was an extraction of a molar tooth (50%). Two-thirds of the patients were of low socio-economic status. CONCLUSION: Antral cancer constituted sizeable proportion of orofacial and head and neck cancers in Nigerians. Clinicians need to maintain a high index of suspicion in cases of maxillary molar toothache or mobility of obscure aetiology. Predisposing aetiologic factors of this disease peculiar to our environment need to be further investigated.

Myoepithelial carcinoma (malignant myoepithelioma): first report of an occurrence in the maxillary sinus.

AIM: We document for the first time the occurrence of a malignant myoepithelioma at a site other than within the major and minor salivary glands. METHODS AND RESULTS: A 67-year-old male presented with progressive symptoms and signs of a space-occupying lesion in the right maxillary sinus. An initial biopsy identified a malignant (myo)epithelial lesion and a radical maxillectomy was performed. Histology, supplemented by immunohistochemistry, confirmed the presence of a malignant myoepithelioma. CONCLUSION: Malignant myoepithelioma is a very rare tumour composed almost exclusively of myoepithelial cells and, to date, has only been described arising in the the major and minor salivary glands. A variety of tumours of salivary tissue have been reported within the head and neck area at sites outside the major and minor salivary glands, probably arising within accessory salivary tissue. We report the first case of a malignant myoepithelioma occurring in the maxillary sinus, also presumably arising in accessory salivary tissue in this location.

Radiation-induced malignant fibrous histiocytoma in patients with nasopharyngeal carcinoma.

OBJECTIVE: To evaluate the prevalence, 15-year cumulative incidence, time interval, and prognosis of radiation-induced malignant fibrous histiocytoma of the head and neck in long-term survivors of nasopharyngeal carcinoma. DESIGN: Cohort. SETTING: Tertiary care hospital. PATIENTS: Eight long-term survivors of nasopharyngeal carcinoma with malignant fibrous histiocytoma in the maxillary sinus or nasal cavity. MAIN OUTCOME MEASUREMENT: Survival of postirradiation malignant fibrous histiocytoma in patients with nasopharyngeal carcinoma. RESULTS: The prevalence of radiation-induced malignant fibrous histiocytoma in long-term survivors of nasopharyngeal carcinoma was 0.38%. The 15-year cumulative incidence was 2.2%. Most tumors occurred in the maxillary sinus and were characterized by spindle-shaped tumor cells with plump nuclei arranged in a whorl or storiform pattern in a fibrous stroma. The mean interval between malignant fibrous histiocytoma and nasopharyngeal carcinoma was 121 months. Local recurrence developed in all cases within 9 months after surgery. Six patients died of disease without distant metastasis within 30 months. Two patients were alive with disease for 20 and 32 months, respectively. CONCLUSIONS: Radiation-induced malignant fibrous histiocytoma in the head and neck region in long-term survivors of nasopharyngeal carcinoma is rare. It takes a long time to occur after irradiation and is locally invasive with poor prognosis.

Non-Hodgkin's lymphoma of the head and neck in association with HIV infection.

People infected with HIV have a greater risk of developing malignancies. The most frequent one is the Kaposi's sarcoma (KS). The second is the Non-Hodgkin's lymphoma (NHL). We report six cases of NHL in association with HIV infection (NHL-HIV) of the head and neck. Three of them had extranodal location (2 oral lymphomas and one case affecting the maxillary sinus). The other 3 cases were located in the cervical nodes. The oral malignancies were treated with radiotherapy (RT). Chemotherapy was applied to the other 4. Only one of our patients has survived more than two years. In one case the NHL was the first manifestation of the infection with HIV. Diagnosis of extranodal NHL requires search for AIDS.

[Postoperative maxillary cyst]. / Pooperacyjna torbiel zatoki szczekowej.

We presented the case report of a postoperative maxillary cyst, which developed 26 years after the radical Caldwel-Luc procedure in the maxillary sinus. This is a rare case in nonoriental population but it has been described well in Japanese literature. Considering the size and damage found in clinical and radiological examination, we suspected a malignance lesion.

[Carcinoma in a 40-year chronic oro-antral communication]. / Karzinom auf dem Boden einer 40jährigen Mund- Antrumverbindung.

A squamous cell carcinoma developed in a 66 year old patient in the region of a traumatically caused oro-antral fistula that has persisted for 40 years. The clinical course and histological findings are presented. Based on the few case reports in the literature the occurrence of malignancies in chronic traumatic fistulae is discussed. Reference is also made to causal relationships between such a fistula and malignancy under the aspect of forensic medical consultations.

Lymphoma of the maxillary sinus in a patient infected with human immunodeficiency virus type 1.

A 27-year-old woman with a history of intravenous drug abuse presented with a stage IE, diffuse, large cell lymphoma of the right maxillary sinus. A test for antibodies to the human immunodeficiency virus was positive. The patient was treated with systemic chemotherapy and local maxillary sinus irradiation which resulted in complete regression of the disease. Therapy was complicated by mucositis, neutropenia, and opportunistic infections. This is the first case report to discuss the presentation and treatment of acquired immunodeficiency syndrome (AIDS)-related lymphoma of the maxillary sinus.