We discuss a unique case of a benign breast neoplasm that initially appeared to be an intracystic papillary carcinoma on digital breast tomosynthesis, ultrasound, and contrast-enhanced mammogram. However, subsequent histopathological examination (HPE) revealed it to be a benign adenomyoepithelioma, an uncommon neoplasm in the breast. Understanding this rare condition and confirming it pre-operatively through HPE can prevent unnecessary aggressive treatment.

Abstract Disclosure: P. Vaduva: None. F. Violon: None. A. Jouinot: None. L. Bouys: None. S. Espiard: None. F. Bonnet-Serrano: None. N. Marie Odile: None. C. Cardot-bauters: None. G. Raverot: None. S. Hieronimus: None. H. Lefebvre: None. M.L. Nunes: None. A. Tabarin: None. L. Groussin Rouiller: None. G. Assié: None. M. Sibony: None. M. Vantyghem: None. P. Eric: None. J. Bertherat: None. Objective: Carney Complex (CNC) is a rare hereditary genetic syndrome, mostly due to inactivating pathogenic variants of the tumor suppressor gene PRKAR1A. It has a wide spectrum of manifestations with frequent pigmented skin lesions, cardiac myxomas, primary pigmented nodular adrenocortical dysplasia (PPNAD) causing Cushing syndrome, acromegaly and thyroid cancers. Breast benign tumors (fibroadenomas, ductal adenomas and myxoid lesions) have been associated with CNC, but so far, association with malignancy has not been investigated. Methods: The present study was designed to describe the characteristics of breast tumors diagnosed in CNC patients and their association with other manifestations of CNC and PRKAR1A genotype. Since breast cancer is the most frequent cancer in women and the leading cause of death from cancer worldwide, malignant breast lesions were carefully analyzed. This cohort comes from a 3 years’ follow-up multicenter French prospective study of CNC patients (Espiard, JCEM 2020). The 50 included women were investigated for CNC manifestations and particularly breast tumors, with breast imaging (echography+/-mammography), genetic and hormonal investigations, in order to characterize them and assess the frequency and average age of breast cancer. Results: Among the 38 women with breast imaging, 14 (28%) had breast tumors, half of them being bilateral. Ten women (20%) presented with benign tumors. Six women presented (12%) with breast carcinomas: five had invasive cancer under 50 years of age (10%) with one contralateral recurrence (4 ductal adenocarcinomas and one solid intracystic papillary carcinoma) and one had ductal carcinoma in situ. The occurrence of breast cancer was more frequent in the CNC women with PRKAR1A pathogenic variants than in the general population (OR=6.2[1.6-17.3]; p=0.006). The mean age at breast cancer diagnosis was 44.7 years old, 17 years younger than in the general population. Cumulative risk of breast cancer for women under 40 years of age was much higher in CNC women, compared to women worldwide (OR=592.1[79.7-3817.4]; p=9.58). Breast cancer had good prognosis factors: 5 out of 6 tumors were T1N0M0, so stage I. One woman had metastatic breast cancer, still alive after 15 years follow up. All breast cancers were negative for HER2, with 100% positivity for estrogen receptor and 50% for progesterone receptor. They all occurred in individuals with familial CNC and PRKAR1A pathogenic variants. Loss of heterozygosity at the PRKAR1A locus observed in 2 investigated breast carcinomas tumor tissue suggests that PRKAR1A bi-allelic inactivation could promote breast cancer development. Conclusions: Breast carcinoma occurs frequently and at an early age in women with CNC, suggesting that CNC predisposes to breast carcinoma. This suggests that adequate screening strategy (starting around 40 years old) and follow up should be discussed in CNC women. Presentation: Thursday, June 15, 2023

Evaluation of the management of intracystic papillary carcinoma

Intracystic Papillary Carcinoma of Breast - A Case Report

An Audit on the Role of SLNB in high-risk DCIS and Intracystic papillary Carcinoma (IPC). Mermaid centre- Royal Cornwall Hospital-UK

