medullary carcinoma

Summary

Summary: A carcinoma composed mainly of epithelial elements with little or no stroma. Medullary carcinomas of the breast constitute 5%-7% of all mammary carcinomas; medullary carcinomas of the thyroid comprise 3%-10% of all thyroid malignancies. (From Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1141; Segen, Dictionary of Modern Medicine, 1992)

Top Publications

  1. Khomsi F, Ben Bachouche W, Bouzaiene H, Chargui R, Ben Hassouna J, Mtaalah M, et al. [Typical medullary carcinoma of the breast: a retrospective study about 33 cases]. Gynecol Obstet Fertil. 2007;35:1117-22 pubmed
    Typical medullary carcinoma of the breast is a rare histological form of breast carcinoma. It represents less than 5% of all breast cancer. It is known for its favourable prognosis...
  2. Albadine R, Wang W, Brownlee N, Toubaji A, Billis A, Argani P, et al. Topoisomerase II alpha status in renal medullary carcinoma: immuno-expression and gene copy alterations of a potential target of therapy. J Urol. 2009;182:735-40 pubmed publisher
    Renal medullary carcinoma is an aggressive renal neoplasm without currently available effective therapy to our knowledge. Topoisomerase II alpha is a gyrase involved in cell proliferation, and DNA maintenance and repair...
  3. Machens A, Ukkat J, Hauptmann S, Dralle H. Abnormal carcinoembryonic antigen levels and medullary thyroid cancer progression: a multivariate analysis. Arch Surg. 2007;142:289-93; discussion 294 pubmed
    ..Medullary thyroid cancer cells are capable of secreting carcinoembyronic antigen (CEA). An abnormal preoperative CEA level may have important implications for the management of this condition...
  4. Hakimi A, Koi P, Milhoua P, Blitman N, Li M, Hugec V, et al. Renal medullary carcinoma: the Bronx experience. Urology. 2007;70:878-82 pubmed
    Renal medullary carcinoma (RMC) is a devastating and extremely rare malignancy primarily afflicting young men with sickle cell trait...
  5. Kageyama K, Sakihara S, Yamashita M, Takahashi K, Kawashima S, Tanabe J, et al. A case of multiple endocrine neoplasia type II accompanied by thyroid medullary carcinoma and pheochromocytomas expressing corticotropin-releasing factor and urocortins. Am J Med Sci. 2008;335:398-402 pubmed publisher
    ..Pathological examination showed that these tumors were pheochromocytoma and thyroid medullary carcinoma, respectively, both of which highly expressed corticotropin-releasing factor, urocortin1, and urocortin3...
  6. Rossi E, Raffaelli M, Mule A, Zannoni G, Pontecorvi A, Santeusanio G, et al. Relevance of immunocytochemistry on thin-layer cytology in thyroid lesions suspicious for medullary carcinoma: a case-control study. Appl Immunohistochem Mol Morphol. 2008;16:548-53 pubmed publisher
    ..This diagnosis may be supported by immunocytochemistry (ICC), which may be difficult to carry out on the conventional smears...
  7. Scheuba C, Kaserer K, Moritz A, Drosten R, Vierhapper H, Bieglmayer C, et al. Sporadic hypercalcitoninemia: clinical and therapeutic consequences. Endocr Relat Cancer. 2009;16:243-53 pubmed publisher
    ..Medullary thyroid cancer was documented in 20% of patients with basal calcitonin >10 pg/ml but <64 pg/ml and stimulated calcitonin >100 pg/ml but <560 pg/ml...
  8. Winn B, Tavares R, Fanion J, Noble L, Gao J, Sabo E, et al. Differentiating the undifferentiated: immunohistochemical profile of medullary carcinoma of the colon with an emphasis on intestinal differentiation. Hum Pathol. 2009;40:398-404 pubmed publisher
    Undifferentiated or medullary carcinoma is characterized by its distinct histologic appearance and relatively better prognosis compared to poorly differentiated colonic carcinoma...
