Paget’s disease (PD) of the nipple is a rare cancer that affects the nipple and areola and accounts for between 0.4% and 5% of breast cancers. It was first described in 1877 by the English physician Sir James Paget. It affects women between 60 and 70 years of age and very rarely affects men. In PD, the skin on the nipple and areola becomes thicker. Clinical presentations are usually erythema, desquamation, or eczematous changes in the nipple, features that can progressively progress to erosion, overt destruction, and ulceration of the papilla. Bloody papillary discharge, itching, nipple retraction, and/or a palpable mass may be associated. Cancer cells, called Paget cells, are malignant, large, with clear, abundant cytoplasm and nuclei with prominent nucleoli. Like glandular cells, they appear either as isolated cell in the epidermal tissue or as groups of cells. Most women diagnosed with PD also have ductal adenocarcinoma, either in situ or invasive. The prevalence is 67–100% of cases, which gives a worse prognosis to the patient. Patients with Paget-associated invasive breast disease have lower hormone receptor expression, greater lymph node involvement, and higher human epidermal growth factor receptor type 2 (HER2) expression. An 82-year-old woman sought the mastology outpatient clinic for a follow-up of carcinoma in situ in the right breast 2 years ago, having been submitted to quadrantectomy and hormone therapy with tamoxifen, with no signs of recurrence. She complained of an exudative pruritic lesion on the left nipple that had started 6 months ago. She reported that the lesion started with itching and burning, associated with a spontaneous discharge of serous secretion from the itchy surface of the breast, which improved with the use of “talcum powder.” On physical examination, the presence of a discrete reddened area with a diameter of 3 mm, eczematous, with bloody areas interspersed with serous secretion was observed on the left nipple. Areola lesions and palpable nodules in the left breast were absent. She underwent mammography, which showed symmetrical breasts with fat-replaced parenchyma, absence of nodules, presence of isolated calcifications, and grouping in the superior lateral region of the left breast, categorized as BIRADS II. On ultrasound, a nodule with angled edges, measuring 5×4 mm in the superomedial quadrant of the left breast, which showed nodular enhancement and persistent kinetic curve on magnetic resonance imaging of the breasts. The histopathological study diagnosed moderately differentiated left breast ductal carcinoma, associated with a high-grade solid intraductal carcinoma and PD of the nipple, without the involvement of the areola. Immunohistochemistry revealed the absence of estrogen and progesterone hormone receptors and HER-2 overexpression in both histological types. She underwent mastectomy with sentinel lymph node biopsy that was free of neoplasia. Oncological follow-up with no signs of recurrence. PD, if left untreated, extends to the areola and other regions of the breast. Therefore, clinical suspicion from the first physical examination allows an early diagnosis of extreme importance, which improves the prognosis and allows less aggressive treatments.
Dermatofibrosarcoma protuberans (DP) is a neoplasm of the deep layer of the dermis and subcutaneous tissue. It presents a rare incidence and constitutes 0.1% of the malignant tumors. It has local aggressive behavior with slow tumor growth, low metastasis rates of around 5%, but has high rates of local recurrence after surgical excision. The diagnosis is histopathological through biopsy of the lesion, and the fluorescence in situ hybridization (FISH) method can help in selected cases by detecting possible chromosomal rearrangements in the tissue. Physical examination, magnetic resonance imaging, and computed tomography may be helpful in assessing the area of tumor extension. The treatment of choice is resections with 3-cm wide margins or Mohs micrographic surgery. The prognosis is directly related to the correct excision of the compromised margins. A woman, 51 years old, presented with a raised, brownish, irregular, 13×8 cm multinodular lesion attached to the overlying skin, in the epigastric region, which extended to the left hypochondrium and lower quadrants of the left breast, without local symptoms or lymph node enlargement. She reported the appearance of a small nodular skin lesion at the site 10 years ago and reports continuous growth of the nodule, with the involvement of the adjacent skin and the left breast starting 5 years ago, after the formation of a hypertrophic scar due to two previous local resections of the initial lesion. Mammography showed a nodule of cutaneous origin in the lower inner quadrant of the left breast, which may correspond to keloids — BIRADS 2. Breast ultrasound showed a solid, echogenic nodule measuring 1.6×1.2 cm in the left breast at 8 am; 2.5 cm from the nipple — suggestive of lipoma, and at 7 am, nodule measuring 2.4×1.6 cm that penetrates the breast parenchyma — BIRADS 3. The lesion was diagnosed as dermatofibrosarcoma on histopathological examination of a skin fragment. The patient underwent resection of the lesion with a safety margin by the mastology team and primary reconstruction using a thigh graft by the plastic surgery team. A surgical specimen was sent for anatomopathological examination that presented a result compatible with a previous biopsy, reiterating the diagnosis of DP, and with peripheral and deep surgical margins free of neoplastic involvement; evolved without postoperative complications or restriction of range of motion; and referred to radiotherapy to assess the need for additional treatment.
Introduction: Synchronous bilateral breast cancer (SBBC) consists of the simultaneous presence of two primary tumors at diagnosis. There is no consensus on its origin, and it may be a metastasis of a primary lesion or a second independent tumor. The incidence of SBBC has been reported to be approximately 3%. The prognosis of SBBC was considered to be reserved, which is why bilateral mastectomy is the preferred approach. Case report: A 44-year-old patient with no family record of gynecological neoplasm sought care reporting bilateral breast pain and palpable nodular lesions on the breasts. On examination, a hard nodule measuring 10×12 cm was palpated on the left breast (LB) associated with ulcerated areas in the superior lateral quadrant. On the right breast (RB), a mobile nodule was palpable, measuring 8×8 cm with chocolate- -colored nipple discharge. Clinically positive axillary lymph nodes are bilateral. She had a mammogram, which showed a nodule with spiculated contours, measuring 2.5 cm in the SLQ of the LB, with apparent associated dermal retraction and multiple, grouped microcalcifications on RB-BIRADS 5. She underwent core biopsy, which resulted in invasive lobular carcinoma and dermal infiltration, with immunohistochemistry (IHC): positive PR and ER, positive HER2, Ki-67 positive in 40% on LB, and carcinoma invasive ductal, non-special type, with IHC: negative RP and RE, HER2 score 3+, and Ki-67 positive in 60% on RB. She underwent neoadjuvant chemotherapy, followed by bilateral mastectomy with sentinel lymph node biopsy. The anatomopathological (AP) study of the LB surgical specimens revealed residual ductal carcinoma in situ, free margins, and neoplasm-free lymph nodes. The RB’s AP revealed high-grade (comedocarcinoma), intermediate-grade residual intraductal carcinoma, alongside an extensive fibro hyalinized area of the stroma, foci of lobular cancerization, absence of residual invasive component, free margins, and absence of lymph node metastasis. The patient underwent adjuvant radiotherapy and hormone therapy with tamoxifen.
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