Local intralesional talimogene laherparepvec therapy with complete local response in oral palatine mucosal melanoma: a case report.

BACKGROUND: Mucosal melanoma, an aggressive type of malignancy different from the cutaneous melanomas commonly seen in the head and neck region, represents < 1% of all malignant melanomas. The pathogenesis of mucosal melanoma is unknown. Targetable mutations commonly seen in cutaneous melanoma, such as in the BRAF and NRAS genes, have a lower incidence in mucosal melanoma. Mucosal melanoma carries a distinct mutational pattern from cutaneous melanoma. Surgery with negative margins is the first-line treatment for mucosal melanoma, and systemic therapy is not well defined. Talimogene laherparepvec, an oncolytic viral immunotherapy, is United States Food and Drug Administration approved for the treatment of advanced malignant cutaneous melanoma, with local therapeutic benefits. Mucosal melanoma was initially excluded from talimogene laherparepvec's initial phase III clinical trial. CASE PRESENTATION: We present the case of a white female patient in her 40s with past medical history of systemic lupus erythematous, scleroderma, and estrogen-receptor-positive invasive ductal breast carcinoma. Following a bilateral mastectomy, the patient was found to have BRAF-negative mucosal melanoma of her hard palate with a soft palate skip lesion. Owing to the presence of a skip mucosal lesion as well as the anticipated defect and need for free-flap reconstructive surgery, nonsurgical management was considered. The patient was referred to medical oncology, where-based on the patient's complicated medical history and the risk of immunotherapy possibly worsening her prior autoimmune diseases-local talimogene laherparepvec injections were chosen as the primary therapy for her mucosal lesions. Though talimogene laherparepvec is approved for the treatment of cutaneous melanoma, there are limited data available on the use of talimogene laherparepvec in mucosal melanomas. CONCLUSION: The patient had a complete local tumor response at both the primary lesion as well as the skip lesion with the local injections. She had no side effects and maintained a high quality of life during treatment.

Recurrent pterygo-palatal angiofibroma with intracranial extension: case report.

We report the case of a patient with recurrent pterygo-palatal angiofibroma and its treatment. A 21-year-old male patient had a long history of recurrent epistaxis with progressive nasal obstruction. He was diagnosed with an angiofibroma centered in the right pterygo-palatine fossa. Initially, he underwent surgical excision with removal of the entire tumor. The evolution was clinically good with no signs of recurrence on the cervico-facial scan of control (CT). Nine months after, he presented a reappearance of epistaxis. A cervico-facial MRI was performed and showed a recurrence of the tumor process, which this time was considered inextirpable, hence the decision to opt for radiotherapy with intensity modulated radiation therapy (IMRT). He has improved clinically with a clear reduction in tumor mass on CT scan. This technique represents an interesting alternative to overcome anatomical complexity of the region, cover the tumor and preserve the organs at risk.

Adenoid cystic carcinoma of head and neck. A 5-year retrospective study: Experience in a single third-level reference center.

INTRODUCTION: Adenoid cystic carcinoma (ACC) is a rare tumor, with an annual incidence of 3-4.5 cases/million. MATERIALS AND METHODS: We conducted a descriptive and retrospective study. We included patients diagnosed with ACC of head and neck of a single third-level referral center, from January 2008 to December 2013. RESULTS: A total of 23 patients were included in the study, 10 males (43.5%) and 13 females (56.5%) (F:M ratio 1.3:1). Median age was 52 years. Localization was predominantly observed in the oral cavity (26%) followed by the palate (17.4%). At presentation, 15 patients (65.2%) had clinical Stage II. The most frequent histology was the cribriform type in 10 patients (43.5%). Following resection, positive margins were observed in 15 (65.2%), neural invasion in 8 (34.8%), and lymphovascular invasion in 2 (8.7%) patients. The median time to recurrence in our population was 23 months. Recurrence at 5 years was observed in 15 patients (65%). At 2 years, recurrence was predominantly local in 7 patients (46%); whereas after 2 years, it was predominantly pulmonary 6 patients (40%). Overall, 5 year-survival was 78%. CONCLUSIONS: Cystic adenoid carcinoma is characterized by a high recurrence rate. Nevertheless, it has a high overall 5-year survival, which justifies an aggressive treatment.

