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Mucoepidermoid carcinoma (MEC) is the most common primary salivary gland-type tumour of the lung. It arises from the excretory ducts of the bronchial mucosa.

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This rare tumor is more common in adults than in children, and infrequently presents with hemoptysis. 

In children and adolescents, primary neoplasms of the tracheobronchial tree and lungs are rare, with most tumors involving the respiratory system being metastatic, small, blue cell tumors of childhood. Of the primary pulmonary neoplasms, most are malignant with mucoepidermoid carcinoma representing about 10% of these malignant tumors. Mucoepidermoid tumours of the tracheobronchial tree carry a more favorable prognosis in children than adults. The tumour can be successfully managed by surgical intervention alone in children and adolescents.

Recurrent pneumonia and persistent cough are the most common presenting findings in children . These tumours are of low-grade malignant potential but they can become locally invasive, extending into cartilage and surrounding soft tissue. 

Mucoepidermoid carcinoma of the tracheobronchial tree, usually located in a segmental bronchus, appears at CT as a smoothly oval or lobulated airway mass. It adapts to the branching features of the airways.

The tumour usually presents as a polypoid endobronchial mass involving the proximal bronchi. High-grade mucoepidermoid carcinoma of the lung may present as a cavitary lesion.

It is classified into low- and high-grade tumours using criteria derived from similar tumours of the major salivary glands.

Low-grade MEC behaves in a benign fashion with less parenchymal and hilar lymph nodal invasion.

Note : Mucoepidermoid carcinoma of the bronchus can be recognized on histology as well as cytology by the presence of three characteristic cell types: mucus secreting, epidermoid and intermediate.

Image of Mucoepidermoid carcinoma of the parotid gland

Histologically, the tumour displays an elaborate tubulocystic epithelial component. It is composed of intermediate, sheets of cells with epidermoid differentiation, and mucus-producing cells containing intra and extracellular mucin, and variable numbers of clear cells, multinucleated giant cells, columnar cells, and oncocytic cells.

Low grade tumour : Marked cellular atypia is absent in the solid or epidermoid component.

High grade tumour: There is marked cellular atypia , prominent mitotic activity, areas of necrosis and hemorrhage. (Differential diagnosis : Poorly differentiated squamous cell carcinoma). PAS and mucicarmine may be useful in identifying scattered mucocytes in poorly differentiated tumours.

In some cases dense lymphoplasmacytic infiltrate with occasional Russell bodies may be present.( Differential diagnosis :  low-grade lymphoma.)

                 

Prognosis of mucoepidermoid carcinoma of the bronchi.Rev Pneumol Clin. 2007 Feb;63(1):29-34.

BACKGROUND: Mucoepidermoid cancer is exceptional in the respiratory tract, accounting for only 0.2% of primary lung cancers. CASE REPORTS: We report three cases of mucoepidermoid carcinoma. The inaugural signs were hemoptysia in a 10-year-old child, recurrent lower respiratory tract infections in a 13-year-old child, and dyspnea with chest pain in a 32-year-old adult. Bronchial fibroscopy disclosed a proximal endobronchial tumor in all three patients. Pathology study of the operative specimen identified low-grade malignant mucoepidermoid carcinoma in the two children and high-grade malignant mucoepidermoid carcinoma in the adult. Surgical resection was performed for the pediatric cases. Outcome was favorable with recurrence-free survival at eight years in the first child. The surgical resection was less radical in the second child due to locoregional extension. This child was lost to follow-up. The clinical course was rapidly fatal in the third patient who presented metastatic spread at diagnosis and died one month later.DISCUSSION: The prognosis of mucoepidermoid tumors of the bronchi is closely related to tumor grade and extension at diagnosis. Unlike high-grade mucoepidermoid carcinoma, the progression of low-grade tumors, which predominate in children, is generally slow, enabling good prognosis if diagnosis is established early. Early search for these tumors in patients presenting chronic or recurrent respiratory manifestations would avoid late diagnosis and improve prognosis.

Pulmonary mucoepidermoid carcinoma with prominent tumor-associated lymphoid proliferation.Am J Surg Pathol. 2005 Mar;29(3):407-11.

