Cancer, Mucinous Breast Carcinoma


Article Author:
Faten Limaiem


Article Editor:
Faran Ahmad


Editors In Chief:
Tod Aeby


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
2/9/2019 8:18:50 PM

Introduction

Mucinous carcinoma of the breast is a relatively rare subtype of breast cancer accounting for about 2% of all breast carcinomas [1]. According to the latest WHO classification of tumors of the breast, mucinous carcinoma is classified as a special type of breast cancer. Based on the cellularity of the tumor, mucinous carcinomas are divided into two subtypes:

  • The pure type mucinous carcinoma.
  • The mixed type mucinous carcinoma.

The pure type consists exclusively of tumor tissue with extracellular mucin production in over 90% of the tumor, while the mixed form also contains an infiltrating ductal epithelial component without mucin [2]. Mucinous breast cancer carries a favorable prognosis with a low recurrence rate and a low incidence of lymph node metastasis. The definitive diagnosis and classification of mucinous breast carcinomas are mainly based on histopathological examination.

Etiology

The origin of mucinous breast cancer is multifactorial and involves diet, reproductive factors and hormones.

Epidemiology

Mucinous breast carcinoma prevails in postmenopausal women with a median age of 70 years [3]. Pure mucinous carcinoma is rarely encountered in young patients under 35 years (1%)[2]

Pathophysiology

Transcriptomic studies have demonstrated that mucinous tumors are of luminal A molecular subtype [4]. The transcriptomic features of mucinous A are distinct from those of mucinous B tumors, the latter showing a pattern of gene expression that is similar to that of neuroendocrine carcinomas [4]. Pure mucinous carcinomas harbor a low level of genetic instability and rare recurrent amplifications, and the genomic profiles of the tumor components of mixed mucinous tumors are remarkably similar to those of pure mucinous carcinomas. More than 90% of pure mucinous carcinomas are diploid whereas only 42% of mixed mucinous carcinoma are diploid [5].

Histopathology

Macroscopic findings:

Gross examination of mucinous carcinoma of the breast shows a glistening gelatinous lesion with pushing margins and a soft consistency that is readily recognizable. The tumors range in size from less than 1 cm to more than 20 cm (mean = 3 cm).

Microscopic findings:

Histologically, mucinous carcinoma is characterized by nests of cells floating in lakes of mucin partitioned by delicate fibrous septae containing capillary blood vessels. The cell clusters are variable in shape and size with an occasional tubular arrangement. Nuclear atypia is generally low in classic mucinous carcinoma, but in rare cases, atypia and mitoses may prevail [6]. A micropapillary or cribriform intraepithelial component is rarely seen. Mucinous carcinoma presenting with large cell clusters, reported by Capella as hypercellular or type B mucinous carcinoma, shows frequent neuroendocrine differentiation [7]. Type A mucinous carcinoma with larger quantities of extra-cellular mucin represents the classic non-endocrine variety. Pure and mixed variants have been described [8]. The most common admixture is with invasive carcinoma of no special type. A pure tumor must be composed of more than 90% mucinous carcinoma [6]. The in situ component may have a papillary, micropapillary, or cribriform pattern. In some cases, the in situ carcinoma may exhibit prominent luminal mucin production. Neuroendocrine differentiation can be identified in some cases using immunohistochemistry and special stains (Grimelius). 

Immunohistochemistry:

Typically, mucinous carcinoma is positive for estrogen and progesterone receptors [9], while androgen receptors are expressed at a low level and HER2 is not amplified [10]. Pure and mixed mucinous carcinomas are reported to express WT [11]. Mucinous breast carcinoma expresses predominantly MUC2 and MUC6 among the family of MUC genes [12].

History and Physical

Mucinous carcinoma usually grows slowly and can reach a large size at the time of diagnosis. This can be explained by the fact that the mucinous content of the tumor does not feel firm or solid upon examination [13]. Most patients with mucinous breast carcinoma present with a palpable breast mass. Fixation to skin or chest wall is rare but is reported in large lesions. With widespread screening, a large proportion of patients are diagnosed because of a mammographic abnormality [14].

Evaluation

Mammography:

On mammography, circumscribed and lobulated breast lesions are common findings for a mucinous carcinoma. A circumscribed margin would favor the diagnosis of a pure mucinous carcinoma [15].

