A rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland.

Carcinoma ex pleomorphic adenoma (CEPA) of the lacrimal gland is a rare malignant tumor that arises from a pre-existing pleomorphic adenoma. Lacrimal gland CEPA with mucoepidermoid histological subtype is exceedingly rare. Diagnosis can be aided by radiographic findings, though the gold standard is histopathological analysis following excisional biopsy. Management options include complete surgical excision with or without adjuvant radiation therapy based on tumor grade and invasiveness. We present a 76-year-old woman with 6 months of diplopia and unilateral proptosis. Her initial exam was remarkable for hypoglobus, proptosis, and limited elevation of the right eye. Computed tomography (CT) scan demonstrated a superior, well-circumscribed, extraconal orbital mass. An excisional biopsy was performed, and histopathological findings were consistent with mucoepidermoid carcinoma ex pleomorphic adenoma with positive margins in the tumor capsule. The patient received radiation therapy and remains markedly improved with no disease recurrence at 5 months post-operatively.

Presentation and outcomes of patients with clinically T1-2, N0 parotid mucoepidermoid carcinoma: The roles of elective neck dissection and adjuvant radiotherapy.

OBJECTIVES: Examine the role of elective neck dissection (END) and adjuvant radiotherapy (RT) in early-stage clinically N0 parotid mucoepidermoid carcinoma (MEC). METHODS: The study is a retrospective analysis of the National Cancer Database, 2004-2016. The study population included adult patients with MEC who underwent parotidectomy. RESULTS: A total of 1233 patients were included. Histopathology demonstrated well, moderately, and poorly differentiated MEC 47.12%, 39.98%, and 12.90% of the time, respectively. END was performed in 78.67% of patients, resulting in nodal upstaging in 4.43% and identification of extracapsular extension (ECE) in 0.72%. RT was utilized in 67.33% of patients with advanced pathological features. Neither END nor RT improved overall survival separately (p < 0.05) or combined (adjusted HR: 1.19, 95%CI: 0.52, 2.70, p = 0.68). CONCLUSION: This study provides an epidemiological perspective regarding patients with clinically T1-2, N0 MEC. There was no observed survival advantage with END and RT.

Prolonged response to checkpoint inhibitor therapy in two metastatic mucoepidermoid salivary gland carcinoma cases: a research report.

Salivary gland tumors (SGTs) are heterogeneous tumors that range from benign masses to aggressive high-grade carcinomas with distant metastatic potential and limited response to chemotherapy. Mucoepidermoid carcinoma (MEC) accounts for 10% of SGTs and has a poor prognosis. In this research report, we describe two cases of metastatic high-grade MECs with prolonged response to immune checkpoint inhibitor pembrolizumab. Case 1 presented with a left neck mass, and biopsy of the parotid mass revealed MEC. The patient underwent surgical resection and adjuvant chemoradiation therapy for stage IVB disease. Post-treatment, she was found to have brain and spinal metastases and was placed on pembrolizumab. Case 2 presented with a left neck mass, and biopsy of the right parotid gland revealed MEC. Further staging demonstrated metastatic disease in the lungs, and he was placed on pembrolizumab. Both cases of MEC demonstrated prolonged extracranial responses to pembrolizumab. Although both cases reported little to no PD-L1 expression, these results demonstrate immunotherapy efficacy in advanced/metastatic MEC.

Iodine-125 brachytherapy for the treatment of central mucoepidermoid carcinoma of the jaw in a pre-teen.

Central mucoepidermoid carcinoma (MEC) of the jaw is a rare malignant neoplasm, even rarer in teenagers. Radical surgical resection, such as en bloc resection or segmental resection, is the main treatment for MEC of the jaw. This surgical treatment results in a loss of integrity of the jaw. The successful application of iodine-125 brachytherapy for the treatment of intraosseous MEC of the mandible in an 11-year-old girl is reported here. No local recurrence or distant metastasis was observed during 6 years of follow-up. The integrity of the mandible was preserved and the development of the mandible was not significantly affected. Iodine-125 brachytherapy is a potential alternative treatment for central mucoepidermoid carcinoma of the jaw, especially in teenagers, and may preserve the continuity and function of the jaw.

