Adenoid cystic carcinoma of the palpebral lobe of the lacrimal gland - case report and literature review.

Epithelial tumors of the lacrimal gland are rare and usually develop in the orbital lobe. We report the exceedingly rare occurrence of a primary adenoid cystic carcinoma in the palpebral lobe of the lacrimal gland. A 26-year-old female was referred for evaluation of a gradually enlarging mass in the lateral upper eyelid, previously diagnosed as a chalazion. Computed tomography revealed a heterogeneous round lesion anterior to the orbital rim. Excisional biopsy was compatible with an adenoid cystic carcinoma. After excluding distant metastasis, and as the patient refused adjuvant radiotherapy, a second surgical procedure, with wide local excision, was indicated. Follow-up showed no recurrence. This case highlights the importance of performing a thorough clinical examination when diagnosing any lateral upper eyelid mass. A high index of suspicion for malignant tumors of the lacrimal gland should always be maintained, and a complete excision with histological analysis should be preferred whenever possible.

Selumetinib for plexiform neurofibromas in neurofibromatosis type 1: a single-institution experience.

BACKGROUND: Plexiform neurofibromas (PN) are the most frequent tumors associated with Neurofibromatosis type 1 (NF-1). PN can cause significant complications, including pain, functional impairment, and disfigurement. There is no efficient medical treatment and, surgical resection of large PN is frequently infeasible. Selumetinib (AZD6244/ARRY-142886) is a mitogen-activated protein kinase enzyme (MEK1/2) inhibitor and works by targeting the MAPK pathway. It is an investigational treatment option for inoperable symptomatic PN associated with NF-1. Herein, we describe a single institutional experience with selumetinib for inoperable PN in NF-1. METHODS: Case series study of demographics, clinical, baseline characteristics, treatment effect, and follow-up of consecutive genetically confirmed NF1 patients with inoperable PN associated with significant or potential significant morbidity treated with selumetinib (April 2018 to April 2019). RESULTS: Nineteen patients were treated with selumetinib. Predominant target locations were head and neck (31.6%, 6/19), chest (26.3%, 5/19) and pelvis (21%, 4/19) and the most important comorbidities were disfigurement (47.4%, 9/19) and pain (26.3%, 5/19). The mean follow-up time was 223 days (range 35-420 days). All but one had sustained clinical improvement, mainly in the first 60-90 days of treatment. In one patient, the treatment was suspended after 168 days (lack of clear benefit and left ventricular ejection fraction drop). There were no adverse effects leading to treatment suspension. CONCLUSIONS: In the first observational study of selumetinib for NF-1 associated PN we showed that the drug was associated with clinical and radiological improvement. Our study also confirms the safety described in the clinical trials.

Medication-Related Osteonecrosis of the Jaw: A Systematic Review of Case Reports and Case Series.

Medication-related osteonecrosis of the jaw (MRONJ) is a rare condition, typically seen in patients receiving antiresorptive or antiangiogenic drugs. This study aims to synthesize reports and case series of MRONJ regarding sociodemographic and clinical characteristics and to evaluate the effectiveness of the treatments applied. Following PRISMA guidelines, a search for case reports and case series was carried out in the PubMed-Medline database until March 2024. A total of 88 articles were included in this review, in a total of 151 cases. The key findings reveal that females were the most affected individuals (71% of the cases) with the average age at diagnosis being 66.27 years with a standard deviation of ±13.03. Stage 2 was the most observed stage, in 43% of cases, and zoledronic acid was the most commonly used drug (32% of cases). The oral route was the most common route of administration, in 26% of cases, with an average administration duration of 60.88 months (standard deviation ± 50.92). The mandible was the most commonly affected anatomical location (in 60% of cases). Amoxicillin + clavulanic acid (875 mg + 125 mg) and chlorhexidine (0.12%) were the most used antibiotics and mouthwash, with 16% and 26% of the cases, respectively. Surgical debridement was the most common surgical procedure, in 32% of cases, while the use of an L-PRF membrane was the most prevalent unconventional treatment, in 21% of cases. This study identified a statistically significant relationship between unconventional treatments and the cure of MRONJ (p < 0.001), indicating the need for further research to confirm these results.

Metastasis of malignant tumors to the oral cavity: Systematic review of case reports and case series.

