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Mucinous cystadenocarcinoma of the lung

mucinous cystadenocarcinoma of the lung
Mucinous cystadenocarcinoma of the lung MCACL is a very rare malignant mucus-producing neoplasm arising from the uncontrolled growth of transformed epithelial cells originating in lung tissue


  • 1 Synonyms
  • 2 Classification
  • 3 Incidence
  • 4 Diagnosis and Symptoms
  • 5 Histogenesis and Genetics
  • 6 Treatment
  • 7 Prognosis and Survival
  • 8 References
  • 9 External links


Several other terms for this lesion have been used in the past medical literature, including mucinous multilocular cyst carcinoma, pseudomyxomatous pulmonary adenocarcinoma, mucinous cystic tumor of low malignant potential, and others1


According to the most recent revision 2004 of the World Health Organization WHO histological classification system for lung tumors "WHO2004", currently the most widely recognized typing scheme for pulmonary neoplasia, MCACL is considered a distinctive variant of adenocarcinoma2

Informally, some experts have included these tumors as a distinct variant among a spectrum of mucus-producing adenocarcinomas, including — in order of increasing relative extent of cellular mucus production and extracellular mucus accumulation — solid adenocarcinoma, mucoepidermoid carcinoma, mucinous bronchioloalveolar carcinoma, signet ring cell adenocarcinoma, mucinous cystadenocarcinoma, and mucinous "colloid" adenocarcinoma3


Accurate incidence statistics on MCACL are unavailable It is a very rare tumor,4 with only a few dozen cases reported in the literature to date1

In the few cases described in the literature to date, the male-to-female ratio is approximately unity, and right lung lesions occurred twice as commonly as left lung lesions Approximately 2/3 of cases have been associated with tobacco smoking1 Cases have been reported in patients as young as 295

Diagnosis and Symptomsedit

This particular variant of lung cancer is usually asymptomatic and is found after chest x-rays are taken for other reasons6 Hemoptysis is seen occasionally6 and, in some cases, distal obstruction of bronchi by blood clots or mucus plugs produces cough and/or infection1 Lesions often enlarge and progress slowly, over many years5

The 1999 World Health Organization classification system defined MCACL as a cystic adenocarcinoma with copious mucin production that, histologically, resembles the more common mucus-producing cystadenocarcinomas originating in the ovary, breast and pancreas7 The 2004 revision of the WHO classification noted that the tumors tend to be well circumscribed by a partial fibrous tissue capsule with central cystic change and copious mucin pooling2 The thin, fibrous wall circumscribing the tumor is highly characteristic of this lesion8 It can sometimes occur within a pulmonary bronchocele, and this tumor entity should be kept in mind after identification of a bronchocele with suspicious or non-prototypical imaging characteristics910

Microscopically, the neoplastic epithelial cells tend to grow along the alveolar walls, in a fashion similar to the mucinous variant of bronchioloalveolar carcinoma, a more common form of adenocarcinoma2

Hemoptysis is seen occasionally6

Positron Emission Tomography PET scanning can be of assistance in diagnosing MCACL,9 as these lesions show intense uptake, typically in the wall of the tumor6

CA 19-9 has been reported to be elevated in MCACL6

Differential diagnosis of MCACL includes secondary metastatic cystadenocarcinomatous lesions, particularly from the pancreas or ovary, mucoepidermoid carcinoma, and pulmonary mucinous bronchioloalveolar carcinoma814 The mouse monoclonal antibody 1D3, developed to detect a high molecular weight mucin found in a number of cystic malignancies of various organs, may be of use in differentiating primary mucinous cystadenocarcinoma of the lung from metastatic lung tumors due to mucinous cystic lesions of the uterus and pancreas, as well as those primary in the colon and stomach11

Histogenesis and Geneticsedit

MCACL has been noted in most cases to show areas of gradual transition wherein cells become more atypical and feature more pronounced characteristics of maignancy as one proceeds from the capsule, or outermost layers of the tumor, toward the center of the mass8 Some experts suggest that this tumor often develops after a slow progression from a relatively benign to a frankly malignant phenotype over a period of years to decades1

The putative cell of origin of this tumor is unknown Electron microscopic studies in 3 cases1213 described intracytoplasmic mucin, convoluted oval nuclei, prominent nucleoli, homogeneous euchromatin with peripheral chromatin condensation, microvilli, junctional complexes, and primitive lumen formation Their failure to identify lamellar bodies and large dense granules seemed to rule out origination from either Clara cells or Type II pneumocytes1

In a review of 66 cystic pulmonary mucinous lesions, Gao and colleagues reported that p53 expression and a Ki-67 index exceeding 20% is characteristic of MCACL1


For treatment purposes, MCACL has been traditionally considered a non-small cell lung carcinoma NSCLC Complete radical surgical resection is the treatment of choice14

