Metastatic low-grade myoepithelial carcinoma of lung managed with low-dose external beam radiotherapy.

Myoepithelial tumor of lung is a rare tumor; the histopathological findings resemble the myoepithelial tumors of the salivary gland. Distinguishing low-grade nonmetastatic myoepithelial carcinoma from benign myoepithelioma can be challenging both radiologically and histomorphologically. We present a case report of a low-grade myoepithelial carcinoma of lung with contralateral lung metastasis which was treated with low-dose external beam radiotherapy.

Epithelial-myoepithelial carcinoma of the parotid gland with primary lung cancer: A rare case report.

INTRODUCTION: Epithelial-myoepithelial carcinoma (EMC) is a rare, low-grade malignancy that occurs primarily in the parotid gland and is most common in women aged 60 to 70 years. Cases of parotid EMC have been reported previously. Furthermore, some studies have suggested an increased risk of salivary gland tumors with secondary primary malignancies. There have been few reports of parotid EMC with other primary tumors. PATIENT CONCERNS: A 62-year-old Chinese man visited the hospital with a complaint of a mass on his left cheek that had persisted for 20 years. Routine pulmonary computed tomography showed a local ground glass shadow in the lower lobe of the right lung. DIAGNOSIS: The pathological diagnosis of lung was right lower lobe lung adenocarcinoma (pT1N0). Immunohistochemistry analysis showed that cytokeratin (CK)-7, NapsinA, and thyroid transcription factor-1 tested positive, while CK5/6, P40, and ALKD5F3 tested negative. The pathological diagnosis of left parotid gland: EMC. On immunohistochemistry staining, the outer cells expressed myoepithelial markers, such as CK5/6, P63, smooth muscle actin, while the inner cells expressed glandular epithelial markers, such as low-molecular-weight CK7 and CK8. INTERVENTIONS: The patient underwent resection of the lung and parotid tumors, and received preventive radiotherapy in the parotid gland area. OUTCOMES: The patient is in good condition. No symptom recurrence, distant metastatic spread or significant toxicity occurred during or after the treatment. The patient remains under regular surveillance. CONCLUSION: We report a rare case of parotid EMC with a second primary lung adenocarcinoma. This case is the third case of primary lung cancer associated with parotid EMC reported to date and the first to be reported in nearly 30 years.

Impact of adjuvant therapy on survival in patients with myoepithelial carcinoma: A systematic review and individual patient data analysis of 691 patients.

INTRODUCTION: Myoepithelial carcinoma (MEC) is an extremely rare low grade salivary gland neoplasm [1-4]. A surgical resection is considered as corner stone of therapy. Role of adjuvant therapy is not clear. METHODOLOGY: We performed systematic review and individual patient data analysis of 691 patients to look into the impact of adjuvant therapy and different prognostic variable for MEC. RESULTS: Data of 691 individual patients were retrieved from 340 publications. Median age of presentation was 56 years (Range: 0-103 years) with a trend of increasing incidence for increase in age. Major salivary glands (36.4%) were the commonest sub-site followed by minor salivary glands, skin and soft tissue, and breast. Median PFS and OS of entire cohort was 48 months (95% CI: 30-65 months) and 167 months (95% CI: 82-251 months). In univariate analysis A R0 resection was associated with significantly better PFS and OS. Median PFS and OS were significantly worse for patients with tumour size >5 cm compared to smaller tumours and for patients with a mitotic index >10/10 high power field (hpf) compared to lower mitotic index. Adjuvant radiation was found to reduce loco-regional recurrence. Adjuvant radiation and chemotherapy both were associated with negative impact on survival in univariate analysis. This negative impact on survival was lost in multivariate analysis. CONCLUSION: MEC appears to be a low grade malignancy with good survival outcome. A R0 resection should be the standard of care. Adjuvant radiation should be considered for patients with adverse risk features to improve loco-regional disease control.

Report of a unique case of myoepithelial carcinoma of left parotid gland with metachronous bilateral cavernous sinus metastasis.

Myoepithelial carcinoma (MC) is a rare, locally aggressive malignant neoplasm of the salivary glands. Only few evidences on its metastatic behavior are available in the literature. We herein present a unique case of MC of left parotid gland which metastasized to bilateral cavernous sinuses. The patient was successfully treated with palliative radiotherapy and chemotherapy.

Neoadjuvant radiation therapy for the management of myoepithelial carcinoma of the upper extremity.