  9. Guo X, Fan Y, Lang R, Gu F, Chen L, Cui L, et al. Tumor infiltrating lymphocytes differ in invasive micropapillary carcinoma and medullary carcinoma of breast. Mod Pathol. 2008;21:1101-7 pubmed publisher
    Tumor infiltrating lymphocytes have been correlated with a better prognosis for some tumors and medullary carcinoma of breast is a good example...

More Information

Publications63

  1. Machens A, Dralle H. Genotype-phenotype based surgical concept of hereditary medullary thyroid carcinoma. World J Surg. 2007;31:957-68 pubmed
    ..A comprehensive appraisal of this body of evidence using evidence-based methodology is pending...
  2. Schaeffer E, Guzzo T, Furge K, Netto G, Westphal M, Dykema K, et al. Renal medullary carcinoma: molecular, pathological and clinical evidence for treatment with topoisomerase-inhibiting therapy. BJU Int. 2010;106:62-5 pubmed publisher
    ..Aetiology (case series) Level of Evidence 4...
  3. Herrmann B, Schmid K, Goerges R, Kemen M, Mann K. Calcitonin screening and pentagastrin testing: predictive value for the diagnosis of medullary carcinoma in nodular thyroid disease. Eur J Endocrinol. 2010;162:1141-5 pubmed publisher
    ..Serum calcitonin (hCT) measurement may be useful for detecting medullary thyroid carcinoma (MTC), but the routine use of hCT after pentagastrin stimulation to screen patients with nodular thyroid disease remains controversial...
  4. Cheung K, Roman S, Wang T, Walker H, Sosa J. Calcitonin measurement in the evaluation of thyroid nodules in the United States: a cost-effectiveness and decision analysis. J Clin Endocrinol Metab. 2008;93:2173-80 pubmed publisher
    ..Calcitonin screening for MTC has not been recommended in recent U.S. practice guidelines...
  5. D Herbomez M, Caron P, Bauters C, Do Cao C, Schlienger J, Sapin R, et al. Reference range of serum calcitonin levels in humans: influence of calcitonin assays, sex, age, and cigarette smoking. Eur J Endocrinol. 2007;157:749-55 pubmed
    ..The objective of this study was to re-evaluate the adult C(T) reference values determined by five different immunoassays and by introducing criteria for selecting control subjects...
  6. Bhatoe H, Badwal S, Dutta V, Kannan N. Pituitary metastasis from medullary carcinoma of thyroid: case report and review of literature. J Neurooncol. 2008;89:63-7 pubmed publisher
    ..We report a case of intrapituitary metastasis from medullary carcinoma thyroid in a 38-year-old male, who had been operated for pituitary adenoma 5 years earlier...
  7. Tavares M, Michaluart P, Montenegro F, Arap S, Sodre M, Takeda F, et al. Skip metastases in medullary thyroid carcinoma: a single-center experience. Surg Today. 2008;38:499-504 pubmed publisher
    ..Level II to V lateral neck dissection is performed if neck metastasis is present or suspected. We conducted this study to identify the frequency and clinical determinants of skip neck metastasis in MTC...
  8. Tominaga J, Hama H, Kimura N, Takahashi S. MR imaging of medullary carcinoma of the breast. Eur J Radiol. 2009;70:525-9 pubmed publisher
    To examine the magnetic resonance imaging (MRI) findings of medullary carcinoma of the breast and to correlate them with histopathologic features.
  9. Papapetrou P, Polymeris A, Karga H, Vaiopoulos G. Heterophilic antibodies causing falsely high serum calcitonin values. J Endocrinol Invest. 2006;29:919-23 pubmed
  10. Moore S, Appfelstaedt J, Zaahl M. Familial medullary carcinoma prevention, risk evaluation, and RET in children of families with MEN2. J Pediatr Surg. 2007;42:326-32 pubmed
    ..The 918 mutation associated with MEN2B is associated with early aggressive behaviour and distant metastatic spread. This has led to active screening of affected families underlining the need for specific intervention strategies...
  11. Cavalheiro B, Junqueira C, Brandão L. Expression of matrix metalloproteinase 2 (MMP-2) and tissue inhibitor of metalloproteinase 2 (TIMP-2) in medullary thyroid carcinoma: prognostic implications. Thyroid. 2008;18:865-71 pubmed publisher
    ..If so, their expression would be a novel indicator of the prognosis of MTC...