ANTECEDENTES: El carcinoma adenoide quístico es un tumor raro, con una incidencia anual de 3-4.5 casos por millón. MATERIALES Y MÉTODOS: Estudio descriptivo, retrospectivo, que incluyó pacientes con carcinoma adenoide quístico de cabeza y cuello de un centro de referencia de tercer nivel, desde enero de 2008 hasta diciembre de 2013. RESULTADOS: Se incluyeron 23 pacientes, 10 hombres (43.5%) y 13 mujeres (56.5%) (relación 1.3:1), con una media de edad de 52 años. Predominó en la cavidad bucal (26%), seguida del paladar (17.4%). Al diagnóstico, 15 pacientes (65.2%) tenían estadio clínico II. La histología cribiforme fue la más frecuente (10 pacientes; 43.5%). Posterior a la cirugía, se observaron márgenes positivos en 15 (65.2%), invasión neural en 8 (34.8%) e invasión linfovascular en 2 (8.7%) pacientes. La media de recurrencia fue de 23 meses. La recurrencia a 5 años se observó en 15 pacientes (65%). A los 2 años predominó la recurrencia local (7 pacientes; 46%); después de los 2 años predominaron las metástasis pulmonares (6 pacientes; 40%). La supervivencia a 5 años fue del 78%. CONCLUSIONES: El carcinoma adenoide quístico se caracteriza por una alta tasa de recurrencia. Sin embargo, tiene una alta supervivencia global a 5 años, lo que justifica un tratamiento agresivo.

Prognostic factors in patients with soft palate squamous cell carcinoma.

BACKGROUND: To define the prognostic factors associated with outcome in patients with soft palate squamous cell carcinoma (SCC). METHODS: Previously untreated patients with soft palate and uvula SCC treated in our institution between 1997 and 2012 were collected. The prognostic value of clinical, hematological, and treatment characteristics was examined. RESULTS: We identified 156 patients, median age 58 years, with 71% drinkers, 91% smokers; 19% had synchronous cancer. Front-line treatment was chemoradiotherapy in 58 (37%), radiotherapy alone in 60 (39%), surgery in 17 (11%), and induction chemotherapy in 21 patients (14%). The 5-year actuarial overall survival (OS) and progression-free survival (PFS) were 41% and 37%, respectively. In univariate analysis, T3-T4 vs T1-T2 stage, N2-N3 vs N0-N1 stage, and neutrophil count >7 g/L were associated with worse OS and PFS (P < .05). CONCLUSION: In patients with soft palate SCC, inflammation biomarkers were associated with OS.

Palatal ancient schwannoma: optical, immunohistochemical and ultrastructural study with literature review.

Schwannoma or neurilemmoma is a benign encapsulated slow-growing tumor that originates from a Schwann cell of a nerve, and is rare at intraoral locations. Different histological variants of schwannomas have been described, of these degenerative or ancient schwannoma is probably one of the least common in the oral cavity with only 16 previously reported cases, of which only one has been described in palatal location. Although ancient schwannoma shows particular characteristics, it is difficult to diagnose based on clinical and imaging appearance alone; as a result, morphological examination assisted by ancillary techniques is necessary to establish a definite diagnosis. We present a clinicopathological description of this rare variant of schwannoma, located in an unusual intraoral site, of a 26-year-old female. We illustrate the optical, immunohistochemical and ultrastructural characterization that aid diagnosis, as well as providing a review of the relevant published data of this unusual tumor.

Delayed Diagnosis of a Nasal Type Lymphoma Misdiagnosed as Persistent Sinusitis.

Nasal Type T/natural killer (NK)-cell lymphomas are rare clinical entities, highly aggressive with a very poor prognosis. We present a case of a 37-year-old immunocompetent man presenting with deep palatal ulceration and a 3-month history of symptoms, which appear to have been misdiagnosed by physicians. The final diagnosis was achieved by a 15-day diagnostic algorithm, during which time the clinical status of the patient worsened severely. In this article, we also provide a succinct update on the clinical and histopathological findings of Peripheral T/NK-cell lymphomas and propose that symptoms that are consistent with these clinical entities should be considered from the early stages to inform a suitable diagnostic pathway Because of their highly aggressive behavior, we suggest that early therapy of T/NK-cell lymphomas may be started before completing the specific diagnostic investigations.

Management of the clinically N0 neck in squamous cell carcinoma of the maxillary alveolus and hard palate.