We report 6 cases of low-grade pulmonary mucoepidermoid carcinoma displaying a striking lymphoplasmacytic infiltrate. All six tumors had a typical pulmonary mucoepidermoid carcinoma presentation as a polypoid endobronchial mass involving the proximal bronchi. The patients were 3 females and 3 males with a mean age of 33 years (range, 5-61 years). Half of the patients were asymptomatic, while half experienced mild symptoms of pneumonia, asthma-like symptoms, or hemoptysis. No tumor-related deaths were observed, with a mean follow-up of 51 months. The tumor size ranged from 2.1 to 3.4 cm (mean, 2.9 cm). The tumors characteristically displayed an elaborate tubulocystic epithelial component composed of intermediate, epidermoid, and mucus-producing cells, and variable numbers of clear cells, multinucleated giant cells, columnar cells, and oncocytic cells. The tumors' lymphoplasmacytic infiltrate with occasional Russell bodies was sufficiently intense to raise concern of a low-grade lymphoma. All tested tumors were immunoreactive with CK7 while nonreactive with TTF-1 and CK20. Recognition of this histologic variant is important for a correct diagnosis of low-grade pulmonary mucoepidermoid carcinoma. The dense lymphoplasmacytic infiltrate is similar to that previously described in salivary glands as tumor-associated lymphoid proliferation.

Mucoepidermal carcinoma of the lung detected by positron emission tomography in a 5-year-old girl.J Pediatr Surg. 2005 Apr;40(4):E1-3.

The authors describe a rare case of mucoepidermal carcinoma of the lung incidentally identified in preoperative assessments for inguinal hernia repair in a 5-year-old girl. This patient was referred for right external inguinal hernia, and a 3.0-cm round-shaped lesion was found in the right lower lung field of a chest x-ray film. She had no respiratory tract complaints, but her serum carcinoembryonic antigen concentration was markedly elevated (21.2 ng/mL). Chest and abdominal computed tomography/magnetic resonance images could not determine the nature of the lesion, but 2-[18 F]fluoro-2-deoxy- d -glucose positron emission tomography (FDG-PET) indicated a malignant tumor pattern. The patient underwent a computed tomography-guided needle biopsy of the lesion (S8), which was soon followed by a right lower pulmonary lobectomy. Histopathology of the resected specimen showed mucoepidermal carcinoma with no regional lymph node metastasis. In childhood asymptomatic pulmonary lesions, it is often difficult to rule out the possibility of malignancy. In the present case, FDG-PET scanning appropriately indicated the therapeutic priority of pediatric thoracic surgery.

Mucoepidermoid carcinoma as an unusual cause for recurrent respiratory infections in a child. J Pediatr Hematol Oncol. 2005 Mar;27(3):162-5.

Recurrent respiratory infections in a 9-year-old girl prompted a chest radiograph and a CT scan, which showed a right middle lobe consolidation. Bronchoscopy revealed a tumor that totally obstructed the middle lobe. Open lung biopsy revealed a low-grade mucoepidermoid carcinoma. Middle and lower right lung lobectomy was performed, followed by an uneventful recovery. Cytogenetic investigation of tumor cells exhibited the translocation t(11;19). This case shows that further diagnostic modalities such as CT scanning should be performed early in children with recurrent lower respiratory tract infections who have suspicious radiographic findings such as persistent atelectasis or recurrent unifocal infiltration. Bronchial mucoepidermoid carcinoma is infrequent, and molecular investigations might shed additional light on the prognosis.

Bronchoscopic Nd-YAG laser surgery for tracheobronchial muco- epidermoid carcinoma--a report of two cases. Int J Clin Pract. 2004 Oct;58(10):979-82.