Myoepithelial tumors of the soft tissue are a rare tumor displaying myoepithelial elements and lacking obvious ductal differentiation. The rarity of these precludes any evidence-based consensus regarding optimal management. Nevertheless, the current approach to these lesions begins with amputation or complete excision. The efficacy of neoadjuvant or adjuvant radiation therapy or chemotherapy has not been established. Here, we present the first report to the authors' knowledge of neoadjuvant radiation therapy for the treatment of this rare soft tissue neoplasm and review the management and outcomes of published cases of myoepithelial carcinoma. A patient with a soft tissue myoepithelial carcinoma that declined both amputation and chemotherapy was treated with neoadjuvant radiation therapy and wide surgical excision followed by a brachytherapy boost to the resected tumor bed. Neoadjuvant radiation therapy resulted in an excellent response with extensive treatment-related changes consisting predominantly of fibrosis, hyalinization and hemorrhage and only 10% residual viable myoepithelial carcinoma present in the surgical specimen.

Locally Advanced Malignant Myoepithelioma of the Parotid Gland Treated With Radiotherapy: A Case Study and Review of the BC Cancer Agency Experience.

OBJECTIVES: Malignant myoepithelioma of salivary glands is a rare neoplasm; most arise in the parotid gland and minor salivary glands of the palate. Surgery has been the mainstay of treatment. METHODS: This is case report of a patient treated with primary radical radiotherapy and retrospective review of institutional experience. RESULTS: An 87-year-old man with locoregionally advanced malignant myoepithelioma of the parotid gland received radiotherapy alone with complete clinical response and sustained 39 months of posttreatment. Between 1981 and 2012, 15 cases of malignant myoepithelioma of the parotid were seen. Thirteen patients received surgical excision and adjuvant radiotherapy. At a median follow-up of 47 months, 12 patients were alive without recurrence, 2 died without recurrence, and 1 died with metastatic myoepithelioma. CONCLUSIONS: Durable locoregional disease control and disease-free-survival was achieved in the majority of patients. The case reported suggests radiation therapy may be an effective treatment option for inoperable cases.

Mioepitelioma maligno recidivante de la mama, una evolución poco frecuente / Recurrent malignant myoepithelioma of the breast, an uncommon progression

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Chromosomal rearrangements in myoepithelial carcinoma of the breast that presented as metachronic double cancer with invasive ductal carcinoma in the ipsilateral breast.

Myoepithelial carcinoma of the breast is an extremely rare tumor composed entirely of malignant spindle cells with myoepithelial differentiation. The majority of previously reported cases have mainly described the clinicopathological features of the disease, and few have presented cytogenetic data. We herein present the case of a 48-year-old woman who was admitted with a left-sided breast lump in the inner upper quadrant that was initially diagnosed as a myoepithelioma with potentially malignant disorder. At 12 months after resection, she complained about a newly developed solid mass in the subareolar region of the ipsilateral breast that was diagnosed as an invasive ductal carcinoma. In addition, 16 months after the initial admission, a re-growing remnant lesion recurred in the inner upper quadrant and was ultimately diagnosed as a myoepithelial carcinoma. Lymph node metastasis of the myoepithelial carcinoma was also observed in her left axillary region 11 months after local recurrence. A cytogenetic analysis showed recurring specific chromosomal alterations both in the locally recurrent and in the lymph-node metastatic lesion: 48, XX, t(5;18)(q13;q23),del(6)(q?),+14. + mar1. To our knowledge, this is the first published report of clonal chromosomal rearrangements in myoepithelial carcinoma of the breast that presented as metachronic double cancer with invasive ductal carcinoma in the ipsilateral breast.

Myoepithelial Carcinoma: The Role of Radiation Therapy. A Case Report and Analysis of Data From the Surveillance, Epidemiology, and End Results (SEER) Registry.

PURPOSE/OBJECTIVE: The role of radiation therapy in the treatment of myoepithelial carcinoma (MC) is unknown. We present a case of a high-grade soft-tissue MC in a pediatric patient and retrospectively examine the effect of postoperative radiation on survival in patients with MC. MATERIALS AND METHODS: Our patient was treated with 4 cycles of ifosfamide, cisplatin, and etoposide followed by 3 cycles of ifosfamide vincristine and etoposide. Radiation was delivered to a total dose of 5580 cGy in 180 cGy/fraction to the surgical bed with a 2 cm margin starting after the third cycle of chemotherapy. The Surveillance, Epidemiology, and End Results (SEER) registry database was queried for cases of surgically resected MC. Retrospective analysis was performed with the endpoint of overall survival (OS). RESULTS: Two hundred thirty-four cases of MC were identified; for 62 of these cases, the grade of the tumor wasidentified. Of these 62 patients, 27 received postoperative radiation. OS was improved with adjuvant radiation therapy in patients with grade III or IV MC (P<0.01) as determined by the log-rank test. CONCLUSIONS: This analysis of SEER data showed an OS benefit with adjuvant radiation therapy in the treatment of high-grade MC. Physicians should report all cases of MC to improve clinical decision making in the treatment of this rare disease.