There is virtually no data regarding new molecular targets or targeted therapy in the literature to date Iwasaki and co-workers failed to find mutations of the epidermal growth factor receptor EGFR or the cellular Kirsten rat sarcoma virus oncogene K-ras in one reported case

Prognosis and Survivaledit

MCACL has a much more favorable prognosis than most other forms of adenocarcinoma and most other NSCLC's914 Cases have been documented of continued growth of these lesions over a period of 10 years without symptoms or metastasis5 The overall mortality rate appears to be somewhere in the vicinity of 18% to 27%, depending on the criteria that are used to define this entity1


  1. ^ a b c d e f g h i Gao ZH, Urbanski SJ July 2005 "The spectrum of pulmonary mucinous cystic neoplasia : a clinicopathologic and immunohistochemical study of ten cases and review of literature" Am J Clin Pathol 124 1: 62–70 PMID 15923171 doi:101309/52XXR6E6U0J2JX0F 
  2. ^ a b c Travis, William D; Brambilla, Elisabeth; Muller-Hermelink, H Konrad; et al, eds 2004 Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart PDF World Health Organization Classification of Tumours Lyon: IARC Press ISBN 92-832-2418-3 Retrieved 27 March 2010 
  3. ^ Tsuta K; Ishii G; Nitadori J May 2006 "Comparison of the immunophenotypes of signet-ring cell carcinoma, solid adenocarcinoma with mucin production, and mucinous bronchioloalveolar carcinoma of the lung characterized by the presence of cytoplasmic mucin" J Pathol 209 1: 78–87 PMID 16463270 doi:101002/path1947 
  4. ^ a b Ishibashi H; Moriya T; Matsuda Y November 2003 "Pulmonary mucinous cystadenocarcinoma: report of a case and review of the literature" Ann Thorac Surg 76 5: 1738–40 PMID 14602331 doi:101016/S0003-49750300657-X 
  5. ^ a b c Sezer O; Hoffmeier A; Bettendorf O April 2006 "Mucinous cystadenocarcinoma—an extremely rare tumor in a young patient" Thorac Cardiovasc Surg 54 3: 216–7 PMID 16639689 doi:101055/s-2005-872950 
  6. ^ a b c d e Iwasaki T, Kawahara K, Nagano T, Nakagawa K March 2007 "Pulmonary mucinous cystadenocarcinoma: an extremely rare tumor presenting as a cystic lesion of the lung" Gen Thorac Cardiovasc Surg 55 3: 143–6 PMID 17447515 doi:101007/s11748-006-0089-z 
  7. ^ Travis, WD; Colby, TV; Corrin, B 1999 Histological Typing of Lung and Pleural Tumors International histological classification of tumours, no 1 3rd ed Berlin: Springer ISBN 3-540-65219-1 
  8. ^ a b c Tangthangtham A, Chonmaitri I, Tungsagunwattana S, Charupatanapongse U October 1998 "Mucinous cystadenocarcinoma of the lung" J Med Assoc Thai 81 10: 794–8 PMID 9803072 
  9. ^ a b c Raza SA, Alexakis C, Creagh M, Lawrence DR, Wood M 2009 "Primary pulmonary mucinous cystadenocarcinoma presenting as a complex bronchocele: a case report" J Med Case Reports 3: 8581 PMC 2737758  PMID 19830231 doi:104076/1752-1947-3-8581 
  10. ^ Butnor KJ, Sporn TA, Dodd LG 2001 "Fine needle aspiration cytology of mucinous cystadenocarcinoma of the lung: report of a case with radiographic and histologic correlation" Acta Cytol 45 5: 779–83 PMID 11575661 doi:101159/000328305 
  11. ^ Gangopadhyay A, Bhattacharya M, Chatterjee SK, Barlow JJ, Tsukada Y April 1985 "Immunoperoxidase localization of a high-molecular-weight mucin recognized by monoclonal antibody 1D3" Cancer Res 45 4: 1744–52 PMID 3884144 
  12. ^ Kragel PJ, Devaney KO, Meth BM, Linnoila I, Frierson HF, Travis WD October 1990 "Mucinous cystadenoma of the lung A report of two cases with immunohistochemical and ultrastructural analysis" Arch Pathol Lab Med 114 10: 1053–6 PMID 1699507 
  13. ^ Dixon AY, Moran JF, Wesselius LJ, McGregor DH July 1993 "Pulmonary mucinous cystic tumor Case report with review of the literature" Am J Surg Pathol 17 7: 722–8 PMID 8317612 doi:101097/00000478-199307000-00010 
  14. ^ a b Gaeta M, Blandino A, Scribano E, Ascenti G, Minutoli F, Pandolfo I 1999 "Mucinous cystadenocarcinoma of the lung: CT-pathologic correlation in three cases" J Comput Assist Tomogr 23 4: 641–3 PMID 10433300 doi:101097/00004728-199907000-00028 

External linksedit

  • 1 World Health Organization Histological Typing of Lung and Pleural Tumors, 4th edition

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