The accessory breast tissue in the axillary region is rare, but as there is breast tissue, there is the possibility of pathological degeneration. With an even greater rarity, breast sarcoma is a diverse group of malignancies derived from mesenchymal tissues. The aim of this report was to describe a case of a young patient with sarcomatous neoplasia in the axillary accessory breast topography. Patient LPMS, 19 years old, female, admitted to the mastology service of the Hospital das Clínicas of the Universidade de São Paulo with a nodule realized in the left axillary region with progressive growth during pregnancy. The patient was healthy with a family history of a maternal aunt with breast cancer at 50 years old. At the clinical examination, she had an extensive tumor affecting the left axillary region measuring 10 cm with clinically negative axilla. Contralateral breast and axilla and supra-/infraclavicular fossas without abnormalities. In the initial mammogram, a hyperdense, oval, and indistinct nodule was found in the left axillary extension, measuring 10.8 cm. In breast magnetic resonance imaging, a heterogeneous mass in the left axillary extension is observed with irregular, lobulated margins, measuring 10.3×10×10.1 cm, heterogeneously and concentric by the contrast. Core biopsy was performed with the result of spindle cell mesenchymal neoplasm. In the systemic staging examinations, there was no evidence of a lesion suspected of distant metastasis. Vincristine 1.5 mg/m2 + Actinomycin D 0.45 mg/kg/day + Cyclophosphamide were prescribed to the patient. After six cycles with no clinical response, it was decided to switch the neoadjuvant chemotherapy to doxorubicin 25 mg/m2 and Ifosfamide 3,000 mg/m2 . After three cycles, the patient remains without a clinical response to neoadjuvant chemotherapy. It was decided to refer the patient to radiotherapy for axillary irradiation on the right, with a subsequent surgical approach. The patient underwent surgery with wide resection of the tumor and axillary lymph nodes. The anatomopathological examination showed sarcoma with immunohistochemistry suggestive of rhabdomyosarcoma measuring 19.3×14.8×14.7 mm with free margins and with sarcoma metastasis in one of the 21 dissected lymph nodes. The patient progresses well postoperatively. Accessory breast tissue has a very rare incidence in the population, with incidence rates of 1–2%. With regard to breast sarcoma, it is a very rare condition. It consists of a heterogeneous group of nonepithelial tumors originating from the mesenchymal tissues of the breast. They account for <1% of all breast malignancies and <5% of all sarcomas. Due to its rarity, current knowledge about breast sarcoma is limited and is mainly based on small retrospectives, case series, or case reports. Angiosarcoma, including secondary angiosarcoma from before breast radiation, is the most frequent type of breast sarcoma. As with other soft-tissue sarcomas, the primary breast sarcoma is associated with genetic conditions such as Li-Fraumeni syndrome, familial adenomatous polyposis, and type 1 neurofibromatosis. Therefore, breast sarcoma treatment generally follows the algorithms derived from trials of soft-tissue sarcomas in the chest wall, as has been done with the reported patient. Surgical treatment is the standard and most accepted treatment for breast sarcoma. The role of chemotherapy for breast sarcoma is also uncertain. There are no prospective studies that specifically assess the benefit of chemotherapy in adjuvant or neoadjuvant settings. Likewise, the benefit of radiotherapy in breast sarcoma is also very doubtful with evidence of benefit in large tumors and with positive margins after surgical resection.
Objective: To select cases of bilateral breast carcinoma (BBC) of patients seen at Hospital de Clínicas of Paraná, besides recognizing clinical and family characteristics, histological and immunohistochemical pattern, and incidences of synchronic/metachronic tumor in these patients. Method: Observational and analytical study of BBC cases of patients treated at Hospital de Clínicas of Paraná, from 2003 to 2019, developed from the analysis of medical records. Result: A total of 42 patients with BBC were selected. The incidence of BBC was 3.64%. All patients were women, mostly of white skin color and postmenopausal, with an average age of 51.82 years. Half patients showed a positive family history for cancer, with breast cancer present in 46%, ovarian cancer in 16%, and other topographies in 68%. In this sample, the synchronous tumor was present in 55% of patients, and the metachronous tumor, in 45%. Regarding patients’ initial clinical staging, 61% had a locally advanced tumor at diagnosis. Both in the group of synchronic and metachronic tumors, the ductal subtype was the most frequent. Regarding the immunohistochemical subtype, patients in both groups had Luminal B tumors more frequently. In the group of metachronic tumors, the average time between the diagnosis of the first tumor and the second tumor was 5.68 years. Conclusion: In this sample, BBC is associated with a relevant family history, with a synchronic presentation pattern, from histology to ductal and immunohistochemistry to Luminal B as the most frequent.