Long-term outcomes of patients in different histological subtypes of primary nasopharyngeal adenocarcinoma: A single-center experience with 71 cases.

OBJECTIVE: This study is aimed to evaluate the long-term outcomes and management approaches in different histological subtypes of primary nasopharyngeal adenocarcinoma (NPAC). MATERIALS AND METHODS: 71 patients with NPAC at our institution between 1984 and 2016 were reviewed, including adenoid cystic carcinoma (ACC) in 43 patients, mucoepidermoid carcinoma (MEC) in 17 patients, and primary traditional adenocarcinoma (AC) in 11 patients. 37 patients received primary radiotherapy and 34 patients underwent primary surgery. RESULTS: The median time of follow-up was 77 months. The 5-year overall survival rate (OS), locoregional failure-free survival rate (LRFFS) and distant metastasis failure-free survival rate (DFFS) were 69.9%, 67.1% and 77.9%, respectively. Patients who received combined modality therapy had better 5-year OS (73.7% vs 66.2%, p = 0.065) and LRFFS (73.1% vs 64.5%, p = 0.047) than patients receiving single modality therapy. Regarding the different histological subtypes, the survival rates of patients with ACC undergoing primary radiotherapy and primary surgery were similar (5-year OS 82.3% vs 68.8%, LRFFS 70.0% vs 70.8%, p＞0.05). As to patients with MEC and AC, those who underwent primary surgery achieved better 5-year OS (75.6% vs 45.5%, p = 0.001) and LRFFS (70.6%% vs 57.1%, p = 0.014) than those who received primary radiotherapy. Multivariate analyses indicated that histological subtypes and radiotherapy technique were independent factors for OS. CONCLUSIONS: The optimal treatment policy for NPAC remained the combination of radiotherapy and surgery. For patients with ACC, radiotherapy could be considered as the primary treatment. Surgery was suggested to be the primary treatment in patients with MEC and AC.

Rare entities in head-and-neck cancer: salvage re-irradiation with carbon ions.

BACKGROUND: The objective of this investigation is to evaluate the outcomes and toxicity of carbon-ion re-irradiation (CIR) in patients with rare head and neck cancers (HNC). There is a paucity of data regarding treatment approaches in this patient cohort, which we aim to address in this work. METHODS: Thirty-two (n = 32) consecutive patients with uncommon HNC treated between 2010 and 2017 were retrospectively analyzed in terms of clinical outcomes, patterns of failure, and toxicity. RESULTS: Mucoepidermoid carcinoma (MEC) was the most common histology (22%). Patients received a median cumulative dose equivalent in 2 Gy fractions (EQD2) after CIR of 128.6 Gy (range, 105.8-146.5 Gy). The local and distant control rates 1 year after CIR were 66 and 72%. No serious acute or late toxicity (≥ grade 3) after CIR was observed. CONCLUSIONS: CIR may represent an effective and safe treatment alternative to palliative systemic therapies in these rare indications.

The impact of age on the outcome of patients treated with radiotherapy for mucoepidermoid carcinoma (MEC) of the salivary glands in the head and neck: A 15-year single-center experience.