BACKGROUND: The purpose of this study was to evaluate active surveillance strategy in the clinically negative neck in maxillary squamous cell carcinoma (SCC). METHODS: One hundred fourteen consecutive patients diagnosed with oral maxillary SCC were analyzed retrospectively from 3 centers in The Netherlands. Analysis parameters included regional disease-free survival of N0 patients stratified for T classification, elective radiotherapy (RT) of the neck; and overall survival of the whole cohort, stratified by N classification; salvage neck surgery rates. RESULTS: Within the N0 cohort, 26.0% of the patients developed neck metastasis in the follow-up visits. Regional recurrence was not related to T classification or postoperative RT of the neck. Regional and locoregional recurrence were associated with diminished overall survival (p < .05). Regional metastasis was operable in 22 of 26 cases (85%). Only 1 patient presented with inoperable neck metastasis without local recurrence. CONCLUSION: Watchful waiting was feasible in this cohort. If meticulous follow-up is not available, elective neck dissection is recommended. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1794-1798, 2016.

Cervical metastases from squamous cell carcinoma of hard palate and maxillary alveolus: a retrospective study of 10 years.

BACKGROUND: The purpose of this study was to investigate the incidence of cervical metastasis in squamous cell carcinoma (SCC) of hard palate and maxillary alveolus and to define its impact factors. METHODS: We conducted a retrospective study of patients surgically treated for SCC of hard palate and maxillary alveolus from 2002 to 2011. In situ hybridization was performed to detect high-risk human papillomavirus (HPV) infection. RESULTS: The incidences of cervical metastasis and occult metastasis were 17.2% (11/64) and 9.8% (5/51), respectively. The pT classification and vascular invasion were correlated with cervical metastasis. Occult metastatic risk was significantly higher among patients with pT4. Presence of positive nodes impaired prognosis significantly. CONCLUSION: SCC of hard palate and maxillary alveolus has nonnegligible incidences of both overall and occult metastasis, which were highly associated with pT classification. We recommend routine, synchronous elective neck dissection for T4 lesions, whereas observation is an alternative for T1 to T3 lesions.

Mammary analog secretory carcinoma of salivary gland with high-grade histology arising in hard palate, report of a case and review of literature.

Mammary gland analog secretary carcinoma (MASC) of salivary gland is typically a tumor of low histologic grade and behaves as a low-grade malignancy with relatively benign course. This tumor shares histologic features, immunohistochemical profile, and a highly specific genetic translocation, ETV6-NTRK3, with secretory carcinoma of breast. Histologically, it is often mistaken as acinic cell carcinoma, adenocarcinoma not otherwise specified, and other primary salivary gland tumors. Here we report a case of MASC with high-grade transformation and cervical lymph node metastases confirmed with ETV6-NTRK3 translocation arising in the hard palate of a 41 year-old adult. Interestingly, the metastatic carcinoma has lower grade than the original tumor which strongly support malignant transformation of the original tumor. Most commonly, MASC arises from the parotid gland and less often in minor salivary glands. Metastasis is relatively uncommon and high-grade histology has only been reported in four cases with three of them arising from the parotid gland and the location of the fourth one has not been reported. This is the first case with high grade histology that arise from minor salivary gland and it emphasizes the importance of molecular screening of salivary gland tumor with high-grade histology for ETV6-NTRK3 translocation. In our literature of 115 cases that includes the current case, MASC occurred predominantly in adult with only a few cases under 18 years of age and a male to female ratio of 1.2:1. Parotid gland is more commonly affected but there is also significant occurrence in minor salivary glands. Except for the cases with high grade histology, the overall prognosis is good.

Leiomyosarcoma of the maxilla in a 24-year-old man who initially presented with odontalgia, and suffered from tumour mismanagement.

Leiomyosarcoma is an uncommon malignant soft tissue tumour originating from smooth muscle cells. It generally occurs in the female genital tract, especially in the uterus, gastrointestinal tract, retroperitoneum and rarely in the head and neck. Here we report a case of leiomyosarcoma in a 24-year-old man with a 6-month history of toothache and extraction of three right upper molar teeth, that was complicated by fistula tract formation and facial swelling. Imaging studies demonstrated tumoural lesions involving the right maxillary sinus, nasal cavity, hard palate and orbit. The histopathological and immunohistochemical studies confirmed that it was leiomyosarcoma.

