Secretory breast carcinoma—case report and review of the medical literature

doi:10.1016/j.breast.2004.01.005

The Breast

Volume 13, Issue 4, August 2004, Pages 353-355

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Abstract

Secretory breast carcinoma (SBC) is a rare type of invasive breast cancer. Since little is known about the biology of this rare tumour, it is useful to report every such case, in order to make as much information as possible available in the medical literature. We present the case of an 18-year-old woman with a SBC treated by mastectomy (Madden) and axillary node dissection (stage pT3N1M0)¹ followed by chemotherapy (FEC² regimen) and radiotherapy. The patient has meanwhile completed 4 years of follow-up with no evidence of recurrence. The authors review the literature and summarize relevant findings concerning definition, pathology, clinical picture, treatment, and follow-up.

Introduction

More than 95% of breast malignancies arise from the epithelial elements of the breast and are therefore carcinomas. Breast carcinomas can be divided into two major groups: (1) carcinoma in situ, in which the tumour cells remain confined to the ducts; and (2) infiltrating carcinoma, whose tumour cells invade the breast stroma and therefore, have the potential to metastasize.

SBC is an extremely rare type of breast cancer, accounting for less than 1% of all infiltrating breast carcinomas. It was originally termed ‘juvenile breast cancer’ by McDivitt and Stewart in 1966, because the only known patients were children or very young women.1., 2., 3. However, subsequent studies have included many adult patients, including a substantial number of postmenopausal women, so much so that approximately two-thirds of the almost 100 published cases have been in adults. Consequently, the term juvenile breast cancer has been replaced by the more descriptive ‘secretory breast cancer’ (SBC).3., 4.

The tumour can occur anywhere in the breast. It frequently presents as a mobile, well-circumscribed, subareolar mass, sometimes simulating fibroadenoma.3., 5.

Although it is well circumscribed macroscopically, there may be foci of invasion in the surrounding breast tissue and associated ductal carcinoma in situ (DCIS), which can be responsible for local recurrence after incomplete excision.3., 4. Three histological patterns are seen, in varying combinations: tubular, compact and honeycomb.³ The tumour is typically composed of cells with abundant granular eosinophilic cytoplasm arranged around small gland-like spaces. The spaces and the cells themselves contain recognizable material.¹ When studied microscopically, SBC proves to be a variant of invasive ductal carcinoma with unusual structural features.⁴

There is no known hormonal abnormality that would explain the prominent secretory activity in this type of carcinoma. It is normally negative for oestrogen and progesterone receptors.⁴

It is necessary to perform a surgical biopsy to establish the diagnosis of SBC, but the lesion is sometimes suspected on examination of an aspiration cytology specimen.4., 6.

Section snippets

Case report

The authors present the case of a 18-year-old woman resident in Portugal, who had been followed in the surgical unit of her local hospital from the age of 15 for an occasional brownish discharge from the left nipple. At the time of her first presentation she had undergone mammary echography, which documented cystic formation with a probable intracystic papilloma, galactography, which showed a normal picture, cytological investigation (negative) and bacteriological examination of the mamillary

Discussion

Surgery is still considered the most appropriate treatment for primary SBC.1., 3. There is some degree of consensus about the extent of surgery, as the principal management problems are late local chest wall recurrence and regional axillary lymph node spread.³ Whenever possible, prepubertal girls should be treated initially by wide local excision. Preservation of the breast bud should be attempted, but this is not always possible, so that breast development can be impaired. Breast tissue

Conclusion

The current case illustrates an unusual presentation of this rare type of breast carcinoma: a large lesion with lymph node involvement at the time of mastectomy and none of the clinicopathological findings that are thought to confer a good prognosis. This was crucial in our decision on adjuvant treatment despite the tumour's biological indolence.

Earlier publications on such tumours include few papers that specify the type of chemotherapy and radiotherapy given, when applicable, and results

References (8)

H.S. Gallager
Pathologic types of breast cancertheir prognoses
Cancer
(1984)
R.W. McDivitt et al.
Breast carcinoma in children
J Am Med Assoc
(1966)
H. Herz et al.
Metastatic secretory breast cancer. Non-responsiveness to chemotherapycase report and review of the literature
Ann Oncol
(2000)
P.P. Rosen et al.
Secretory carcinoma of the breast
Arch Pathol Lab Med
(1991)

There are more references available in the full text version of this article.

Cited by (34)