INTRODUCTION: Data regarding treatment and survival outcome of patients with mucoepidermoid carcinoma of the head and neck are limited to case reports and case series. As a consequence of lacking evidence, treatment guidelines do not exist. We aimed to analyze the effect of modern radiotherapy in form of intensity modulated radiotherapy (IMRT) either with simultaneously integrated boost or carbon ion boost on local control and survival for a relatively large patient collective. MATERIALS AND METHODS: Patient records of 62 consecutive patients treated with postoperative (n = 53, 85%) or definitive (n = 9, 15%) radiotherapy between 2004 and 2019 were analyzed retrospectively. Kaplan-Meier estimates for overall survival (OS), distant progression-free survival (PFS), local control (LC) and locoregional control (LRC) were statistically calculated and prognostic factors were identified using the log-rank test. Toxicity was assessed according to the Common Terminology Criteria for Adverse Events (CTCAE). RESULTS: The median follow-up was 47 months (range, 4-188 months). The 3-year OS, DPFS, LC and LRC, estimated by Kaplan-Meier curves, were 82%, 87%, 89% and 92%, the estimated 5-year OS, DPFS, LC and LRC were 78%, 87%, 84% and 88%, respectively. In univariate analysis, age >56 years (vs. age ≤56 years) was identified as the only independent negative prognostic factor for decreased OS (HR = 1.078; 95%-CI = 1.029-1.130; p = 0.001), DPFS (HR = 1.055; 95%-CI = 1.000-1.114; p = 0.051) and LC (HR = 1.087; 95%-CI = 1.022-1.157; p = 0.008). Treatment was well tolerated without any grade ≥4 toxicity. Acute and late grade 3 toxicities were rare with 16% acute (n = 10) and 13% late toxicities (n = 8). CONCLUSION: Radiotherapy with intensity modulated radiotherapy including either simultaneously integrated photon boost or active raster-scanning carbon ion boost for mucoepidermoid carcinomas of the head and neck resulted in excellent survival outcome and locoregional control with moderate toxicity. However, patients older than 56 years seem to have a disadvantage in all calculated endpoints (OS, DPFS, LRC) due to frequent local and distant relapses. CONDENSED ABSTRACT: Modern radiotherapy with intensity modulated radiotherapy including either a simultaneously integrated photon boost or carbon ion boost for mucoepidermoid carcinoma results in excellent survival outcome and locoregional control with moderate toxicity. The 5-year OS, DPFS, LC and LRC, estimated by Kaplan-Meier curves, were 89%, 75%, 84% and 80%, respectively. Patients older than 56 years seem to have a disadvantage in all calculated endpoints (OS, DPFS, LRC).

Efficacy of combined surgery and 125 I seed brachytherapy for treatment of primary mucoepidermoid carcinoma of the parotid gland.

BACKGROUND: This study aimed to determine the effectiveness and safety of surgery combined with postoperative 125 I seed brachytherapy for treatment of primary mucoepidermoid carcinoma (MEC) of the parotid gland. METHODS: Retrospective analysis of data of patients with MEC (n = 108) treated with surgery plus postoperative 125 I seed brachytherapy between 2004 and 2016. Overall survival (OS), disease-free survival (DFS), local control rate (LCR), distant metastasis, and radiation-associated toxicities were analyzed, and factors affecting outcomes were evaluated. RESULTS: The 5- and 10-year OS were 98.8% and 95.8%, respectively. The DFS and LCR at 5 and 10 years were all 91.4%. Age ≥ 60 years (hazard ratio [HR] = 6.86, 95% confidence interval [CI]: 1.54-30.55) and T4 disease (HR = 7.15, 95% CI: 1.40-36.52) were poor prognostic factors. Acute radiation-associated toxicities were minor. CONCLUSION: Surgery plus 125 I seed brachytherapy appears to be an effective treatment for parotid gland MEC, capable of providing satisfactory outcomes with few complications.

Mucoepidermoid Carcinoma of the Parotid: Very Close Margins and Adjuvant Radiotherapy.