Mucoepidermoid carcinoma (MEC) of the tracheobronchial tree represents 0.2% of all lung tumours. It arises from the excretory ducts of the bronchial mucosa and is classified into low- and high-grade tumours using criteria derived from similar tumours of the major salivary glands. Low-grade MEC behaves in a benign fashion with less parenchymal and hilar lymph nodal invasion. The traditional method of treatment is by thoracotomy. The bronchoscopic approach to this lesion using lasers has rarely been reported. This article reports two cases of low-grade tracheobronchial MEC, which were both managed through bronchoscopic neodymium yttrium aluminium garnet (Nd-YAG) laser surgery. The patients were free from disease, 26 and 36 months after surgery. Bronchoscopic laser surgery promises to be an effective alternative treatment modality for tracheobronchial MEC. It is minimally invasive, results in less hospital stay and does not impair pulmonary functions.

Mucoepidermoid carcinoma of the lung presenting as a cavitary lesion.J Med Assoc Thai. 2004 Aug;87(8):988-91.

The authors describe a 62-year-old female patient who presented with a progressively enlarging cavitary lesion in the right upper lobe of the lung. Acid-fast bacilli were recovered from a bronchial washing fluid and identified as Mycobacterium tuberculosis. She received antituberculous therapy for 5 months without improvement in her clinical symptoms and chest radiograph. A lobectomy was performed and pathological review demonstrated a high-grade mucoepidermoid lung carcinoma with extensive central necrosis. Staging revealed metastases in her left adrenal gland, kidney and spine. High-grade mucoepidermoid carcinoma of the lung may present as a cavitary lesion. The presence of M. tuberculosis should not preclude clinicians from pursuing adequate diagnostic procedures for a possible malignant lesion.

Mucoepidermoid carcinoma of the bronchus presenting with a negative chest X-ray and normal pulmonary function in two teenagers: two case reports and review of the literature.Pediatr Pulmonol. 2004 Jan;37(1):81-4.

Two adolescents presented with a history of dyspnea upon exertion and cough. In both cases, the chest X-ray and pulmonary function testing, including flow-volume loop, were normal. A bronchial tumor was diagnosed by CT scan, which was ordered after each patient had an episode of hemoptysis. The sedimentation rate was the only abnormal laboratory test in both cases. Mucoepidermoid carcinoma of the bronchus, a rare tumor in childhood, was found at pathology in both cases. There was no evidence of metastases to local lymph nodes or distal sites. There were 47 previously reported cases in children. Recurrent pneumonia and persistent cough were the most common presenting findings. These tumors are of low-grade malignant potential but they can become locally invasive, extending into cartilage and surrounding soft tissue. Prognosis is good with complete resection.

Intranuclear inclusions in fine needle aspirates of bronchial low grade mucoepidermoid carcinoma with clear cell change: a report of two cases. Acta Cytol. 2002 Jan-Feb;46(1):57-60.

BACKGROUND: Mucoepidermoid carcinoma of the bronchus is a rare neoplasm that can be recognized on histology as well as cytology by the presence of three characteristic cell types: mucus secreting, epidermoid and intermediate. We encountered two cases displaying unusual cytologic features, including clear intranuclear inclusions. CASES: Two females, aged 33 and 39, presented with an intrabronchial tumor and pulmonary parenchymatous mass, respectively. Fine needle aspiration of both tumors showed similar cytologic features, with a dominant population of cells with bland nuclei and wide cytoplasm, and frequent intranuclear inclusions. A minor component of mucus-secreting cells was also recognized. Histologically, both tumors corresponded to the clear cell variant of mucoepidermoid carcinoma. CONCLUSION: The cytologic picture in our cases has not been described previously in fine needle aspirates of mucoepidermoid carcinoma, in neither the bronchus nor salivary gland. The differential diagnosis of a monotonous population of epithelial cells with intranuclear inclusions involves bronchioloalveolar carcinoma, but the absence of the characteristic sheet pattern, as well as the clinical and image findings, excludes this possibility. The lack of atypia and intrabronchial location limits the scope to carcinoid and salivary gland-type tumors of the bronchus. Since we were aware of the possibility of unusual cytologic presentations of mucoepidermoid carcinomas, search for different cellular populations suggested the precise diagnosis.

Three cases of pulmonary mucoepidermoid carcinoma.Kyobu Geka. 2000 Jul;53(8 Suppl):702-5.