(125)I interstitial brachytherapy for the treatment of myoepithelial carcinoma of the oral and maxillofacial region.

PURPOSE: This study evaluated the treatment of myoepithelial carcinoma (MC) of the oral and maxillofacial region with radioactive iodine ((125)I) seed implantation. METHODS AND MATERIALS: Twenty-seven patients with MC in the oral and maxillofacial region were treated with (125)I seed implantation between March 2006 and October 2012. Thirteen of the 27 patients (8/8 patients with primary disease and 5/19 patients with recurrent disease) were treated on an adjuvant setting after resections, and the other 14 patients were treated by brachytherapy after a recurrence precluding a surgical resection for salvage. The sites of the MC were the parotid for 18 patients, oral cavity for 2 patients, and base of skull for 7 patients. Recurrence-free survival (RFS), overall survival (OS) rates, and side effects were retrospectively reviewed. RESULTS: Patients were followed for 6-105 months (median 37 months). The 3- and 5-year RFS rates were 51.9% and 46.1%, respectively. The 3- and 5-year OS rates were 68.6% and 51.5%, respectively. The OS and RFS were significantly better among the 8 patients treated upfront in comparison with the 19 patients treated for salvage at relapse. The OS was worst for the 7 patients with base of skull region disease. No severe complications were observed during followup. CONCLUSIONS: This study showed (125)I brachytherapy is a feasible and effective modality for the treatment of MC. These findings should be interpreted cautiously due to the small number of patients and the relatively short followup.

Iodine-125 brachytherapy for the treatment of a large parotid epithelial-myoepithelial carcinoma in a child.

Epithelial-myoepithelial carcinoma (EMC) in the salivary glands is rare. Tumours originating from salivary glands are also rare in children. Radical resection is the main treatment used for EMC in salivary glands. However, this surgery is commonly associated with functional and/or cosmetic deficits. There is also a high percentage of local recurrence after a tumourectomy. We present a typical case of recurrent EMC in the left parotid gland of an 8-year-old girl. The patient was treated with iodine-125 brachytherapy. At the 2-month follow-up, the tumour size was reduced by 80%, and at 1 year, no tumour tissue was detected on positron emission tomography/computed tomography. At the 6-year follow-up, no local recurrence or distant metastasis was found and no complications had occurred. Iodine-125 brachytherapy is a potentially appropriate alternative treatment for EMC in the salivary glands, especially for paediatric patients.

Primary myoepithelial carcinoma of the vulva and review of the literature.

Myoepithelial carcinoma of the vulva is extremely rare, with only five cases reported. Here, we describe a case of vulvar myoepithelial carcinoma along with a review of the literature. The patient, a 49-year-old woman, was referred for a tumor on the right labium majora. She underwent a wide local excision and bilateral inguinal lymph node dissection. Pathological examination revealed an unencapsulated, infiltrative pattern, with solid, nested and trabecular components and areas with myxoid or hyalinized stroma. The tumor consisted of oval to round epithelioid cells with moderate nuclear pleomorphism. By immunohistochemistry, the tumor cells were diffusely positive for cellular adhesion molecule (CAM) 5.2, epithelial membrane antigen (EMA), S-100 protein, and vimentin and focally positive for carcinoembryonic antigen (CEA) and p63, while negative for alpha- smooth muscle actin (SMA). The tumor was diagnosed as a myoepithelial carcinoma of the vulva, with metastases to the bilateral inguinal lymph nodes. Following completion of adjuvant radiotherapy, the patient remained alive without any evidence of recurrence at 56 months. A review of six cases of this tumor (including the present case), demonstrated variable morphology with some overlapping features. Therefore, immunohistochemistry using a panel of epithelial and myogenic markers is essential for definitive diagnosis. Two cases had inguinal lymph node metastases and received adjuvant radiotherapy or concurrent chemoradiotherapy, which resulted in good local control. One case had lung metastasis and was successfully treated by chemotherapy. Given the rarity of this disease and its uncertain prognosis, no clinical trials have been conducted regarding the necessity of adjuvant therapy. Myoepithelial carcinomas of the vulva are extremely rare making case series the most viable means of optimizing diagnosis and therapy.