BACKGROUND/AIMS: The importance of adjuvant radiotherapy in patients with close margin resections for mucoepidermoid carcinoma of the parotid gland remains unclear. METHODS: Patients who underwent parotidectomy for mucoepidermoid carcinoma with or without adjuvant radiotherapy at a single academic tertiary care center from 2000 to 2014 were identified. Included patients had negative but close (≤2 mm) surgical margins without other high-risk histopathological factors including advanced T-stage, positive nodal disease, lymphovascular or perineural invasion, or high-grade histology. RESULTS: Nineteen patients were identified, of whom 15 (79%) were observed postoperatively, while 4 (21%) underwent adjuvant radiotherapy. There were no significant differences in extent of parotidectomy, elective neck dissection, T staging, or tumor size between patients who were observed and those undergoing adjuvant radiation. There were no locoregional or distant recurrences in any patients at a mean follow up 74.3 months. Patients undergoing adjuvant radiation, however, had significantly more intermediate-grade as compared to low-grade histology (75% vs. 13%, difference 62%, 95% CI 4% to 100%). CONCLUSIONS: Patients with negative but close (≤2 mm) surgical margins without other high-risk histopathological factors have excellent long-term locoregional control with surgery alone. The effects of adjuvant radiotherapy for those who have intermediate-grade disease remain uncertain.

GPR55 controls functional differentiation of self-renewing epithelial progenitors for salivation.

GPR55, a lipid-sensing receptor, is implicated in cell cycle control, malignant cell mobilization, and tissue invasion in cancer. However, a physiological role for GPR55 is virtually unknown for any tissue type. Here, we localize GPR55 to self-renewing ductal epithelial cells and their terminally differentiated progeny in both human and mouse salivary glands. Moreover, we find GPR55 expression downregulated in salivary gland mucoepidermoid carcinomas and GPR55 reinstatement by antitumor irradiation, suggesting that GPR55 controls renegade proliferation. Indeed, GPR55 antagonism increases cell proliferation and function determination in quasiphysiological systems. In addition, Gpr55-/- mice present ~50% enlarged submandibular glands with many more granulated ducts, as well as disordered endoplasmic reticuli and with glycoprotein content. Next, we hypothesized that GPR55 could also modulate salivation and glycoprotein content by entraining differentiated excretory progeny. Accordingly, GPR55 activation facilitated glycoprotein release by itself, inducing low-amplitude Ca2+ oscillations, as well as enhancing acetylcholine-induced Ca2+ responses. Topical application of GPR55 agonists, which are ineffective in Gpr55-/- mice, into adult rodent submandibular glands increased salivation and saliva glycoprotein content. Overall, we propose that GPR55 signaling in epithelial cells ensures both the life-long renewal of ductal cells and the continuous availability of saliva and glycoproteins for oral health and food intake.

Radiation treatment to a postresection primary mucoepidermoid carcinoma (MEC) of the conjunctiva with positive margins at the Tenon's fascia-A case study.

The primary occurrence of mucoepidermoid carcinoma (MEC) of the conjunctiva is extremely rare, aggressive, and easily mistaken for squamous cell carcinoma. With fewer than 50 cases reported in the literature, there is no consensus on the most effective treatment. Radiation is an alternative to enucleation or orbital exenteration with the potential of eye preservation. We investigated several radiation approaches for a case with postresection positive margins at Tenon's fascia, and reported the patient's clinical course during the treatment and for a short time thereafter. An otherwise healthy 64-year-old male presented with MEC extending to the deep margin at the Tenon's fascia. Plans for 4 different radiation therapy treatment modalities (Intensity Modulated Radiation Therapy [IMRT], Volumetric Modulated Radiation Therapy [VMAT], double scattering (DS) proton, and reverse eye plaque low dose rate [LDR] ) were created and compared based on tumor coverage and normal tissue doses. The planning target volume (PTV) was too large and nonuniform for an eye plaque application. Using the largest plaque available (22 mm), the calculated minimum dose to the PTV was 57% while the dose to the skin was 1000% of the prescription. The proton plan completely spared the contralateral ocular structures and reduced the max doses to the ipsilateral macula and optic nerve, but was not clinically available at the time of treatment. The IMRT and VMAT plans produced similar dose distributions to each other, but VMAT further minimized dose to the ipsilateral eye. Due to the uniqueness of this case, a thorough study of the available radiation treatment options was deemed necessary. All of the external beam treatment techniques produced acceptable plans with VMAT producing the best available plan in this case. The patient was treated with the VMAT plan with a prescription of 6600 cGy in 30 fractions. At 5 months post-treatment, the patient is recovering from expected acute responses to radiation with follow ups scheduled.