Ultrasonography:

On ultrasonography, mucinous carcinoma is usually a complex mass with cystic and solid components micro-lobulation, vascularity, and distal enhancement. The histologic subtype of mucinous carcinoma can be predicted based on the echogenicity. Isoechoic masses are found in pure mucinous carcinoma, whereas hypoechoic masses are found in mixed mucinous carcinoma [16].

Magnetic resonance imaging:

On an MRI, mucinous carcinoma has a lobular shape, rim or heterogeneous enhancement, a persistent pattern on the time-intensity curve, and homogeneous strongly high signal intensity on T2-weighted images [17] [15]

Tissue biopsy:

Tissue biopsy is mandatory in the evaluation of mucinous breast carcinoma. Tissue specimen can be obtained by core biopsy (Trucut), fine needle aspiration cytology and incisional or excisional biopsy.

Treatment / Management

The treatment of mucinous breast carcinoma is based on surgery associated with post-operative hormone therapy in hormone responsive(ER/PR) tumors.

A recent study recommended axillary staging by sentinel lymph node biopsy and administration of adjuvant radiotherapy and hormone therapy after breast-conservation for mucinous carcinoma [9][18].

Since most mucinous carcinomas are negative for the protein HER2/neu, they are not treated with trastuzumab. Trastuzumab is approved for both the treatment of advanced breast cancer and as adjuvant therapy for early-stage HER2- positive tumors [19].

Some authors have stated that adjuvant chemotherapy can be omitted in cases with favorable risk factors [20].

Differential Diagnosis

Histological differential diagnosis:

Mucocele-like-lesion

Metastatic mucinous carcinoma

Radiological differential diagnosis:

Myxomatous fibroadenoma

Treatment Planning

Following factors may impact treatment decisions:

Tumor Biology: Since mucinous carcinoma is more prevalent in the older population, hence estrogen(ER) and progesterone receptors(PR) are expressed more often than in younger women. Similarly, there are lower rates of HER-2 overexpression. This impacts the selection of appropriate therapy. 

General Health status: Higher prevalence of comorbidity, frailty and cognitive decline in elderly patients are considered limiting factors when selecting treatment. 

Functional Status: This refers to the ability to perform routine daily tasks. Presence of functional limitation is independently associated with worse life expectancy and hence determines the overall survival of the patients. Comprehensive Geriatric Assessment (GCA) can be utilized to evaluate elderly patients to optimize mucinous cancer treatment. 

Staging

Staging of mucinous breast carcinoma is determined clinically by physical examination and imaging studies. The stage is determined pathologically by histological examination of the tumor and lymph nodes after surgery. The TNM classification system is used to classify mucinous breast carcinoma based on :

  • Primary tumor size (T), 
  • Regional lymph nodes status (N),
  • and Distant metastasis if there are any (M).

The system that is most recently used is that of the American Joint Committee on Cancer (8th edition).

Prognosis

Mucinous carcinoma has a favorable prognosis and infrequent lymphatic metastasis compared to invasive ductal carcinoma. Axillary lymph node metastases occur in 12-14% of the cases [19]. Pure mucinous carcinoma has a better prognosis than mixed mucinous breast carcinoma [3].  The 5-year disease-free survival rates range from 81% to 94% (the latter if lymph nodes are negative). Late distant metastases may occur in pure mucinous carcinoma [21]. In a  multivariate analysis of 11,422 patients with pure mucinous carcinoma, tumor size was found to be an independent prognostic indicator but less significant than nodal status [2]. Other studies have found that tumor size is not a significant prognostic factor and does not affect survival since most tumor volume consists of mucin[13].

Complications

Complications of breast mucinous carcinoma include:

  • Axillary lymph node metastases
  • Late distant metastases
  • Recurrence after surgical excision

Consultations

Surgical oncology

Oncology

Deterrence and Patient Education

Who should be screened? It is important for women at age 40 and over to talk to a doctor, discuss the benefits and drawbacks of breast cancer screening and decide when to get screening. Women 40 to 74 years or some older healthy women may be offered a screening test with the mammogram. Some women who are at high risk (BRCA gene or first-degree relatives with breast cancer) may need to begin screening at a younger age.