Calponin expression in laryngeal myoepithelial carcinoma and its prognostic implications: a case report and literature review.

A case report of laryngeal myoepithelial carcinoma (MEC) is presented and the literature concerning prognostic factors in MEC is reviewed. A 61-year old man was admitted to hospital with hoarseness and progressive dyspnoea. On examination, both vocal cords were fixed in the midline with a glottic fissure of only 3 mm. No tumour was seen, but the subglottis was not completely visible. A computed tomography scan showed a soft mass below the right vocal cord obstructing two-thirds of the larynx. On suspension laryngoscopy, a dull mass (1.5 x 1.5 cm) was seen below the right vocal cord, which was malignant on frozen biopsy. A total laryngectomy was performed and the patient received radiotherapy. He died of recurrence 25 months later. The tumour was positive for cytokeratin 14, S-100 protein and calponin. MEC of the larynx is extremely rare. The clinical behaviour of MEC is variable and prognostic factors have been poorly analysed. Calponin expression may be a prognostic factor, but other factors also affect the outcome in MEC.

Radioresistant malignant myoepithelioma of the breast with high level of ataxia telangiectasia mutated protein.

Malignant myoepithelioma of the breast (MMB) is a rare and often aggressive disease with poor prognosis. Little is known regarding its optimal treatment and progression. We describe the clinical history of a woman following excision of a benign adenomyoepithelioma which recurred years later as a radioresistant malignant myoepithelioma with high levels of ataxia telangiectasia mutated protein and mutant p53 (Cys135Phe). MMB requires close follow-up and aggressive treatment. If adjuvant radiotherapy is adopted to improve local control, minimal postoperative delay and higher doses than for standard post-mastectomy radiation are recommended.

Primary malignant myoepithelioma of the left maxillary sinus: a case report.

We describe the case of a 41-year-old woman who presented with a malignant myoepithelioma (MME) in her left maxillary sinus. Exploratory biopsy of the left maxillary sinus was performed and pathological examination demonstrated that the tumour was positive for calponin and cytokeratin 14, which are indicative of MME. Lateral rhinotomy and left total maxillectomy were undertaken and the patient received radiotherapy and chemotherapy post-surgery. Primary recurrence and metastasis to the left angle of the mandible occurred 9 months after the surgery. The patient died of cachexia 13 months after the surgery.

[Epithelial-myoepithelial carcinoma of the submandibular gland: a case report]. / Submandibuler bezde epitelyal-miyoepitelyal karsinom: Olgu sunumu.

Epithelial-myoepithelial carcinomas are rare, low-grade, malignant salivary gland tumors. They are mostly seen in the parotid gland. The submandibular gland is a rare localization. A 54-year-old woman presented with a painless lump on the left side of the neck. Examination showed a mass with smooth surface in the left submandibular region. Fine needle aspiration cytology of the mass was reported as malignant epithelial tumor metastasis. Computed tomography of the neck showed left submandibular gland hypertrophy. Left submandibular gland excision with functional neck dissection was performed. The histopathological diagnosis was epithelial-myoepithelial carcinoma of the submandibular gland with one lymph node metastasis.

Epithelial-myoepithelial carcinoma of the submandibular gland with symptomatic lung metastases treated with chemotherapy.

This report concerns a patient with symptomatic lung metastases from an epithelial-myoepithelial carcinoma of the submandibular gland. Although the efficacy of chemotherapy is unknown in this disease, our patient was treated with cisplatin combined with 5-fluorouracil and later with paclitaxel and cyclophosphamide. Chemotherapy allowed disease stabilization and relief of the pulmonary symptoms. This is the first report on the use of chemotherapy in this very rare salivary gland carcinoma.

Radiotherapy and chemotherapy for myoepithelioma of the sellar region.

BACKGROUND: Myoepithelioma might arise in the head and neck area, especially within the salivary glands. It is very uncommon as a primary intracranial tumor. CASE REPORT: A 34-year-old African patient with proliferating myoepithelioma originating in the sellar region is described. After subtotal resection, the tumor recurred locally despite postoperative radiotherapy to a total dose of 54 Gy (Figures 1 and 2). When intracranial metastases developed, chemotherapy with ifosfamide and, later, BCNU was administered. No objective response was obtained with any of the nonsurgical approaches. Uncontrolled intracranial tumor growth led to the patient's death 20 months after the initial diagnosis. CONCLUSION: Most tumors of the sellar region have a favorable prognosis. However, this case of incompletely resected proliferating myoepithelioma showed both local and distant recurrences, which did not respond to further treatment. Thus, complete surgical resection is recommended, whenever technically feasible.