Postoperative radiotherapy for T1/2N0M0 mucoepidermoid carcinoma positive for CRTC1/3-MAML2 fusions.

BACKGROUND: The National Comprehensive Cancer Network (NCCN) guidelines recommend considering postoperative radiotherapy (PORT) for completely resected T1/2N0M0 salivary mucoepidermoid carcinomas when they show tumor spillage, perineural invasion, or intermediate/high-grade histology. CRTC1/3-MAML2 fusions have been associated with a favorable clinical outcome. METHODS: Forty-seven T1/2N0M0 mucoepidermoid carcinoma cases positive for CRTC1/3-MAML2 fusions were completely resected and were not treated with PORT. RESULTS: Pathologically, none of the cases showed tumor spillage or perineural invasion. Cases with intermediate/high-grade histology numbered 9 (19%) to 26 (55%) with the currently used 3 different grading systems. During the follow-up (median 60 months), locoregional tumor recurrence occurred in 4 cases, which were treated with surgery alone. At the last follow-up (median 60 months; 7-160), all patients were alive with no evidence of disease. CONCLUSION: An excellent prognosis may be achieved without PORT in T1/2N0M0 mucoepidermoid carcinoma patients positive for CRTC1/3-MAML2 fusions when the tumors are completely resected without tumor spillage.

Adjuvant conundrum in central mucoepidermoid carcinoma of the mandible: case presentation and literature review.

The cornerstone modality of treatment of central mucoepidermoid carcinoma (CMEC) of the mandible is surgery, optimally, an en bloc resection with/without segmental or hemimandibulectomy. Notwithstanding the documentation of a survival benefit in few case reports with the addition of postoperative radiotherapy in carefully selected high-risk patients, there does not exist a clearly defined consensus regarding the role of adjuvant radiotherapy. We report the case of a 49-year-old man who presented with right lower jaw swelling which on imaging was found to be a multiloculated lesion causing bony expansion and cortical destruction of the mandible and was diagnosed with CMEC after radiological and histopathological criteria were met. He underwent right hemimandibulectomy and histopathology showed squamous and mucinous cells with positive mucicarmine staining and characteristic immunohistochemistry markers confirming the diagnosis of CMEC. He subsequently underwent adjuvant radiotherapy and is disease free 5 years since treatment completion.

Multi-institutional retrospective study of mucoepidermoid carcinoma treated with carbon-ion radiotherapy.

This study aimed to evaluate the clinical outcomes of patients with mucoepidermoid carcinomas in the head and neck treated with carbon-ion radiotherapy. Data from 26 patients who underwent carbon-ion radiotherapy in four facilities were analyzed in this multi-institutional retrospective study: the Japan Carbon-ion Radiation Oncology Study Group. The median follow-up time was 34 months. One patient experienced local recurrence, and the 3-year local control rate was 95%. One patient developed lymph node recurrence and five developed distant metastases. The 3-year progression-free survival rate was 73%. Five patients died, two of mucoepidermoid carcinoma and three of intercurrent disease. The 3-year overall survival rate was 89%. Acute mucositis and dermatitis of grade 3 or higher were experienced by 19% and 8% of patients, respectively; these improved with conservative therapy. Late mucositis and osteonecrosis of jaw were observed in 12% and 23% of patients, respectively. The 3-year cumulative rate of any late adverse event of grade 3 or higher was 14%. None of the patients died of the acute or late adverse events. Carbon-ion radiotherapy was efficacious and safe for treating mucoepidermoid carcinoma in this multi-institutional retrospective study (registration no. UMIN000024473). We are currently undertaking a prospective multicenter study.

