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.

Application of prolonged submental perforator flap to repair the postoperative defect of upper airway malignancy.

OBJECTIVES: To explore the feasibility of making a submental perforator flap distal to the connecting line between the mastoid and the sternoclavicular joint under the guidance of neck-enhanced CT and repairing the postoperative defect of upper airway malignancy. MATERIALS AND METHODS: This study retrospectively analysed 19 cases of upper airway malignant tumours treated in our department from January 2021 to September 2022, including 17 males and 2 females, aged 43-70 years. SITE OF LESIONS: 15 cases were in the laryngopharynx, 2 cases in the nasal cavity and paranasal sinus and 2 cases on the soft palate. All the lesions were malignant and at stages T2-4N0-2M0. SURGICAL METHOD: The extended submental perforator flap (size 22-15 × 6-7 cm) was prefabricated distal to the connecting line between the mastoid and the sternoclavicular joint. After tumour resection, the flap was used to repair the postoperative defect. Fifteen cases of laryngopharyngeal malignant tumours were repaired using the extended submental perforator flap with the vascular pedicle located on the opposite side of the tumour body. Two cases of nasal cavity and paranasal sinus tumours were repaired using the extended submental perforator flap combined with the temporalis muscle flap. The soft palate was completely removed in two patients with soft palate cancer and repaired using the folded extended submental perforator flap. RESULTS: Before the surgery, the reflux vein was observed by neck-enhanced CT, including 12 cases returning to the internal jugular vein and 7 cases to the external jugular vein. All 19 cases in which flaps were used survived, and 1 case had a postoperative infection. All the patients had nasal feeding removed after surgery. The tracheal cannula was removed from the patients with laryngeal preservation, and the pronunciation was satisfactory. Among them, patients with soft palate cancer repair had mild nasal reflux symptoms with smooth breathing. During the follow-up period of 4-24 months, 18 patients had no tumour recurrence or metastasis, and 1 patient had cervical lymph node metastasis. CONCLUSIONS: This study highlights the use of a submental perforator flap distal to the connecting line between the mastoid and the sternoclavicular joint to repair postoperative defects for upper airway malignancy as an innovative surgical approach that provides more tissue and good arteriovenous blood supply to adjacent sites. This method has high clinical value and provides an effective option for repairing postoperative defects of upper airway malignancy.

Clinical diagnostic approach for oral lymphomas: a multi-institutional, observational study based on 107 cases.

OBJECTIVE: To evaluate oral lymphomas' clinical manifestations and investigate whether clinical features are associated with lymphoma subtypes. STUDY DESIGN: Oral lymphomas with at least 1 representative clinical image were evaluated. They were classified according to their microscopic grade (high vs low), predominant cell size (small vs medium/large), and cellular lineage (B cell vs T cell). Clinical images were described according to tumor location, number, swelling, ulcer, necrosis, telangiectasia, predominant color, and lobulation. Lymphomas affecting the palate were compared with salivary gland tumors (SGTs) affecting this location. RESULTS: Data from 107 cases were included. High-grade subtypes (80.4%), with medium/large-sized cells (52.3%), and diagnosed as diffuse large B cell lymphomas (29%) predominated. High-grade lymphomas often presented as painful, ulcerative, and osteolytic diseases (P < .05). Tumors predominantly composed of medium/large-sized cells were associated with painful lesions, ulcerated, with necrosis and bone destruction (P < .05). When only palate tumors were considered, multiple and bilateral lesions, the presence of pain, ulceration, and necrosis were significantly more associated with a diagnosis of lymphoma than SGT (P < .001). CONCLUSION: High-grade oral lymphomas are more associated with destructive presentation than low-grade subtypes, and bilateral lesions in the palate are more associated with a lymphoma diagnosis than SGT.

Oral-visceral iatrogenic Kaposi sarcoma following treatment for acute lymphoblastic leukemia: a case report and review of the literature.

BACKGROUND: There have hardly been any reported cases of children presenting with Kaposi sarcoma as a second malignancy following treatment for acute lymphoblastic leukemia outside a transplant setting. CASE PRESENTATION: We report a case of a 5-year-old boy of Bantu origin, which, to our knowledge, could be only the second reported case of oral-visceral Kaposi sarcoma after acute lymphoblastic leukemia treatment. The patient presented with a 1-month history of progressive, non-painful, soft tissue oral mass, 1 month after completing treatment for high-risk acute lymphoblastic leukemia. He was successfully treated for Kaposi sarcoma on a two-drug regimen (bleomycin and vincristine) with good clinical response. CONCLUSION: Visceral Kaposi sarcoma as a second malignancy may occur after pediatric acute lymphoblastic leukemia treatment, but its rarity makes it unlikely to raise suspicion among clinicians, thus precluding early diagnosis and treatment. We recommend routine evaluation for Kaposi sarcoma lesions in children undergoing long-term surveillance following treatment for childhood acute leukemia.