  12. Foschini M, Eusebi V. Rare (new) entities of the breast and medullary carcinoma. Pathology. 2009;41:48-56 pubmed publisher
    ..Here some of these tumours are described, in which the authors have most familiarity. An outline of medullary carcinoma is also presented.
  13. Correia Deur J, Toledo R, Imazawa A, Lourenço D, Ezabella M, Tavares M, et al. Sporadic medullary thyroid carcinoma: clinical data from a university hospital. Clinics (Sao Paulo). 2009;64:379-86 pubmed
    ..In this study, a set of cases with s-medullary thyroid carcinoma are documented and explored...
  14. Yalcin M, Dyskin E, Lansing L, Bharali D, Mousa S, Bridoux A, et al. Tetraiodothyroacetic acid (tetrac) and nanoparticulate tetrac arrest growth of medullary carcinoma of the thyroid. J Clin Endocrinol Metab. 2010;95:1972-80 pubmed publisher
    ..Tetrac also inhibits angiogenesis initiated by vascular endothelial growth factor and basic fibroblast growth factor...
  15. Flucke U, Flucke M, Hoy L, Breuer E, Goebbels R, Rhiem K, et al. Distinguishing medullary carcinoma of the breast from high-grade hormone receptor-negative invasive ductal carcinoma: an immunohistochemical approach. Histopathology. 2010;56:852-9 pubmed publisher
    ..Our aim was to establish novel diagnostic markers distinguishing MCs from hormone receptor-negative high-grade IDCs...
  16. Watanabe I, Billis A, Guimaraes M, Alvarenga M, de Matos A, Cardinalli I, et al. Renal medullary carcinoma: report of seven cases from Brazil. Mod Pathol. 2007;20:914-20 pubmed
    We report seven cases of renal medullary carcinoma collected from several institutions in Brazil. In spite of a relatively high incidence of sickle cell trait in Brazil, this is a rare tumor...
  17. Boikos S, Stratakis C. Molecular mechanisms of medullary thyroid carcinoma: current approaches in diagnosis and treatment. Histol Histopathol. 2008;23:109-16 pubmed publisher
    ..This report reviews the RET involvement in the etiology of MEN 2 and MTC and updates the therapeutic approach in preclinical and clinical studies...
  18. Yaday S, Singh I, Singh J, Aggarwal N. Medullary carcinoma in a lingual thyroid. Singapore Med J. 2008;49:251-3 pubmed
    ..We report a case of medullary carcinoma in a total ectopic lingual thyroid occurring in a 45-year-old woman who presented with dysphagia, plummy ..
  19. Thirunavukarasu P, Sathaiah M, Singla S, Sukumar S, Karunamurthy A, Pragatheeshwar K, et al. Medullary carcinoma of the large intestine: a population based analysis. Int J Oncol. 2010;37:901-7 pubmed
    b>Medullary carcinoma (MC) of the colorectum is a relatively new histological type of adenocarcinoma characterized by poor glandular differentiation and intraepithelial lymphocytic infiltrate...
  20. You Y, Lakhani V, Wells S, Moley J. Medullary thyroid cancer. Surg Oncol Clin N Am. 2006;15:639-60 pubmed
    ..With the advent of therapies that target the RET-activated pathways, new hope may be emerging for patients who have locally advanced or metastatic disease...
  21. Banville N, Geraghty R, Fox E, Leahy D, Green A, Keegan D, et al. Medullary carcinoma of the pancreas in a man with hereditary nonpolyposis colorectal cancer due to a mutation of the MSH2 mismatch repair gene. Hum Pathol. 2006;37:1498-502 pubmed
    ..b>Medullary carcinoma of the pancreas is a recently described rare variant of pancreatic adenocarcinoma...
  22. Dorairajan N, Siddharth D, Kanna S. Review analysis of medullary carcinoma of the thyroid: a 15-year Indian experience. Int Surg. 2006;91:162-7 pubmed
    The aim of this study was to emphasize the importance of adequate primary surgery in cases of medullary carcinoma of the thyroid...