OBJECTIVE: To summarize published information regarding malignant tumors with metastasis to the oral cavity. MATERIALS AND METHODS: This was a systematic review with meta-analysis. An electronic search of Pubmed, Scopus, and Google Scholar databases from inceptions to February 2022 were performed. Only case reports or case series with histopathological results demonstrating metastasis to the oral cavity were included. The main outcomes included demographics, primary site, metastatic site, clinical manifestations, and patient survival. The quality of primary articles was assessed using the Joanna Briggs Institute - University of Adelaide scorecards for case reports and case series. Descriptive analysis and a Kaplan-Meier survival curve were performed. RESULTS: 273 articles were selected (50 case series and 223 case reports), for a total of 950 cases. The mean age was 57.11 years. Males were more affected (57.5%). The most common primary sites in women and men was breast (29.8%), and lung (24.8%), respectively. In ∼1/3 of the cases, oral metastasis preceded tumor dissemination. Jawbones were more affected (56.7%) than soft tissues (37.9%), with the mandible being the most affected site (45.5%), followed by the gingiva (19.9%). The most common clinical manifestation was a mass or nodule. Most radiographic evidence was radiolucency of the jaw (60.6%). 3-year and 5-year survival rates were 14.2 and 10.7%. In the majority of cases, the primary tumor was the first to be diagnosed, while in 30.4%, metastasis was the first sign of the disseminated disease. This can be implied that the oral metastasis should be included in the differential diagnosis list of the oral diseases. CONCLUSION: Clinicians should be aware of the possibility of, albeit uncommon, oral metastases. Because the extracted data in this review was relatively generalized, the investigators cannot develop the diagnostic clues of oral metastasis, which require further investigations.

Ectopic thyroid gland resembling a thyroglossal duct cyst.

Key Clinical Message: This rare diagnosis highlights the importance of anatomical and embryological knowledge in the differential diagnosis of neck masses. Abstract: A 41-year-old woman was referred to the head and neck surgery department because of a large midline neck mass at the level of the hyoid bone that was diagnostic for functioning thyroid tissue in a totally ectopic location.

Retrospective Study of 114 Free Flaps for Head and Neck Oncological Reconstruction in a Portuguese Tertiary Cancer Center.

INTRODUCTION: The Portuguese experience in microsurgical reconstruction of the head and neck after oncological surgery is scantly described. The primary aim of this study was to characterize the use of microvascular reconstruction after head and neck tumor resection in a Portuguese tertiary oncological centerMaterial and Methods: The authors retrospectively evaluated 114 microvascular free flap procedures performed for head and neck reconstruction after oncological resection in a department of Head and Neck Surgery of a Portuguese tertiary oncological center. Patients were operated on from January 2012 to May 2018. Data on patient demographic features, tumour characteristics, perioperative complications, postoperative aesthetic and functional results, survival time and time to recurrence were extracted. RESULTS: Most tumours mandating microsurgical reconstruction were mucosal squamous cell carcinomas (85%) and were located in the oral region (95.6%). Around 45% of the patients had a T4a tumour and 30% a T2 tumour. Cervical metastases were present in 45.6% of the cases. The radial forearm flap and the fibular flap were the most commonly used microsurgical reconstructive options (58% and 41%, respectively). More than 80% of patients had no post-operative complications. Partial necrosis of the flap occurred in 6.1% of patients, while total flap necrosis occurred in 3.5% of cases. Aesthetic and functional results were considered at least satisfactory in all patients in which the flaps survived. DISCUSSION: This study is by far the largest series of microsurgical head and neck reconstruction after oncological surgery reported by a single tertiary centre in Portugal. Survival and functional benefits are similar to those reported in other large oncological centres in the world. CONCLUSION: Microvascular reconstruction seems like a reliable treatment option in head and neck oncological surgery at our institution.

Introdução: A experiência portuguesa na reconstrução microcirúrgica da cabeça e pescoço após cirurgia oncológica está escassamente descrita. O objectivo deste estudo foi caracterizar a reconstrução microcirúrgica da cabeça e pescoço num centro de referência terciário português. Material e Métodos: Os autores avaliaram retrospetivamente 114 procedimentos de retalhos livres microvasculares realizados para reconstrução de cabeça e pescoço após ressecção oncológica num departamento de Cirurgia de Cabeça e Pescoço de um centro oncológico terciário português. Os doentes foram operados no período de janeiro de 2012 a maio de 2018. Foram registadas as características demográficas dos doentes, as características do tumor, as complicações peri operatórias, os resultados estéticos e funcionais pós-operatórios, bem como o tempo de sobrevida e o tempo de recorrência. Resultados: A maior parte dos tumores estava localizada na região oral (95,6%), sendo o carcinoma de células escamosas o tipo histológico mais frequente. Os retalhos antebraquial radial e fibular foram as opções reconstrutivas mais usadas (58% e 41%, respetivamente). Mais de 80% dos doentes não apresentaram complicações pós-operatórias. A necrose parcial do retalho ocorreu em sete doentes (6,1%), enquanto a necrose total do retalho ocorreu em apenas quatro casos (3,5%). Os resultados estéticos e funcionais foram considerados pelo menos satisfatórios em todos os doentes em que os retalhos sobreviveram. Discussão: Este trabalho, que descreve a maior série de casos de reconstrução microcirúrgica após cirurgia oncológica da cabeça e pescoço, identificou benefícios funcionais e de sobrevivência semelhantes aos descritos em outros centros oncológicos mundiais. Conclusão: A reconstrução microvascular parece ser uma opção fiável e eficaz no âmbito da cirurgia oncológica de cabeça e pescoço na nossa instituição.