[Follicular dendritic cell sarcoma of soft plate: one case report].

Follicular dendritic cell sarcoma is a rare low-grade malignant tumor. At present, only twenty ca ses was discovered all over the world. This paper reports a case treated in our hospital, explores the clinical manifestations, pathological diagnosis and treatment to provide certain help to clinical doctor in diagnosis and treatment to reduce the misdiagnosis of the disease.

Adenoid cystic carcinoma of hard palate with coincidental metastases to lung and liver.

Adenoid cystic carcinoma is the most frequent pathology occurring in the minor salivary glands .It is usually slow growing; however, it can spread via perineural invasion, haematogenous and lymphatogenous metastasis. Most common sites of metastasis are lung and bone. Involvement of the other sites is not common. In this article, we present a woman with coincident lung and liver metastases.

A matched cohort comparison of mTHPC-mediated photodynamic therapy and trans-oral surgery of early stage oral cavity squamous cell cancer.

Photodynamic therapy (PDT) of early stage oral cavity tumors have been thoroughly reported. However, statistical comparison of PDT to the surgical treatment is not available in published literature. We have identified and matched cohorts of patients with early stage oral cavity cancers undergoing surgery (n = 43) and PDT (n = 55) from a single institute experience. The groups are matched demographically and had the same pre-treatment screening and follow-up schedule. Both groups consisted only of tumors thinner than 5 mm to ensure comparability. The endpoints were local disease free survival, disease free survival, overall survival and response to initial treatment. Local disease free survival at 5 years were 67 and 74 % for PDT and surgery groups, respectively [univariate HR = 1.9 (p = 0.26), multivariable HR = 2.7 (p = 0.13)]. Disease free survival at 5 years are 47 and 53 % for PDT and surgery groups, respectively [univariate HR = 0.8 (p = 0.52), multivariable HR = 0.75 (p = 0.45)]. Overall survival was 83 and 75 % for PDT and surgery groups, respectively [(univariate HR = 0.5 (p = 0.19), multivariable HR = 0.5 (p = 0.17)]. In the PDT group, six patients (11 %) and in the surgery group 11 patients (26 %) had to receive additional treatments after the initial. All of the tested parameters did not have statistical significant difference. Although there is probably a selection bias due to the non-randomized design, this study shows that PDT of early stage oral cavity cancer is comparable in terms of disease control and survival to trans-oral resection and can be offered as an alternative to surgical treatment.

Oral pigmentation: case report and review of malignant melanoma with flow charts for diagnosis and treatment.

The color of the human oral mucosa is not uniform. Chromatic variations can occur, depending on the degree of keratinization, melanogenic activity, number of melanocytes, vascularization, and type of submucosal tissue. If a pathological pigmentation is suspected, its benign or malignant nature will guide the treatment plan. This article presents a rare case of malignant melanoma of the anterior maxilla with asymptomatic palatal pigmentation and regional lymph node metastasis. Flow charts for the diagnosis and treatment of pigmented lesions (with special emphasis on treatment of primary and metastatic melanoma) are included.

Long-term treatment outcome of minor salivary gland carcinoma of the hard palate.

Minor salivary gland carcinoma of the hard palate is rare, and its long-term survival rate is high, making it difficult to evaluate the prognostic factors and the efficacy of treatment. This study was designed to evaluate the treatment outcome of minor salivary gland carcinoma of the hard palate. 103 cases of minor salivary gland carcinoma of the hard palate treated with surgery alone or underwent surgery combined with post-operative radiotherapy hospitalized in Cancer Center, Sun Yet-Sen University, from 1968 to 2008 were reviewed retrospectively. The most common histologic types were adenoid cystic carcinoma in 48 patients(46.6%), mucoepidermoid carcinoma in 37(35.92%), malignant mixed tumor in 15(14.56%), and acinic cell carcinoma in 3(2.91%). The median follow-up time was 74.83 months (range 0.9-356.57 months). Overall outcomes at 5 and 10 years were overall survival (OS), 77.9% and 65.7%; recurrence-free survival (RFS), 64.4% and 53.2%; and disease specific survival (DSS), 77.9% and 67.7%, respectively. There was no significant difference in overall survival (P=0.52), recurrence-free survival (P=0.762) and disease specific survival (P=0.449) between patients who underwent surgery alone and those who underwent surgery plus post-operative radiotherapy. Surgery has been accepted as the primary treatment for minor salivary gland carcinoma of hard palate. Sufficient surgical excision with adequate margins is essential for a favorable outcome. We advocate using radiotherapy in the post-operative context for patients with poorly differentiated, cervical lymph node metastasis, positive or close margins, and large primary lesions.