Introduction: Bilateral breast cancer (BBC) is a rare clinical entity. This pattern of neoplasia can be considered synchronous (simultaneous) or metachronous (1 month to 1 year later). Objective: To select cases of BBC patients seen at HC-UFPR and to recognize in these patients clinical and family characteristics, histological and immunohistochemical patterns, and incidences of synchronous/metachronous occurrences. Method: An observational and analytical study of BBC cases of patients treated at HC-UFPR, from January 2003 to October 2019, based on the analysis of medical records, was developed. Result: 42 patients with BBC were selected and 4 patients were excluded from the study due to incomplete information in the medical record. The incidence of BBC in the surveyed period was 3.64%. All patients are women with a mean age of 51.82 years. White ethnicity is the most prevalent one (82%). With regard to menopausal status, 42% of the subjects are pre-menopausal and 58%, post-menopausal. Regarding parity, only 16% were nulliparous. Half of the patients have a positive family history for neoplasms, with breast cancer present in 46%, ovarian cancer in 16%, and other topography in 68%. In this sample, the synchronous tumor was present in 55% of the patients and the metachronous tumor in 45%. Regarding the patients’ initial clinical staging, 61% presented with locally advanced tumor at the first consultation. In the group of synchronous tumors, the ductal type was the most frequent one (93%), followed by the lobular type (7%). Regarding immunohistochemical subtypes, patients had Luminal B tumors (43%), followed by HER (29%), Triple negative (24%), and Luminal A (5%). Comparing the immunohistochemical profile in both tumors, 62% were in agreement and 48%, in disagreement. In the group of metachronous tumors, the mean time between the diagnosis of the first tumor and that of the second tumor was 5.68 years. The most common histological type was ductal carcinoma (73%), followed by lobular carcinoma (11%), medullary carcinoma (9%), and metaplastics (7%). Regarding the immunohistochemical profile, the most present was Luminal B in 32%, Luminal A in 29%, Triple negative in 24%, and HER 2 in 15%. The immunohistochemical profile was consistent in only 29% of the patients. Conclusion: In this sample, BBC is associated with relevant family history, with a pattern of presentation, synchronous; frequently, ductal is histological and Luminal B is immunohistochemistry.
Introduction: Gestational gigantomastia (GG) is a rare condition known for the massive growth of one or both breasts during pregnancy. Objectives: to describe three cases of GG in patients assisted Hospital de Clínicas do Paraná. Case Reports: Case 1 ‒ Twenty-four year old pregnant woman, of 9 weeks, presented with mastalgia and increasing breast volume associated with phlogistic signs and orange peel-like skin. Breast ultrasound showed diffuse inflammatory process. A biopsy was performed and showed ductal ectasia and stromal fibroadenomatoid changes. On the 20th week, she was hospitalized with fever, voluminous breasts and drainage of purulent secretion and long ulcers in the inframammary fold. She was administered antibiotics and local care. The choice was to interrupt the pregnancy of 33 weeks. She presented with relative reduction of the breasts and breastfed, but had severe psychological problems caused by the aesthetic dissatisfaction. Case 2 ‒ Eighteen-year old patient presented with infiltrating ductal breast carcinoma in the right breast, treated with sectorectomy, with sentinel lymph node and conventional radiotherapy. After one year of follow-up, ductal carcinoma in situ (DCIS) was diagnosed in the left breast, being submitted to sectorectomy and intraoperative radiotherapy. Two years after DCIS, she became pregnant and presented with GG only in the left breast, with remarkable asymmetry and skin edema. After the Cesarean section, breastfeeding was normal on the GG side, but not on the right side, due to a sequel from total radiotherapy. Case 3 ‒ Twenty-two year old pregnant women, of 12 weeks, presented with mastalgia and exaggerated growth of the breasts, with and orange peel-like skin. Breast ultrasound showed bilateral inflammatory process. On the 15th week, she presented with bleeding ulcers, right breast with latero-lateral diameter of 48 cm, and craniocaudal diameter of 56 cm, and left breast with 49 cm and 58 cm, respectively. Due to the fast and progressive growth of the breasts, with difficulties to breath, major venous and lymph stasis and extensive skin ulceration/maceration, with risk of infection, the patient and relatives agreed on conducting a therapeutic abortion, followed by a reduction mammoplasty. Discussion: It is a rare disease, and its etiology remains unknown. Medicinal therapy seems to be first option, but it is often necessary to complement it with a surgical procedure. Conclusions: GG is a pathology of great morbidity, with tendency to recede in the puerperium. In case it is not possible to wait, the recommendation is to intervene in the pregnancy.
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