Oncocytic Polyp in a Unicystic Mucoepidermoid Carcinoma of the Palate: A Unique Presentation.

We report a palatal mucoepidermoid carcinoma (MEC) with unusual pathological features showing salivary duct cyst-like architecture, varied epithelial linings (excretory duct, mucous goblet cell, and apocrine epithelium phenotypes), scarce luminal tumor plaques, and a large intraluminal oncocytic polyp. To our knowledge, such a unicystic variant of MEC with pedicled oncocytic proliferation in the lumen is unprecedented in the literature. In this particular case, the microscopic diagnosis of MEC is problematic because of the large number of potential mimics.

Impact on patients with oral squamous cell carcinoma in different anatomical subsites: a single-center study in Taiwan.

The incidence of oral cavity squamous cell carcinoma (OSCC) is particularly high in South Asia. According to the National Comprehensive Cancer Network, OSCC can arise in several subsites. We investigated survival rates and the clinical and pathological characteristics of OSCC in different anatomical subsites in the Taiwanese population. We retrospectively analyzed data for 3010 patients with OSCC treated at the Changhua Christian Hospital. Subsequently, we compared clinical and pathological features of OSCC in different subsites. Pathological T4 stage OSCCs occurred in the alveolar ridge and retromolar trigone in 56.4% and 43.7% of cases, respectively. More than 25% of patients with tongue OSCC and 23.4% of those with retromolar OSCC had lymph node metastasis. The prognosis was worst for hard palate OSCC (hazard ratio 1.848; p < 0.001) and alveolar ridge OSCC (hazard ratio 1.220; p = 0.017). Retromolar OSCC recurred most often and tongue OSCC second most often. The risk for cancer-related mortality was highest for hard palate OSCC, followed by alveolar ridge and retromolar OSCC. We found distinct differences in survival among the different subsites of OSCC. Our findings may also help prompt future investigations of OSCC in different subsites in Taiwanese patients.

Large irritation fibroma of hard palate: a case report of a rare clinical entity.

Fibromas are benign tumors of connective tissue common in the oral cavity but rare on hard palate. This paper reports on an asymptomatic, slowly growing mass on the hard palate of a 90-year-old lady, with a reported use of denture for two decades. The patient presented with a 2.2cm, smooth-surfaced, well-circumscribed nodule attached with a stalk to the palatal mucosa. After excision, the histopathological examination revealed a mass of fibrous connective tissue, covered by stratified squamous epithelium with focal low-medium grade hyperplasia and hyperkeratosis. These findings were consistent with irritation fibroma of hard palate, a rare entity, which should be considered as a possible diagnosis for tumors of the area by every physician.

Pleomorphic adenoma of hard palate: a case report.

Pleomorphic adenoma is a benign mixed tumor, which is composed of myoepithelial and epithelial cells. A fibrous capsule separates these cells from the surrounding tissues. Pleomorphic adenoma is the most common salivary gland tumour accounting for 40-70% of all major and minor salivary gland tumours. It is also the commonest minor salivary gland benign tumours accounting for 70% of all tumours. Hard palate is the commonest site followed by upper lip, buccal mucosa, tongue, floor of mouth, retromolar trigone. This case report discusses a case of pleomorphic adenoma of hard palate in an old man after complete excision of the tumour, which was confirmed by a biopsy specimen.

Maxillary Sinus Myxofibrosarcoma Mimicking Nodular Fasciitis: A Rare Case Report.

Myxofibrosarcoma (MFS) is a fibroblastic soft tissue sarcoma that is extremely rare in the maxillofacial region. Due to its non-specific clinicoradiographic findings and challenging histopathological features, the diagnosis is difficult. Here, we present a case of MFS which was first diagnosed as nodular fasciitis. The initial examination of the incisional biopsy showed a benign-appearing proliferation of fibroblasts without features of malignancy. The patient returned with recurrence four months after surgical excision of the primary lesion. The second histologic study revealed a high-grade spindle cell sarcoma with myxoid features most compatible with MFS. Definitive diagnosis of MFS was confirmed by these histopathologic features and supportive immunohistochemical stains. Unfortunately, the patient died of disease 3 months later.