  23. Santos M, Nunes A, Abelin N, Ezabella M, Toledo R, Lourenço D, et al. [Genetic screening of multiple endocrine neoplasia type 2: experience of the USP Endocrine Genetics Unit]. Arq Bras Endocrinol Metabol. 2006;50:7-16 pubmed
    ..In 50 typical MEN-2 patients analyzed using both methods, we found no false results suggesting that DGGE is a reliable screening method for RET proto-oncogene mutation analysis...
  24. Gulben K, Berberoglu U, Boyabatli M. Prognostic factors for sporadic medullary thyroid carcinoma. World J Surg. 2006;30:84-90 pubmed
    ..Approximately 84% of cases are sporadic. The aim of this study was to evaluate the outcomes of treatment for sporadic medullary thyroid carcinoma (SMTC) and define the prognostic factors for overall survival...
  25. Fialkowski E, Moley J. Current approaches to medullary thyroid carcinoma, sporadic and familial. J Surg Oncol. 2006;94:737-47 pubmed
    ..Newer systemic treatments, with agents that target abnormal RET proteins, hold promise and are being tested in clinical trials for patients with metastatic disease...
  26. Ito Y, Yoshida H, Nakamura Y, Tomoda C, Uruno T, Takamura Y, et al. Expression of Jun activation domain-binding protein 1 and p27 (Kip1) in thyroid medullary carcinoma. Pathology. 2005;37:216-9 pubmed
    ..In this study, we investigated the expression of these proteins in medullary thyroid carcinoma...
  27. Ito Y, Nakamura Y, Yoshida H, Tomoda C, Uruno T, Takamura Y, et al. Polo-like kinase 1 expression in medullary carcinoma of the thyroid: its relationship with clinicopathological features. Pathobiology. 2005;72:186-90 pubmed
    ..However, its protein expression in human carcinoma has not been studied in depth. In this study, we investigated PLK1 expression in medullary thyroid carcinoma by means of immunohistochemistry...
  28. Peczkowska M, Januszewicz A. Multiple endocrine neoplasia type 2. Fam Cancer. 2005;4:25-36 pubmed
    ..Introduced in recent years and still developing genetic testing of individuals at highest hereditary risk of MEN 2 syndrome holds the possibility of early detection and improved treatment and prognosis...
  29. Kuroda H, Tamaru J, Sakamoto G, Ohnisi K, Itoyama S. Immunophenotype of lymphocytic infiltration in medullary carcinoma of the breast. Virchows Arch. 2005;446:10-4 pubmed
    b>Medullary carcinoma (MC) of the breast is characterized by large anaplastic cells and infiltration by benign lymphocytes...
  30. Pelizzo M, Boschin I, Bernante P, Toniato A, Piotto A, Pagetta C, et al. Natural history, diagnosis, treatment and outcome of medullary thyroid cancer: 37 years experience on 157 patients. Eur J Surg Oncol. 2007;33:493-7 pubmed
  31. Costante G, Meringolo D, Durante C, Bianchi D, Nocera M, Tumino S, et al. Predictive value of serum calcitonin levels for preoperative diagnosis of medullary thyroid carcinoma in a cohort of 5817 consecutive patients with thyroid nodules. J Clin Endocrinol Metab. 2007;92:450-5 pubmed
    ..Routine serum calcitonin (CT) measurement in patients with thyroid nodules for diagnosis of medullary thyroid carcinoma (MTC) is controversial...
  32. Jimenez C, Dang G, Schultz P, El Naggar A, Shapiro S, Barnes E, et al. A novel point mutation of the RET protooncogene involving the second intracellular tyrosine kinase domain in a family with medullary thyroid carcinoma. J Clin Endocrinol Metab. 2004;89:3521-6 pubmed
    ..Here, we describe a novel missense mutation of the RET protooncogene that substitutes arginine for proline at codon 912 of the intracellular tyrosine kinase domain in a family with medullary thyroid carcinoma...
  33. Elisei R, Cosci B, Romei C, Bottici V, Sculli M, Lari R, et al. RET exon 11 (G691S) polymorphism is significantly more frequent in sporadic medullary thyroid carcinoma than in the general population. J Clin Endocrinol Metab. 2004;89:3579-84 pubmed
    ..A putative role of the G691S polymorphism as genetic modifier in the normal subjects remains to be established...