Three cases of pulmonary mucoepidermoid carcinoma, which were surgically treated in our hospital, were reported. The first case was 28-year-old male, who had hemoptysis, underwent right upper lobectomy and mediastinal lymph node dissection on October 31, 1973. The tumor located and obstructed the ostium of right B2. The diameter was 2.3 x 1.8 cm. The second case was 63-year-old male, who had obstructive pneumonia, underwent left upper lobectomy and mediastinal lymph node dissection on November 18, 1998. The tumor located and obstructed the ostium of lingular bronchus, and the diameter was 1.8 x 1.2 cm. The third case was 25-year-old male, who had obstructive pneumonia, underwent left sleeve lower lobectomy and mediastinal lymph node dissection on May 26, 1999. The tumor located in ostium of left B6, and it obstructed lower bronchus and expanded into the lung. The diameter was 4 x 2 cm. All cases were diagnosed as low grade malignancy with no lymph node metastasis. The first case survived without recurrence at least 5 years as far as we followed, and the others are surviving until now without any signs of recurrence. These three cases were 0.19% of total resected lung cancers in our hospital from 1969 to 1999.

Cytology of primary pulmonary mucoepidermoid and adenoid cystic carcinoma. A report of four cases.Acta Cytol. 1999 Nov-Dec;43(6):1091-7.

BACKGROUND: Mucoepidermoid and adenoid cystic carcinomas are very rare primary pulmonary neoplasms that can be classified under the broader heading of salivary gland-like neoplasms (SGN). Both entities need to be considered in the cytologic differential diagnosis of lung tumors. We reviewed cytologic findings in primary pulmonary neoplasms diagnosed at our institution during the time period 1981 to the present along with outside consultation cases. CASES: Three cases of primary mucoepidermoid carcinoma and one case of primary adenoid cystic carcinoma of the lung were diagnosed based on cytology during the period examined. Patient ages were 16, 25, 47 and 78 years, respectively. The mucoepidermoid cytology specimens were composed of three cell types, mucinous, squamous and intermediate cells, at times associated with extracellular mucin. The adenoid cystic carcinoma consisted of small, uniform cells with dark nuclei, scant cytoplasm and associated, acellular balls of basement membrane material. CONCLUSION: The differential diagnosis for primary pulmonary neoplasms needs to include the rare SGN. Cytologic features of adenoid cystic carcinoma are diagnostic; those of mucoepidermoid carcinoma are at least suggestive.

Mucoepidermoid carcinoma of the tracheobronchial tree: radiographic and CT findings in 12 patients.Radiology. 1999 Sep;212(3):643-8.

PURPOSE: To determine the radiographic and computed tomographic (CT) findings and clinical features of mucoepidermoid carcinoma of the tracheobronchial tree. MATERIALS AND METHODS: Chest radiographic and CT findings and clinical features of 12 histopathologically proved mucoepidermoid carcinomas in 12 consecutive patients (five male, seven female; age range, 9-72 years; mean age, 36 years) were reviewed retrospectively. RESULTS: The tumors were located at the distal trachea (n = 1) or at a main (n = 2), lobar (n = 1), or segmental (n = 8) bronchus. On chest radiographs, the tumors appeared as central masses with post-obstructive pneumonia or peripheral atelectasis in four patients and as solitary pulmonary or endotracheobronchial nodules in eight. At CT, the tumors were all smoothly oval (n = 6) or lobulated (n = 6) in shape (ranging 9-40 mm in diameter), adapting to the branching features of the airways. Punctate calcification within the tumor was seen in six patients. Neither metastasis nor recurrence was seen after the surgical resection (follow-up of 8-103 months; mean, 30 months). CONCLUSION: Mucoepidermoid carcinoma of the tracheobronchial tree, usually located in a segmental bronchus, appears at CT as a smoothly oval or lobulated airway mass. It adapts to the branching features of the airways.

Tracheobronchial mucoepidermoid carcinoma in childhood and adolescence: case report and review of the literature.Int J Pediatr Otorhinolaryngol. 1998 Oct 15;45(3):265-73.