Osteosarcoma of the hard palate in a dog - case report / [Osteossarcoma em palato duro de cão - relato de caso]

The osteosarcoma (OSA) is the most diagnosed primary bone cancer in canine patients. This work reports a case of a canine, six years old, mongrel, female, intact, with an OSA in the hard palate. Physical examination detected a firm mass in the palate. Thoracic radiographs, hematological and biochemical exams, histopathological exams and computed tomography were requested. A chondroblastic OSA was diagnosed and the tumor was characterized by immunohistochemistry. There was never evidence of metastasis in this case. The treatment consisted of the combination of conventional chemotherapy, metronomic chemotherapy, and palliative care, aiming at greater survival and well-being of the patient since surgical excision was not possible due to the location and extension of the tumor. Osteogenic sarcomas of the hard palate are rarely seen and described in the literature. In this article we present a characterization of the osteosarcoma with uncommon localization in the hard palate.(AU)

O osteossarcoma (OSA) é a neoplasia óssea primária mais diagnosticada em pacientes caninos. Este trabalho relata o caso de um canino, com seis anos de idade, sem raça definida, fêmea, não castrado, apresentando OSA em região de palato duro. Ao exame clínico, constatou-se uma massa de consistência firme em região palatina. Além do exame clínico, foram solicitadas radiografias torácicas, exames hematológicos e bioquímicos, exames histopatológicos e tomografia computadorizada. Fora então dado o diagnóstico de OSA condroblástico, e o tumor foi caracterizado pela imuno-histoquímica. Não foram observadas evidências de metástases nesse caso. O tratamento instituído consistiu na combinação de quimioterapia convencional, quimioterapia metronômica e cuidados paliativos, almejando maior sobrevida e bem-estar do paciente, uma vez que a excisão cirúrgica não foi possível devido à localização e à extensão do tumor. Neste artigo, apresentou-se uma caracterização do osteossarcoma com localização incomum no palato duro.(AU)

Palatal Soft Tissue Myxoma in a Patient with Carney Complex.

Carney complex (CNC) is a rare, autosomal dominant multiple neoplasia syndrome. Although cutaneous myxomas commonly occur in CNC patients, intraoral myxomas are extremely rare. We present a case of a palatal myxoma in a 21-year-old female patient with CNC, along with a review of the pertinent literature. She presented with a sessile nodule on the hard palate that microscopically showed a multilobulated and highly vascularized myxomatous tissue composed of loosely-arranged spindle, polygonal, and stellate cells, suggestive of myxoid neurofibroma. Six years after the oral lesion was removed, she presented with a growth hormone (GH)-producing pituitary adenoma, a cardiac myxoma, two cutaneous myxomas on the lower abdomen area, and one myxoma in the vaginal mucosa. Therefore, the final diagnosis of the palatal lesion was of a soft tissue myxoma related to CNC. The patient remains on close follow-up, with no recurrences of the palatal myxoma after 7 years.

Delayed Infection of Porous Polyethylene Implants After Oncologic Maxillectomy and Reconstruction: 2 Case Reports and Review of Literature.

Medpor porous polyethylene implants are commonly used for facial skeletal reconstruction due to reported biocompatibility, fibrovascularization, and durability. While uncommon, late implant infections are an important consideration. We report delayed infections in 2 patients after unilateral total oncologic maxillectomy and reconstruction using Medpor implants for an ossifying fibroma and squamous cell carcinoma, respectively. In the first patient, annual interval computed tomography (CT) scans showed no recurrence of tumor or inflammatory changes. The second was lost to follow-up after adjuvant chemoradiation 1 year after resection. Patients both presented with swelling, drainage, and erythema around the implant at a mean of 4.5 years following maxillectomy. Both failed several attempts at conservative treatment. Cultures of implants removed at a mean of 2.5 months after infection grew α-hemolytic Streptococcus in the first and multiple organisms in the second, showing that the potential for delayed infection should be considered years after reconstruction.

Tumor pardo del paladar, presentación atípica de hiperparatiroidismo primario en adolescente: buena evolución y normalización de la masa ósea tras paratiroidectomía / Brown tumor of the palate, atypical presentation of primary hyperparathyroidism in an adolescent: good evolution and bone mass normalization after parathyroidectomy

El hiperparatiroidismo primario (HPTP) es poco frecuente en niños y adolescentes. Hay escasos datos para el manejo de estos pacientes en pediatría. Las glándulas paratiroideas son glándulas endocrinas que secretan hormona paratiroidea (PTH) y regulan el metabolismo del calcio y del fósforo. La sobreexpresión de PTH se llama hiperparatiroidismo, que se clasifica en primario, secundario y terciario. En los adolescentes, 80 a 92% de los hiperparatiroidismos primarios se deben a adenoma paratiroideo. Presentamos el caso clínico de una adolescente con una primera manifestación atípica de HPTP, la presencia de un tumor pardo del paladar, presentación rara de adenoma paratiroideo, acompañado de hipercalcemia, marcada elevación de PTH y varias lesiones óseas. (AU)

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Guidelines for management in pediatric patients are limited. Parathyroid glands are endocrine glands that secrete parathyroid hormone (PTH) and regulate calciumphosphate metabolism. The overexpression of PTH is called hyperparathyroidism, and is classified as primary, secondary, and tertiary. In adolescents, 80 to 92% of PHPT cases are due to a parathyroid adenoma. We present here a case report of an adolescent with a brown tumor of the palate as the first manifestation of the disease, atypical and rare presentation of parathyroid adenoma in an adolescent. She had hypercalcemia, marked elevation of PTH and bone lesions. (AU)