  34. Wick M, Vitsky J, Ritter J, Swanson P, Mills S. Sporadic medullary carcinoma of the colon: a clinicopathologic comparison with nonhereditary poorly differentiated enteric-type adenocarcinoma and neuroendocrine colorectal carcinoma. Am J Clin Pathol. 2005;123:56-65 pubmed
    ..Medullary CRC seems to be a distinct clinicopathologic variant of CRC, which does not have a neuroendocrine lineage. The biologic behavior of MCRC was better than that of ECRC or NEC...
  35. Blitman N, Berkenblit R, Rozenblit A, Levin T. Renal medullary carcinoma: CT and MRI features. AJR Am J Roentgenol. 2005;185:268-72 pubmed
    We review the cross-sectional imaging findings of six cases of pathologically proven renal medullary carcinoma in patients with sickle cell trait. MRI findings were available in three of the patients...
  36. Kouvaraki M, Shapiro S, Perrier N, Cote G, Gagel R, Hoff A, et al. RET proto-oncogene: a review and update of genotype-phenotype correlations in hereditary medullary thyroid cancer and associated endocrine tumors. Thyroid. 2005;15:531-44 pubmed
    ..Recommendations on the timing of prophylactic thyroidectomy and the extent of surgery are based upon a model that utilizes genotype- phenotype correlations to stratify mutations into three risk levels...
  37. Barbosa S, Rodien P, Leboulleux S, Niccoli Sire P, Kraimps J, Caron P, et al. Ectopic adrenocorticotropic hormone-syndrome in medullary carcinoma of the thyroid: a retrospective analysis and review of the literature. Thyroid. 2005;15:618-23 pubmed
    ..EAS is a rare complication of MTC. The prognosis is poor because of frequency of metastasis at diagnosis. Persistent hypercortisolism can, by itself, lead to death, and has to be treated specifically...
  38. Cebrian A, Lesueur F, Martin S, Leyland J, Ahmed S, Luccarini C, et al. Polymorphisms in the initiators of RET (rearranged during transfection) signaling pathway and susceptibility to sporadic medullary thyroid carcinoma. J Clin Endocrinol Metab. 2005;90:6268-74 pubmed
    ..However, most MTC occur in individuals without a family history...
  39. D Aloiso L, Carlomagno F, Bisceglia M, Anaganti S, Ferretti E, Verrienti A, et al. Clinical case seminar: in vivo and in vitro characterization of a novel germline RET mutation associated with low-penetrant nonaggressive familial medullary thyroid carcinoma. J Clin Endocrinol Metab. 2006;91:754-9 pubmed
    ..RET mutation analysis provides useful information on the clinical outcome of medullary thyroid carcinomas (MTCs) and the risk of disease in the family members...
  40. Peixoto Callejo I, Américo Brito J, Zagalo C, Rosa Santos J. Medullary thyroid carcinoma: multivariate analysis of prognostic factors influencing survival. Clin Transl Oncol. 2006;8:435-43 pubmed
    ..Medullary thyroid carcinoma (MTC) is a rare development of thyroid cancer with a no negligible mortality rate. Our aim was to determine factors that predict outcome in patients with MTC...
  41. Frank Raue K, Buhr H, Dralle H, Klar E, Senninger N, Weber T, et al. Long-term outcome in 46 gene carriers of hereditary medullary thyroid carcinoma after prophylactic thyroidectomy: impact of individual RET genotype. Eur J Endocrinol. 2006;155:229-36 pubmed
    ..Recommendations on the timing and extent of thyroidectomy are based upon a model that utilises genotype-phenotype correlations to stratify mutations into three risk groups...
  42. Bell M. Response to paclitaxel, gemcitabine, and cisplatin in renal medullary carcinoma. Pediatr Blood Cancer. 2006;47:228 pubmed
  43. Toledo S, dos Santos M, Toledo R, Lourenço D. Impact of RET proto-oncogene analysis on the clinical management of multiple endocrine neoplasia type 2. Clinics (Sao Paulo). 2006;61:59-70 pubmed
  44. Barbet J, Campion L, Kraeber Bodéré F, Chatal J. Prognostic impact of serum calcitonin and carcinoembryonic antigen doubling-times in patients with medullary thyroid carcinoma. J Clin Endocrinol Metab. 2005;90:6077-84 pubmed
    ..After unsuccessful surgery, medullary thyroid carcinoma (MTC) may be fatal or remain stable for decades, and precise survival predictors are needed...