What if my mammogram report is abnormal? 9 out of 10 women with an abnormal mammogram turn out not to have breast cancer. Do not panic. You will need more tests to find out what is really going on. 

How frequent should mammogram be done? Many experts suggest screening every 2 years with a screening mammogram. 

 

Enhancing Healthcare Team Outcomes

Management of mucinous breast carcinoma needs a multidisciplinary approach involving a team that consists of a surgical oncologist, an oncologist, a pathologist, and a radiologist. After treatment of mucinous breast carcinoma, long-term follow up is necessary to detect local and distant relapse. 


  • Image 8503 Not availableImage 8503 Not available
    Image courtesy S Bhimji MD
Attributed To: Image courtesy S Bhimji MD

Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Cancer, Mucinous Breast Carcinoma - Questions

Take a quiz of the questions on this article.

Take Quiz
Which of the following breast malignancies have a gelatinous consistency on palpation?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following is true about mucinous carcinoma?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which type of breast cancer has the best prognosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
63 year old patient presented with palpable mass in her right breast. Diagnostic mammogram showed a spiculated mass measuring 1.7 cm. Ultrasound demonstrated a hypoechoic mass measuring 1.8 cm x 1.1 cm x 0.9 cm. Biopsy revealed a well differentiated mucinous carcinoma that was ER/PR positive and HER2/neu negative. Lumpectomy and sentinel node biopsy revealed 1.8 cm mucinous carcinoma with widely negative margins. No associated ductal carcinoma in situ or lymphovascular invasion was noted. Two sentinel nodes were negative. Oncotype score was 3. What is the treatment recommendation?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 55-year old female presents with a 3 cm mass in her right breast. She noticed the mass a few days ago while showering. She is a smoker but has no major medical problems. She did have mild depression in the past, which was treated with psychological therapy. She undergoes a breast biopsy which reveals the presence of a mucinous breast cancer. What will you tell the patient about this particular cancer?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 69-year-old woman presents with a palpable mass in the left breast. Mammography shows a relatively well-circumscribed lobulated mass in the right upper portion of the left breast. Ultrasonography reveals a well-defined, oval, isoechogenic solid mass with small cystic component measuring approximately 2.8cm in the left breast. An excisional biopsy is performed. Microscopic evaluation reveals nests of cells floating in lakes of mucin partitioned by delicate fibrous septa containing capillary blood vessels. The cell clusters are variable in shape and size with an occasional tubular arrangement. Immunohistochemical studies show positive immunostaining of tumor cells for estrogen and progesterone receptors. HER2 is not amplified. What is the most likely diagnosis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following statements regarding mucinous breast carcinoma is least likely to be true?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following statements regarding mucinous breast carcinoma is most likely incorrect?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following statements describes the typical immunohistochemical profile of mucinous breast carcinoma?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following statements regarding mucinous breast carcinomas is true?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Cancer, Mucinous Breast Carcinoma - References