Aggressive Salivary Malignancies at Early Stage: Outcomes and Implications for Treatment.

BACKGROUND: Few studies have examined whether the use of adjuvant treatment impacts survival for early stage high-grade salivary tumors. METHODS: A retrospective review of the SEER database between 1973 and 2012 was performed. Patients with high-grade major salivary gland tumors including salivary duct carcinoma, carcinoma ex-pleomorphic adenoma, high-grade mucoepidermoid carcinoma, or adenocarcinoma, NOS were identified. Only stage I-II tumors were included. The impact of radiation status on observed and relative survival was examined. RESULTS: Five hundred seventy-four patients with high-grade, early stage salivary tumors met inclusion criteria. Sixty-seven percent of patients received radiation therapy. There was no difference in observed or relative survival based on having received radiation. CONCLUSIONS: Adjuvant radiation is indicated for advanced stage tumors or early stage tumors with adverse features. For early stage tumors without adverse features, there was no survival benefit from radiation therapy. Adjuvant radiation should be decided on a case-by-case basis for these patients.

Definitive carbon-ion radiotherapy for locally advanced parotid gland carcinomas.

BACKGROUND: The purpose of this study was to present our evaluation of the safety and efficacy of carbon-ion radiotherapy (C-ion RT) for locally advanced parotid gland carcinomas. METHODS: Clinicopathological features and outcomes were evaluated in 46 patients receiving C-ion RT for parotid gland carcinomas. RESULTS: Sixteen patients had adenoid cystic carcinoma; 8 had adenocarcinoma, 8 had mucoepidermoid carcinoma, and 14 had other carcinomas. T2, T3, T4a, and T4b diseases were diagnosed in 3, 18, 8, and 17 patients, respectively. C-ion RT was provided to 25 patients as the primary treatment, to 20 patients for local recurrences after surgery, and to 1 patient for residual tumor after surgery. During follow-up (median duration, 62 months), 5-year local control and overall survival (OS) rates were 74.5% and 70.1%, respectively. Of the 30 patients without facial nerve palsy before C-ion RT, 25 showed no radiation-induced facial nerve palsy. CONCLUSION: C-ion RT is effective and has acceptable toxicity levels for locally advanced parotid gland carcinomas. © 2016 Wiley Periodicals, Inc. Head Neck 39: 724-729, 2017.

Persistent Pneumonia: Time to Take a Closer Look.

Primary pulmonary tumors are rare in pediatrics. When they are encountered, they are usually carcinoid tumors or mucoepidermoid carcinomas. We present a patient who presented to both his primary care physician and the pediatric emergency department with recurrent bouts of wheezing and pneumonia, none of which ever completely resolved despite appropriate treatment. The patient had multiple chest films, which demonstrated the persistence of what appeared to be a right-sided infiltrate/atelectasis. Ultimately, the patient underwent a diagnostic workup that included a computed tomography scan and bronchoscopy. These studies revealed the presence of a bronchial mucoepidermoid carcinoma. The patient was successfully treated with photoablation of the lesion through the involvement of multiple subspecialists, including pediatric pulmonology, pediatric surgery, pediatric otolaryngology, and pediatric oncology. We discuss the incidence and epidemiology of pediatric bronchial tumors in general and mucoepidermoid carcinoma in particular as well as diagnosis, treatment options, and prognosis. Emergency physicians must maintain a high index of suspicion for alternate diagnoses in patients whose disease fails to respond to traditionally accepted therapy.

Carbon ion therapy (C12) for high-grade malignant salivary gland tumors (MSGTs) of the head and neck: do non-ACCs profit from dose escalation?