  45. Papi G, Corsello S, Cioni K, Pizzini A, Corrado S, Carapezzi C, et al. Value of routine measurement of serum calcitonin concentrations in patients with nodular thyroid disease: A multicenter study. J Endocrinol Invest. 2006;29:427-37 pubmed
    ..The routine measurement of serum calcitonin (CT) has been proposed for patients with nodular thyroid disease (NTD), to detect unsuspected medullary thyroid carcinoma (MTC) before surgery...
  46. Simpson L, He X, Pins M, Huang X, Campbell S, Yang X, et al. Renal medullary carcinoma and ABL gene amplification. J Urol. 2005;173:1883-8 pubmed
    We characterized the clinical course of renal medullary carcinoma (RMC) and performed an expanded analysis of BCR-ABL.
  47. Shaha A, Cohen T, Ghossein R, Tuttle R. Late-onset medullary carcinoma of the thyroid: need for genetic testing and prophylactic thyroidectomy in adult family members. Laryngoscope. 2006;116:1704-7 pubmed
    Sporadic late-onset medullary carcinoma of the thyroid is quite rare...
  48. Mirallie E, Iacobone M, Sebag F, Henry J. Results of surgical treatment of sporadic medullary thyroid carcinoma following routine measurement of serum calcitonin. Eur J Surg Oncol. 2004;30:790-5 pubmed
    ..The aim of this study was to evaluate the results of the surgical management of medullary thyroid carcinoma (MTC), following the introduction of systematic calcitonin measurement in patients referred for thyroid diseases...
  49. Cuccuru G, Lanzi C, Cassinelli G, Pratesi G, Tortoreto M, Petrangolini G, et al. Cellular effects and antitumor activity of RET inhibitor RPI-1 on MEN2A-associated medullary thyroid carcinoma. J Natl Cancer Inst. 2004;96:1006-14 pubmed
    ..Medullary thyroid carcinoma is a component of multiple endocrine neoplasia type 2 or MEN2 syndromes...
  50. Roman S, Lin R, Sosa J. Prognosis of medullary thyroid carcinoma: demographic, clinical, and pathologic predictors of survival in 1252 cases. Cancer. 2006;107:2134-42 pubmed
    ..Therefore, the authors used the population-based Surveillance, Epidemiology, and End Results (SEER) registry to update what to their knowledge is one of the largest series of patients with MTC reported to date...
  51. Eichhorn J. Medullary carcinoma, provocative now as then. Semin Diagn Pathol. 2004;21:65-73 pubmed
    ..The term "medullary carcinoma" dates to the 19th century, but the modern entity was introduced in 1949 by Moore and Foote, who ..
  52. Kotlan B, Simsa P, Foldi J, Fridman W, Glassy M, McKnight M, et al. Immunoglobulin repertoire of B lymphocytes infiltrating breast medullary carcinoma. Hum Antibodies. 2003;12:113-21 pubmed
    ..This new approach might have a benefit for further antibody engineering, as difficulties in search for tumor binders by phage library selection might be reduced and the time for selection shortened...
  53. Rakha E, Aleskandarany M, El Sayed M, Blamey R, Elston C, Ellis I, et al. The prognostic significance of inflammation and medullary histological type in invasive carcinoma of the breast. Eur J Cancer. 2009;45:1780-7 pubmed publisher
    The new gene expression molecular taxonomy of breast cancer places medullary carcinoma in the basal group...
  54. Ahrens P, Kreipe H. [BRCA1- and BRCA2- associated breast carcinomas]. Pathologe. 2009;30 Suppl 2:207-9 pubmed publisher
    ..Cancers with a BRCA2 germ line mutation are less frequent and resemble sporadic cases with a comparably higher proliferative activity and a lack of Her2 overexpression...