T cell non-Hodgkin's lymphoma with colesional mucormycosis presenting as palatal perforation: a case report.

Non-Hodgkin's lymphoma (NHL) is predominantly a disease of lymph nodes, but extranodal involvement is not very uncommon. Palatal involvement by NHL is rare. Mucormycosis is a devastating fungal infection commonly seen in immunocompromised individuals, including those with NHL, but it is affecting the same region has been reported very rarely. Simultaneous infiltration of hard palate by NHL and mucormycosis is extremely unusual. Herein we describe a patient who presented with palatal hole with histopathological examination revealing presence of lymphoma with colesional mucormycosis. The identification of mucor was vital because chemotherapy alone in the absence of antifungals would have had devastating consequences as the mortality of untreated mucormycosis is high.

Primary oral mucosal melanoma: advocate a wait-and-see policy in the clinically N0 patient.

PURPOSE: Oral mucosal melanoma (OMM) is a rare disease associated with a very poor prognosis. Because well-established treatment protocols for OMM are in short supply, prognostic information regarding recent treatment modalities for this disease were sought. PATIENTS AND METHODS: A retrospective chart review was performed of 61 patients who were treated for OMM from 1998 through 2005. The clinical features and treatment modalities were identified and correlated with the outcomes. RESULTS: There were 41 male and 20 female patients (ratio, 2.1:1) with a mean age of 54.1 years. The mean follow-up was 31.9 months, and the overall 2-year and 5-year survival rates were 51.1% and 30.3%, respectively. According to the seventh edition of the American Joint Committee on Cancer staging system, there were 31 patients (50.8%) with stage III tumors. A more advanced stage and a tumor of at least 2 cm were associated with worse survival (P < .001 and P = .036, respectively). Elective lymph node dissection and biochemotherapy were not associated with a higher total survival rate (P = .53 and P = .76, respectively). CONCLUSIONS: OMM has a male predilection. The seventh edition of the American Joint Committee on Cancer stage and tumor size are effective prognostic parameters for patients with OMM. The American Joint Committee on Cancer staging system provides useful information for predicting the ultimate outcome and should be used as the primary staging system. Elective node dissection and adjuvant biochemotherapy offer no additional advantage in increasing the patient survival rate. A wait-and-see policy is advocated for patients with clinical stage N0 cancer.

Primary myoepithelial carcinoma of palate.

OBJECTIVES: The aim of this study was to present a rare neoplasm, Primary myoepithelial carcinoma arising from the palate, and to review its diagnostic criteria, pathologic and clinical characteristics, treatment options and prognosis. CLINICAL PRESENTATION AND INTERVENTION: Myoepitheliomas are tumors arising from myoepithelial cells mainly or exclusively. Myoepitheliomas mostly occur in salivary glands, as well as in breast, skin, and lung. Case of myoepitheliomas in palate has rarely been reported. Myoepithelial carcinoma is malignant counterpart of myoepitheliomas. Adenomyoepithelioma is also a different disease from myoepitheliaomas. Immunohistochemically, tumor cells of myoepithelial carcinoma express not only epithelial markers such as cytokeratin, epithelial membrane antigen (EMA), but also markers of smooth muscle origin such as calponin. The immunohistochemical criteria of myoepithelial differentiation are double positive for both cytokeratins and one or more myoepithelial immunomarkers (i.e., S-100 protein, calponin, p63, GFAP, maspin, and actins). Myoepithelial carcinomas of salivary and breast demonstrate copy number gains and gene deletion. The overall prognosis of myoepithelial carcinoma is poor. There is rarely recurrence or metastasis in benign myoepithelial tumors. Complete excision with tumor-free margin is always the preferred treatment, while local radiation therapy and chemotherapy are suggestive treatment options. Here, a rare case of myoepithelial carcinoma arising from the palate has been described and discussed for the treatment and outcome. Pathological and clinical characters of myoepitheliomas are also compared and discussed. CONCLUSION: The case report serves to increase awareness and improve the index of diagnosis and treatment of myoepitheliomas.