References

Tan PH,Tse GM,Bay BH, Mucinous breast lesions: diagnostic challenges. Journal of clinical pathology. 2008 Jan;     [PubMed]
Righi L,Sapino A,Marchiò C,Papotti M,Bussolati G, Neuroendocrine differentiation in breast cancer: established facts and unresolved problems. Seminars in diagnostic pathology. 2010 Feb;     [PubMed]
Komaki K,Sakamoto G,Sugano H,Morimoto T,Monden Y, Mucinous carcinoma of the breast in Japan. A prognostic analysis based on morphologic features. Cancer. 1988 Mar 1;     [PubMed]
Domfeh AB,Carley AL,Striebel JM,Karabakhtsian RG,Florea AV,McManus K,Beriwal S,Bhargava R, WT1 immunoreactivity in breast carcinoma: selective expression in pure and mixed mucinous subtypes. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc. 2008 Oct;     [PubMed]
Lacroix-Triki M,Suarez PH,MacKay A,Lambros MB,Natrajan R,Savage K,Geyer FC,Weigelt B,Ashworth A,Reis-Filho JS, Mucinous carcinoma of the breast is genomically distinct from invasive ductal carcinomas of no special type. The Journal of pathology. 2010 Nov;     [PubMed]
Barkley CR,Ligibel JA,Wong JS,Lipsitz S,Smith BL,Golshan M, Mucinous breast carcinoma: a large contemporary series. American journal of surgery. 2008 Oct;     [PubMed]
Di Saverio S,Gutierrez J,Avisar E, A retrospective review with long term follow up of 11,400 cases of pure mucinous breast carcinoma. Breast cancer research and treatment. 2008 Oct;     [PubMed]
Kaoku S,Konishi E,Fujimoto Y,Tohno E,Shiina T,Kondo K,Yamazaki S,Kajihara M,Shinkura N,Yanagisawa A, Sonographic and pathologic image analysis of pure mucinous carcinoma of the breast. Ultrasound in medicine     [PubMed]
Chang YW,Kwon KH,Lee DW, Synchronous bilateral mucinous carcinoma of the breast: case report. Clinical imaging. 2009 Jan-Feb;     [PubMed]
Okafuji T,Yabuuchi H,Sakai S,Soeda H,Matsuo Y,Inoue T,Hatakenaka M,Takahashi N,Kuroki S,Tokunaga E,Honda H, MR imaging features of pure mucinous carcinoma of the breast. European journal of radiology. 2006 Dec;     [PubMed]
Zhang L,Jia N,Han L,Yang L,Xu W,Chen W, Comparative analysis of imaging and pathology features of mucinous carcinoma of the breast. Clinical breast cancer. 2015 Apr;     [PubMed]
Garcia Hernandez I,Canavati Marcos M,Garza Montemayor M,Lopez Sotomayor D,Pineda Ochoa D,Gomez Macias GS, Her-2 positive mucinous carcinoma breast cancer, case report. International journal of surgery case reports. 2018;     [PubMed]
Memis A,Ozdemir N,Parildar M,Ustun EE,Erhan Y, Mucinous (colloid) breast cancer: mammographic and US features with histologic correlation. European journal of radiology. 2000 Jul;     [PubMed]
Wilson TE,Helvie MA,Oberman HA,Joynt LK, Pure and mixed mucinous carcinoma of the breast: pathologic basis for differences in mammographic appearance. AJR. American journal of roentgenology. 1995 Aug;     [PubMed]
Toikkanen S,Kujari H, Pure and mixed mucinous carcinomas of the breast: a clinicopathologic analysis of 61 cases with long-term follow-up. Human pathology. 1989 Aug;     [PubMed]
Weigelt B,Horlings HM,Kreike B,Hayes MM,Hauptmann M,Wessels LF,de Jong D,Van de Vijver MJ,Van't Veer LJ,Peterse JL, Refinement of breast cancer classification by molecular characterization of histological special types. The Journal of pathology. 2008 Oct;     [PubMed]
Adsay NV,Merati K,Nassar H,Shia J,Sarkar F,Pierson CR,Cheng JD,Visscher DW,Hruban RH,Klimstra DS, Pathogenesis of colloid (pure mucinous) carcinoma of exocrine organs: Coupling of gel-forming mucin (MUC2) production with altered cell polarity and abnormal cell-stroma interaction may be the key factor in the morphogenesis and indolent behavior of colloid carcinoma in the breast and pancreas. The American journal of surgical pathology. 2003 May;     [PubMed]
Toikkanen S,Eerola E,Ekfors TO, Pure and mixed mucinous breast carcinomas: DNA stemline and prognosis. Journal of clinical pathology. 1988 Mar;     [PubMed]
Komenaka IK,El-Tamer MB,Troxel A,Hamele-Bena D,Joseph KA,Horowitz E,Ditkoff BA,Schnabel FR, Pure mucinous carcinoma of the breast. American journal of surgery. 2004 Apr;     [PubMed]
Park S,Koo J,Kim JH,Yang WI,Park BW,Lee KS, Clinicopathological characteristics of mucinous carcinoma of the breast in Korea: comparison with invasive ductal carcinoma-not otherwise specified. Journal of Korean medical science. 2010 Mar;     [PubMed]
Yang M,Li X,Chun-Hong P,Lin-Ping H, Pure mucinous breast carcinoma: a favorable subtype. Breast care (Basel, Switzerland). 2013 Mar;     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Ob Gyn-Medical Student. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Ob Gyn-Medical Student, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Ob Gyn-Medical Student, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Ob Gyn-Medical Student. When it is time for the Ob Gyn-Medical Student board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Ob Gyn-Medical Student.