Objective To study clinicopathological features, treatment strategies, and prognosis of papillary carcinoma of breast. Material and Methods. Data from 58 patients were retrospectively reviewed from January 2010 to December 2016. Four types of papillary carcinoma (on final resected specimen) were included, i.e., invasive papillary carcinoma (IPC), intracystic (encapsulated) papillary carcinoma (EPC), solid papillary carcinoma (SPC), and papillary DCIS (ductal carcinoma in situ). Various features of the four types were observed and compared. Results Of the 58 patients, 8 were males (13.7%). The mean age at presentation was 61 years; the mean tumor size was 33 mm. The frequency of each histological type was as follows: IPC (n = 22/38%), EPC (n = 22/38%), SPC (n = 12/20.6%), and papillary DCIS (n = 2/3.4%). Only two patients were ER negative (both IPC). HER-2 Neu was positive in 3 patients only, out of which 2 died of progressive disease (one EPC and one IPC). LN metastasis was present in 3 (5%) patients (one in each of 1st three types) and only one died of bone metastasis that was also Her-2Neu positive. All patients underwent upfront surgery except two patients who had synchronous IDC on the contralateral side. Breast conservation surgery (BCS) was performed in 34 (58.6%) and mastectomy in 22 (37.9%) patients. 13 patients did not undergo invasive axillary staging; the rest of 43 (74%) patients did (32 sentinel biopsy and 11 axillary dissection). Chemotherapy was given to 18 patients (31%), mostly to IPC (n = 12). Only 2 patients had bone metastasis (one was IPC and one EPC). Cancer-related death was observed in 3 patients. For all groups combined, 5-year OS was 98% and DFS was 92%. Conclusion Overall, papillary carcinoma of the breast has an excellent prognosis, even though less intense treatment modalities were used. It is still difficult to define the optimum management and avoid overtreatment, given the limited data in the literature.

Papillary lesions in the breasts are uncommon and have a wide range of pathologies. Due to diverse non-specific findings radiologically and histologically, papillary neoplasms are always a challenge to radiologists. Encapsulated papillary carcinomas (EPCs) of the breast, also known as intracystic papillary carcinomas, are a subgroup of intraductal papillary lesions of the breast. We present a case of a painless right breast lump with the aim to describe a rare encapsulated papillary carcinoma and its imaging features.

BackgroundEncapsulated Apocrine Papillary Carcinoma (EAPC) of the breast are very rare tumors. They usually present as a cystic mass with mural nodule in women aged 44–84 years. Affected patients may be asymptomatic or complain of breast swelling, or nipple discharge. Mammographic features are nonspecific. The tumor appears as a high-density round or oval mass with circumscribed or spiculated margins on mammography. On Ultrasonography (US) a classical lesion appears as predominantly cystic mass with intracystic solid papillary projections and area of vascularity within it. Contrast Enhanced Breast MRI helps to further characterize the lesion by showing intense contrast enhancement in early phase along with washout curve. Histological features of these tumors are similar to those of classical encapsulated papillary neoplasm, in that myoepithelial cells are absent within the papillary structures and at the periphery of the cyst. They show variable degree of cytological atypia and mitotic activity. Such tumors consist of abundant granular eosinophilic cytoplasm and large nuclei with prominent nucleoli and are androgen receptor positive. However, a multidisciplinary approach is crucial for diagnosis and tissue histology is essential to suitably formulate treatment guidelines. Surgical excision is the preferred treating option for these tumors. Little consensus is available about local radiation and adjuvant therapy for the treatment of such tumors, yet they have been reported to have good prognosis.Case presentationThis rare case report describes the intriguing journey towards the diagnosis of an encapsulated intracystic apocrine papillary carcinoma in a 52-year-old premenopausal female who presented with palpable breast swelling in left breast. The patient underwent mammography, breast ultrasound and breast MRI concluding it to be a BIRADS 4A lesion. Then the patient was subjected to trucut core biopsy and finally surgical excision was performed. On Histopathology this lesion was diagnosed as encapsulated apocrine papillary carcinoma with androgen receptor positivity and triple negative hormonal status. At present patient is on regular follow-up.ConclusionsEncapsulated intracystic apocrine papillary carcinoma has a favorable prognosis with low recurrence rate and excellent long-term survival regardless of its invasive nature. Therefore, we present this rare entity to highlight the importance of radiological and histopathology findings in its diagnosis.

Intracystic papillary carcinoma (IPC) of the breast is a rare malignant tumour, found mainly but not exclusively in elderly women. IPC may be asymptomatic or presents with a palpable mass or blood-stained nipple discharge. Radiologic manifestations of IPC are not specific. On ultrasonography, it can be a pure cyst, a mixed image, or a solid mass. Histologic features of the tumor include cellular proliferations surrounding fibrovascular cores, with or without invasion. The mainstay of treatment is breast-conserving surgery or mastectomy. Sentinel node biopsy could be considered in invasive cases. Adjuvant radiotherapy and/or endocrine therapy is considered in appropriate cases. Through the observation of a 58-year-old patient, we report the epidemiological, clinical and radiological data of papillary breast carcinoma.