PURPOSE: To evaluate the use of high-dose radiotherapy using carbon ions (C12) on non-adenoid cystic malignant salivary gland tumors (MSGT). PATIENTS AND METHODS: Between 2009 and 2013, patients with biopsy-proven non-ACC MSGT histologies of the head and neck received a combined regimen of IMRT plus C12 boost. Treatment toxicity (CTC v3), response (RECIST 1.1), control and survival rates were retrospectively analyzed. RESULTS: 40 patients with pathologically confirmed non-ACC MSGT (T4: 45 %; N+: 40 %; gross residual: 58 %; mucoepidermoid carcinoma (MEC): 45 %; adenocarcinoma: 20 %) were treated with a median of 74 GyE (80 Gy BED). Chemoradiation was given in 5 patients with MEC. Grade III acute toxicity was observed in up to 15 % (mucositis, dermatitis, dysphagia), no higher-grade late toxicity occurred to date. At a follow-up of 25.5 months, LC, and PFS at 2 and 3 years are 81.5 % (LC) and 66.8 % (PFS), OS at 2 and 3 years is 83.6 % and 72.8 %. Most frequent site of disease progression was distant metastasis. Histologic subtype correlated with LC and PFS. Resection status (gross vs microscopic disease) had no significant effect on LC, PFS, or OS. CONCLUSION: The treatment is well tolerated, no higher grade late effects were observed. Considering the negative pre-selection, LC, PFS and OS are promising. While histology and site of origin significantly influenced control and survival rates, resection status did not, potentially due to the effect of dose escalation.

Carcinoma Mucoepidermóide do Pulmão ­ estado da arte a propósito de um caso clínico / Mucoepidermoid carcinoma of the lung ­ state of art with regard to a case report

O Carcinoma Mucoepidermóide do Pulmão (CMP) é um tumor com baixo potencial de malignidade; acredita-se que é indolente, mas pouco se sabe sobre as suas características dada a sua baixa incidência (0,2% de todos os tumores pulmonares). Os autores apresentam um caso clínico de um Carcinoma Mucoepidermóide do Pulmão de alto grau, a que se associa, habitualmente, metastização à distância, recorrência do tumor e mau prognóstico. Destaca-se a exuberante metastização (pleural, pericárdica, ganglionar e cerebral), que contrasta com o curso da doença, relativamente indolente, volvidos 12 meses do diagnóstico. Dado não ter indicação cirúrgica, a doente foi tratada com radioterapia e quimioterapia, apesar de ainda não estar definido um tratamento standard para este tipo histológico. A raridade do diagnóstico e os escassos estudos da literatura condicionam a abordagem terapêutica sistémica destes doentes, constituindo um desafio para a comunidade médica. O possível papel da terapêutica dirigida, como os inibidores da tirosina cinase do receptor do fator de crescimento epidérmico (EGFR) ou a terapêutica contra o oncogene de fusão CRTC1-MAML2, tem sido investigado, em doentes com Carcinoma Mucoepidermóide do Pulmão de alto grau.

Mucoepidermoid Carcinoma of the Lung (MECL) is a tumor of low malignant potential; we believe it is indolent, but little is known about its clinical features because of the low incidence rate (incidence of 0.2% of all lung cancers). We present a clinical case of a high degree Mucoepidermoid carcinoma of the lung, which frequently have distant metastasis, tumor recurrence and a bad prognosis. It is highlighted in this case the exuberant metastases (pleural, pericardial, cerebral, lymph nodes), contrasting with the relatively indolent course of the disease since the diagnosis (10 months ago). Our patient was treated with radiotherapy and chemotherapy, although effective treatment measures for high-grade tumors have not been established. The rarity of the diagnosis and the rare studies in the literature affect the systemic approach to these patients, becoming a challenge for the scientific community. The role of targeted therapy directed against the epidermal growth factor receptor (EGFR) and the novel fusion oncogene CRTC1-MAML2, is being investigated in high-grade tumors.