Surgical management of secretory breast carcinoma in children
2023, Current Problems in Cancer: Case Reports
Breast cancer is extremely rare in children and consequently no consensus has been reached on the optimal treatment modalities. We have reported the medical history of secretory breast carcinoma (SBC) in a 6-year-old girl. An areolar-sparing mastectomy was performed without axillary surgery, and without adjuvant therapy given her limited disease and the histological type. We have reviewed the literature and summarized relevant findings concerning the clinical and histological data, treatment approaches, and outcomes for 33 cases of SBC (15 children and 18 adolescents). International data suggests that local excision without axillary surgery may be a suitable therapeutic approach for children with SBC.
Local Recurrence of Invasive Secretory Breast Carcinoma in a Gravid Patient Post-Mastectomy
2022, Radiology Case Reports
Citation Excerpt :
Radiation was deferred at original presentation and recommended only after recurrence. Factors associated with recurrent secretory carcinoma includes larger initial tumor size and older age at time of initial diagnosis [2]. To our knowledge, no prior published literature has described pregnancy affiliated recurrence of secretory carcinoma, or local recurrence of secretory carcinoma following nipple sparing mastectomy.
This is a case of locally recurrent invasive secretory carcinoma of the breast during pregnancy, detected as a palpable mass in the reconstructed right breast of a 32-year-old female at 24 weeks gestation. The patient was initially diagnosed with secretory carcinoma 8 years prior, for which she underwent nipple sparing mastectomy followed by adjuvant chemotherapy and endocrine therapy. Due to pregnancy, the recurrence was treated initially with conservative excision alone, followed by definitive management postpartum which included wide local excision, sentinel lymph node biopsy and adjuvant chest wall radiation. Secretory carcinoma of the breast is a rare cancer with a predilection for young age and indolent course. This case report describes an unusual case of recurrent secretory carcinoma, of interest due to both its diagnosis during pregnancy, and its recurrence after nipple sparing mastectomy.
Secretory breast carcinoma: A clinicopathological and immunophenotypic study of 15 cases with a review of the literature
2012, Modern Pathology
Secretory breast carcinoma is a rare breast cancer with indolent clinical behavior. Recent research showed that secretory breast carcinoma belongs to the phenotypic spectrum of basal-like breast carcinomas. In this study, a clinicopathological and immunophenotypic analysis of secretory breast carcinomas from 15 Chinese patients was conducted. This patient group consisted of 2 males and 13 females, with ages ranging from 10 to 67 years old (median, 36 years old). All patients presented with a painless and firm mass. Tumor size ranged from 10 to 55 mm. Most tumors were located in the outer upper quadrant of the breast. Two patients (2 of 13, 15%) displayed positive axillary lymph nodes. At the microscopic level, the presence of intracellular and extracellular secretory material was the most remarkable feature. Most cases showed mild dysplasia cytologically. All cases were negative for estrogen receptor, progesterone receptor and HER2. The expression rate of the basal-like marker (CK5/6 or epidermal growth factor receptor) was 87% (13 of 15). The basal-like phenotype was identified in 13 cases (87%). Follow-up time ranged from 10 to 55 months (median, 19 months). None of the cases had evidence of recurrence and metastasis. Our study reveals that secretory breast carcinoma is a distinct subset of invasive breast carcinoma, with expression of basal-like markers. It should be noted that secretory breast carcinoma is different from conventional basal-like breast carcinomas. Future studies are required to further understand the prognostic significance of the basal-like markers expression in secretory breast carcinomas.
Adjuvant therapies for special types of breast cancer
2011, Breast
Recent developments in the adjuvant treatment of breast cancer include an increasing attention to systemic therapies prescribed in homogeneous groups of patients according to the higher chance of benefit. A clear consequence of the current adjuvant treatment strategy is the importance of accurate and reliable histopathological assessment. A proper pathological evaluation may effectively support the definition of prognosis and treatment choice in niches of patients diagnosed with special types of breast cancer. Through the identification of special types of breast cancer, that account for up to 25% of all invasive breast carcinomas, it is possible to select patients with a very good prognosis often close to that of the general population (e.g. tubular and pure cribriform carcinoma). Other features, such as those related with invasive classical lobular carcinoma, might have important correlates of responsiveness to therapy other than indicators of outcome. It was in fact demonstrated that the response to primary chemotherapy is significantly lower in invasive lobular carcinoma, if compared with the ductal histotype. However, the use of available information on special types of breast cancer has been limited in tailoring adjuvant therapy, owing to the absence of standardized criteria and partial reproducibility for diagnosis. Moreover, due to the relative rarity of the disease a large number of features that identify for special types of breast carcinomas have today no particular correlation with the prognosis, and limited data are available on the biology of a large number of breast cancer subtypes. The development of more effective therapies for patients with special types of breast cancer requires tailored treatment investigations through international cooperation and should not rely on information predominantly contributed from small retrospective analyses. Examination of patterns of relapse and treatment response within subpopulations in multiple randomized trials is also mandatory to make progress and reach consensus on how to treat individual patients with special types of breast cancer.
Unusual Tumors of the Breast
2009, Current Problems in Surgery
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Estrogen and progesterone receptor studies are usually negative.62,63 Secretory carcinoma usually has a good prognosis.65,68 Surgical excision is the primary treatment for this tumor.
Less Common Triple-Negative Breast Cancers
2022, A Comprehensive Guide to Core Needle Biopsies of the Breast, Second Edition

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¹: American Joint Commission on Cancer (1997).

²: FEC—5-fluorouracil (5-FU)+epirubicin+cyclophosphamide.

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Case reportSecretory breast carcinoma—case report and review of the medical literature

Abstract

Introduction

Section snippets

Case report

Discussion

Conclusion

Pathologic types of breast cancertheir prognoses

Cancer

Breast carcinoma in children

J Am Med Assoc

Metastatic secretory breast cancer. Non-responsiveness to chemotherapycase report and review of the literature

Ann Oncol

Secretory carcinoma of the breast

Arch Pathol Lab Med

Case report
Secretory breast carcinoma—case report and review of the medical literature