Accessory Breasts can occur anywhere along the milk line extending from the axilla to the groin. In view of cosmetic reasons, accessory breasts are removed. We present three cases of accessory breasts with intra-cystic papillary carcinoma in them. Intra-cystic papillary carcinoma is a rare carcinoma that has finger like projections. It has a good prognosis and hence a simple excision biopsy is enough for these patients.

Abstract Aims Male breast cancer (MBC) is rare and accounts for 1% of all breast cancers. We present a case series of three very rare histological subtype MBC within one year in a UK hospital. Methods We retrospectively identified all MBCs from the local breast cancer database. Their presentations, radiology and histopathology were analysed. Results Three MBCs were identified from 2019 to 2020. A healthy 28-year-old presented with a right breast lump. Ultrasound (USS) revealed a partially cystic 15mm U3 lesion. Mammography (MMG) was suggestive of gynaecomastia (M2). Biopsy was inconclusive. Diagnostic excision revealed Papillary Ductal Carcinoma in Situ (DCIS) with involved margin. Mastectomy and Sentinel Node Biopsy (SNB) confirmed low-grade Papillary DCIS. A 48-year-old with background of hyperlipidaemia presented with a 2-year history of left axillary lump. PET CT demonstrated an FDG-avid lesion. USS showed a superficial U3 28mm lesion. MMG showed a 34mm density (M4). Biopsy identified Mucinous Carcinoma. Wide Local Excision and SNB confirmed grade 2 Mucinous Carcinoma. A 75-year-old with history of cardiac disease, COPD and Type 2 Diabetes, presented with a right retro-areolar lump. MMG identified a 47mm mass (M4). USS showed a 41mm cystic lesion with wall thickening (U4). Cytology was inconclusive. Biopsy identified intra-cystic papillary carcinoma. Mastectomy and SNB confirmed intra-cystic papillary carcinoma. All three histological subtypes are extremely rare and account for less than 4% of all MBCs. Conclusions MBC is rare but increasing in incidence. More research and awareness are needed to minimise delays in diagnosis and treatment even in young men.

Intracystic papillary carcinoma breast is an uncommon breast cancer consisting of 0.5%–1.0% of all breast cancers. Papillary carcinoma is a rare type of breast carcinoma which is divided into two types, which are intraductal and intracystic papillary carcinoma. Intracystic papillary carcinoma may present as pure intracystic carcinoma or may be associated within situcarcinoma. Here, we report a case of intracystic papillary carcinoma of both breasts which created diagnostic dilemma.

The intracystic papillary carcinoma (IPC) is one of the rarest types of breast cancer, mainly in men, representing less than 1% of the malignant diseases in the male sex. It is frequently associated with the ductal carcinoma in situ (DCIS), but there are also other forms such as the pure and the invasive ones. The male population breast cancer diagnosis is late and, therefore, it has a worse prognosis. The diagnosis is given by imaging tests and anatomopathological studies. The treatment consists of excisional tumor therapy, which can be carried out conservatively or through mastectomy, with or without adjuvant therapy.

Malignancy of breast presenting as cystic lesion is a complex entity to the surgeon and pathologist especially when there is a large cyst at the previous mastectomy site. The incidence of papillary carcinoma is 0.5% of all the invasive carcinomas of the breast. In localised variety, it forms a mass which can be cystic or solid. It can be diffuse with terminal duct lobular units and correspond to papillary variant of Ductal Carcinoma in-situ (papillary DCIS). Here, authors reported an interesting case of a 62-year-old female who presented with large cystic lesion on left breast that after excision was reported as papillary carcinoma of breast. Another patient, 73-year-old also presented with cystic lesion on breast which was diagnosed as papillary carcinoma after excision biopsy. The main fact to be noted here is that approximately half of patients with intracystic carcinoma are associated with underlying ductal carcinoma (in-situ/invasive).