In children and adolescents, primary neoplasms of the tracheobronchial tree and lungs are rare, with most tumors involving the respiratory system being metastatic, small, blue cell tumors of childhood. Of the primary pulmonary neoplasms, most are malignant with mucoepidermoid carcinoma representing about 10% of these malignant tumors. We present an 8-year-old Hispanic male with hemoptysis and several episodes of pneumonia which initially was thought to be infectious upon biopsy during bronchoscopy, but proved to be mucoepidermoid carcinoma of the tracheobronchial tree by microscopic examination during an open lung biopsy. This rare tumor is more common in adults than in children, and infrequently presents with hemoptysis. Mucoepidermoid tumors of the tracheobronchial tree carry a more favorable prognosis in children than adults. In the adult population, the overall mortality is slightly less than 30%. In contrast, of the 31 reported cases of tracheobronchial mucoepidermoid carcinoma in pediatrics, all children are free of tumor involvement with a mean follow-up period of 5.8 years (range, 0.7-21 years). Based upon the available clinical outcome and survival data, it would appear that tracheobronchial mucoepidermoid carcinoma may be successfully managed by surgical intervention alone in children and adolescents.

A child with a t(11;19)(q14-21;p12) in a pulmonary mucoepidermoid carcinoma.Virchows Arch. 1998 Dec;433(6):579-81.

We report on a mucoepidermoid carcinoma (MEC) of the lung in a 6-year-old girl with a t(11;19)(q14-21;p12) as the sole karyotypic abnormality. An apparently identical t(11;19) has been reported previously in a MEC originating from the major and minor salivary glands. Our findings indicate that the t(11;19) is intimately associated with the mucoepidermoid phenotype and may be used as a diagnostic marker for this tumour type.

An experience with surgical treatment for mucoepidermoid carcinoma of the lungs.Kyobu Geka. 1993 Nov;46(12):1077-9.

Mucoepidermoid carcinoma (MEC) of the lungs is thought to arise in the bronchial glands. It is a tumor that rarely develops and it has a low grade of malignancy. In this paper, we describe one case of infiltrative MEC, which we were able to diagnose preoperatively. Surgery revealed a high grade malignancy which is reported here with a discussion based on the related literature. The patient was a 63-year-old male who was referred to our hospital by another physician due to a cough and left chest pain. A simple chest X-ray revealed a tumor shadow and a fascicular shadow on its periphery in the upper left lobe. Bronchoscopy disclosed complete circumferential stenosis at B1+2,3 and reddening from this region to the main bronchus, but it was impossible to directly confirm the tumor. Pulmonary arterography did not depict the left upper pulmonary vein, but obstruction due to a tumor of that vein was observed. Given the above findings, under a diagnosis of infiltrative MEC, a left total lobectomy accompanied by a combined left atriectomy was performed. Although most cases of MEC have a low grade malignancy, there have been some reported cases with a very high grade of malignancy. Therefore, evaluation of the progress of this type of carcinoma by preoperative diagnosis as well as radical excision appropriate to lung cancer are considered to be important.

Peripheral low-grade mucoepidermoid carcinoma of the lung--needle aspiration cytodiagnosis and histology.Cytopathology. 1992;3(4):259-65.

Mucoepidermoid carcinoma of the lung is a rare tumour, and is not usually considered in the differential diagnosis of a peripheral lung mass. The cytological and histological features of an intimate admixture of polygonal intermediate cells, well differentiated mucinous and squamous cells, as illustrated in this case report, serve to differentiate a well differentiated mucoepidermoid carcinoma from adenosquamous carcinoma, low grade adenocarcinoma, bronchioloalveolar carcinoma, and benign reactive changes.

Childhood tracheobronchial mucoepidermoid carcinoma: a case report and review of the literature.J Pediatr Surg. 1988 Apr;23(4):367-70.

Mucoepidermoid carcinoma of the lung presenting in childhood is an uncommon neoplasm. Symptoms of progressive bronchial obstruction proceed the recognition of this lesion. The well-confined local growth and minimal metastatic potential of childhood mucoepidermoid carcinoma make local resection with maximum conservation of pulmonary parenchyma the recommended operative treatment. Adequate surgical resection precludes the need for further radiation therapy or chemotherapy. This report details the presentation and treatment of a child with this rare tumor and reviews the present literature experience with childhood mucoepidermoid carcinoma.