Ghost cell odontogenic carcinoma of the left maxilla.

We report the case of a 25-year-old man with a maxillary ghost cell odontogenic carcinoma (GCOC). The patient presented to the maxillofacial and head and neck surgery clinic with a growing lump in the left maxilla. Initial workup with CT revealed a cystic lesion in the left upper jaw with associated bone erosion and an enhancing soft-tissue component. Enucleation showed a GCOC associated with a calcifying odontogenic cyst. After the diagnosis was obtained, the patient underwent widening of the first surgical resection. GCOCs are rare odontogenic neoplasms with unspecific clinical and imaging presentation, whose definitive characterisation is based on pathology. Current treatment approaches mainly involve surgical excision, but the prognosis is highly unpredictable due to intertumoral heterogeneity. As tumour recurrences occur in 73% of cases, radical surgery with negative margins is highly recommended. Therefore, it is essential to recognise this entity to offer patients adequate management.

Dentinogenic ghost cell tumor - Case report of a rare entity.

INTRODUCTION: Dentinogenic ghost cell tumor (DGCT) is an entity with about 60 cases reported in the literature. It is a benign odontogenic tumor, despite being locally invasive and associated with a risk of local recurrence. PRESENTATION OF CASE: A 47-year-old woman presented with a 2-year-old expansive bone lesion. Radiologically, a multilocular mass was identified in the left superior maxilla, compatible with a tumor of odontogenic origin. She was submitted to an extended resection, and the histology was consistent with a DGCT. DISCUSSION: Central DGCT affects mainly male patients between the fourth and sixth decades, with a predilection for the posterior portion of the jaws. The symptoms are unspecific, and a vast percentage of patients is asymptomatic. Radiographically a unilocular feature is commonly found, unlike this case. The recommended treatment is extended local resection due to its high recurrence rate. CONCLUSION: Due to its rarity, knowledge of this entity is necessary for a better diagnostic and therapeutic guidance.

Pretreatment Blood Markers in the Prediction of Occult Neck Metastasis: A 10-Year Retrospective Study.

Introduction The purpose of this study was to clarify the role of inflammatory blood markers in the management of early-stage (T1-T2) oral squamous cell carcinoma (OSCC) of the tongue in patients with a clinically negative neck.  Materials and methods We undertook a retrospective chart review of 102 patients with early-stage OSCC of the tongue, subjected to tumor resection and elective neck dissection. Based on postsurgical histopathological examination results, we divided our cohort into pN+ and pN0 groups. Afterwards, we analyzed the role of pretreatment inflammatory blood markers in predicting occult neck metastasis. We also evaluated neutrophil-lymphocyte ratio (NLR) association with the depth of invasion (DOI) of the primary tumor. Results We found a significant association of NLR (p=0.001) and monocyte-lymphocyte ratio (p=0.011) with neck status on univariate analysis. Multivariate analysis showed that only NLR (p=0.02) was an independent risk factor for occult metastasis among inflammatory blood markers. Receiver Operating Characteristic curve analysis and Younden's Index determined the NLR value of 2.96 as the most adequate cut-off value for neck status prediction. NLR values of pretreatment workup also had a significant association with the DOI of the primary tumor (p=0.018). Conclusion Our study supports the role of pretreatment NLR in predicting occult neck metastasis in early-stage OSCC of the tongue. It also sheds some light over the potential of NLR as a predictor of the primary tumor's DOI.

Mucinous cystadenocarcinoma of the oral tongue: malignant transformation from a mucinous cystadenoma?

Mucinous cystadenocarcinoma of minor salivary glands is an extremely rare entity that has only recently been described, with a few published cases in the English literature. A 42-year-old woman with a history of a surgically excised mucinous cystadenoma of the oral tongue, presented with a painful swelling in the oral tongue slowly growing for 1 month. On clinical examination, there was a firm, relatively well-circumscribed mass in the left posterior border of the mobile tongue. Subsequent MRI scan revealed a heterogeneous lesion composed of multiple cysts separated by contrast enhancing septa, in the posterior two-thirds of the left tongue. Imaging findings were similar to those of the previously resected mass, suggesting local relapse of the primary lesion. A complete surgical excision was performed and the histopathological examination revealed typical features of a low-grade mucinous cystadenocarcinoma of minor salivary glands.