Carcinoma of breast is rare in men with an estimated incidence of one per 100,000 per annum. Of all the histological types of breast cancer, Intra-cystic papillary carcinoma (IPC) of the breast is rare accounting for less than 1% of all breast malignancies. A 61 year old gentleman presented with painless, progressively enlarging lump in his left breast since 3 years associated with serosanginous nipple discharge of 3 months duration. On examination, a single 8 x 3 cm well defined cystic swelling in the retro-areolar region was felt with normal overlying skin and nipple-areola complex. No axillary lymph nodes were palpable. FNAC of the lump showed features of infiltrating ductal carcinoma. Patient was taken up for modified radical mastectomy under general anaesthesia. Histopathological examination of the specimen revealed a cyst measuring 7x4 cm containing hemorrhagic fluid. On gross examination, the cyst wall exhibited grey white papillary excrescences that protruded into the lumen. Histopathological diagnosis of intracystic papillary carcinoma was given. Being a rare entity, there is no definite guideline for the treatment of IPC; however, a surgical excision with a clear resection margin forms the mainstay of the treatment. These patients have very good outcome. The addition of hormonal treatment does not appear to have an impact on the patient outcome. Keywords: Intra cystic, Papillary, Male, Breast carcinoma.

Breast carcinoma in men is rare and intra-cystic papillary carcinoma of male breast is an extremelyrare entity. Papillary lesions of male breast comprise of the spectrum of lesions ranging from benignintraductal papilloma to intraductal papillary carcinoma and invasive papillary carcinoma. It occursmost commonly in the seventh decade of life and accounts for <1% of all breast malignancies. Thepresent case report is of a comparatively younger, 37 years old male who presented with a mass onlower quadrant (retro-areolar region) of the left breast.

Intracystic/encapsulated papillary carcinoma remains a poorly understood disease of the breast with a little amount of reports that describe it. It shares features with DCIS and IDC and predominantly affects postmenopausal women. This study aims to evaluate the clinical presentation, treatment, and outcomes in IPC patients managed at our institution. We retrospectively pooled twenty-eight IPC patients' medical records at our institution. Descriptive analysis of clinicopathological characteristics, approach, and outcomes was done along with a quantitative statistical analysis. Cases were divided into three groups: isolated IPC, IPC associated with DCIS, and IPC associated with Invasive Carcinoma. Treatment modalities varied according to the IPC type and its associated components. All patients presented with a palpable mass. Immunohistochemical staining revealed that all isolated IPCs were ER and PR positive and HER2 negative. Lymph node dissection proved necessary only in IPC associated invasive carcinoma. Irregular borders and lobulations, among others, were found on non-invasive core biopsies that turned out to be associated with invasion on surgical pathology. All patients were alive after a median follow-up time of 23months when the study was over with no reports of recurrence. IPC cases and treatment approaches at our institution appear similar to the available literature and confirm the excellent prognosis among IPC. Even more, further studies into the key features such as BMI, family history, and radiological findings are necessary for a potential algorithm that could assess for risk of finding invasion in surgical pathology and subsequently the need for axillary/sentinel lymph node biopsy.

A 70 year old lady presented with two lumps in the right breast since around fifteen years which were increasing in size since the past one year. Mammography showed a solid-cystic and a lobulated lesion in the right breast which were categorized as BIRADS (Breast Imaging- Reporting and Data System) category 4/5. On FNAC, a diagnosis of Atypical Ductal Hyperplasia, NHSBSP (National Health Service Breast Screening Programme) Category C4 was made and histopathologic examination advised. Subsequently right sided simple mastectomy was received the larger mass in which showed extensive areas of sclerosing adenosis with superimposed ductal carcinoma in situ. The subareolar mass showed a papillary lesion displaying epithelial overgrowth with features compatible with ductal carcinoma in situ. A thorough search revealed invasive nests in the vicinity of the papillary lesion. A diagnosis of Infiltrating Ductal Carcinoma arising in a multifocal complex sclerosing lesion with extensive intraductal carcinoma and intracystic papillary carcinoma was made. This case offers to display a pathologic continuum of sclerosing breast lesions evolving into atypical hyperplasias, intraductal carcinoma and invasive carcinoma. This falls in line with the current thoughts on premalignant nature of sclerosing lesions of the breast.

Published data on male breast cancers (MBCs) are scare in Ghana. We report a case of breast cancer in a 79-year old man, who presented at the Tamale Teaching surgical unit with a clinically advanced stage of the disease. He had modifified mastectomy and an invasive intracystic papillary cancer was diagnosed histologically. Immunohistochemically, this tumour was ER+/PR+ /HER2-However, hormonal therapy could not be started, for the 79-year old before he died in the third week after being discharge from the hospital.

Intracystic papillary breast cancer