Combined epidermoid and adenocarcinoma in diffuse interstitial pulmonary fibrosis.Hum Pathol. 1982 Jun;13(6):580-3.

This report documents a case of combined epidermoid and adenocarcinoma with idiopathic diffuse interstitial fibrosis developing in the lung. This type of carcinoma with diffuse interstitial fibrosis occurs only rarely in the lungs, in contrast to such carcinoma without fibrosis, which occurs less rarely. A 79-year-old man died of respiratory insufficiency three years after the was diagnosed as having diffuse interstitial pulmonary fibrosis. Six months prior to his death. a tumor shadow was noticed on a radiograph of his chest. Postmortem examination revealed diffuse interstitial pulmonary fibrosis and a primary lung tumor situated peripherally in the right lower lobe. The histologic features of the tumor closely resembled those of mucoepidermoid carcinoma of the major bronchi. However, the tumor had no relation to any bronchi or bronchial glands, and it was evident that no relation to any bronchi or bronchial glands, and it was evident that it had originated from the surface epithelium of the abnormally altered distal airspaces of the honeycomb lung. It is suggested that the malignantly transformed cells originally possessed the potential for bidirectional differentiation to epidermoid and mucous cells.

Carcinoid and mucoepidermoid carcinoma of bronchus in children.Ann Otol Rhinol Laryngol. 1980 Sep-Oct;89(5 Pt 1):425-7.

Four patients with bronchial carcinoid and two patients with mucoepidermoid carcinoma of the bronchus are presented. Presenting symptoms of these rare childhood tumors are chronic cough, hemoptysis, repeated bouts of pulmonary infection, and chest pain. Bronchoscopic examination should be considered in patients with these symptoms and is highly reliable in diagnosing these tumors Surgical resection is the treatment of choice. Bronchial carcinoid and mucoepidermoid carcinoma have an excellent prognosis following conservative surgical resection.

Mucoepidermoid carcinoma of the bronchus: an electron microscopic study of the low grade and the high grade variants.Cancer. 1979 May;43(5):1720-33.

Two cases of mucoepidermoid carcinoma of the bronchus--one a low grade tumor and the other a high grade tumor--are presented with findings by light and electron microscopy. This represents the first report of the ultrastructure of mucoepidermoid carcinoma of the bronchus and demonstrates the ultrastructural similarities between the low grade exophytic tumor confined to the bronchus and the high grade infiltrating tumor with lymph node and pulmonary metastases. The ultrastructural features are similar to those described for mucoepidermoid carcinoma of the salivary gland and are consistent with the proposed origin of the tumor from the submucosal bronchial gland duct. These two cases and a review of previously reported cases indicate that, analogous to mucoepidermoid carcinoma of the salivary glands, mucoepidermoid carcinoma of the bronchus may occur as either a low grade or high grade variant which can be identified on the basis of growth characteristics and histologic features.

Tracheobronchial mucoepidermoid carcinoma. Clinicopathological features and results of treatment.J Thorac Cardiovasc Surg. 1978 Oct;76(4):431-8.

Mucoepidermoid carcinomas of the tracheobronchial tree are extremely uncommon and, as a result, opinions regarding their natural history are conflicting. In an effort to determine whether the tumors are aggressive or relatively benign, we have collected seven well-documented, previously unreported cases from among 4,250 primary pulmonary carcinomas and 116 bronchial adenomas. The two tracheal and five endobronchial lesions presented here include one high-grade and six low-grade tumors. Curative resections were performed, including segmental tracheal resections in two patients, lobectomy in three patients, and pneumonectomy in two patients, and the follow-up is complete to the time of this report. Long-term survivals ranging from 5 to 23 years, averaging 12.8 years, have been achieved in the six patients with a low-grade carcinoma. The one high-grade variant proved fatal within 28 months of diagnosis despite two surgical attempts at control and radiotherapy. It is concluded that these tumors exhibit a spectrum of virulence with low-grade lesions amenable to long-term surgical cure. The optimum treatment of high-grade lesions remains problematical.

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