Dermatofibrosarcoma protuberans in the breast: Case report

Introduction and importanceDermatofibrosarcoma protuberans (DFSP) represents about 1% of soft tissue sarcomas with an estimated incidence of 0.8–5.0 cases per million per year. The involvement of DFSP in breast is very rare and very few cases have been reported in the literature. DFSP was recurred in situ, not spread to distant site. The complete surgical excision with wide, pathologically negative margins of 3 cms is the optimal treatment for primary or recurrent tumor.Presentation of caseA 46-year-old woman presented with palpable lump in the in the right breast. On ultrasonography, a lesion appeared as hypoechoic, circumscribed mass of approximately 37 mm × 30 mm in diameter in the upper central part of the right breast. The mass of right breast was demonstrated DFPS by pathologic examination. Chest computerized tomography (CT) scan and 2-[18F]-fluoro-2-deoxy-d-glucose (18F-FDG) positron-emission tomography (PET) showed only primary lesion in subcutaneous layer and no enlarged lymph node. The patient underwent excision of the tumor widely. There was no evidence of DFSP local recurrence after five years of follow-up of the patient.DiscussionDFSP is a rare tumor arising from dermis and subcutaneous mesenchymal tissue. Whereas, characteristic imaging feature of DFSP in the breast are not well-defined. The primary treatment for DFSP is considered to be surgical excision.ConclusionDFSP in breast is extremely uncommon and can mimic a primary breast tumor. Surgical excision with adequate resection margins is recommended to ensure local control of the disease.     

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation arising at the site of breast implant

Fibrosarcomatous transformation of dermatofibrosarcoma protuberans (DFSP) is a rare variant with higher risk of recurrence and metastasis, and no known associations with breast implants. We report a rare case of fibrosarcomatous dermatofibrosarcoma protuberans arising at the site of breast implant in a 33‐year‐old patient followed by brief discussion on fibrosarcomatous DFSP.     

Dermatofibrosarcoma protuberans and breast cancer: genetic link or coincidental association?

Dermatofibrosarcoma protuberans is a low-grade cutaneous neoplasm originating from the dermis with a high propensity for local recurrence. These malignancies are classified as soft tissue sarcoma although regional or distance metastatic spread is rare. Germ-line mutations of p53 have been described with a rare autosomal dominant syndrome in patients with soft tissue sarcoma and breast adenocarcinoma (Li Fraumeni's syndrome). The described case illustrates the rare occurrence of dermatofibrosarcoma protuberans (DFSP) and breast adenocarcinoma with an emphasis on the possibility of a molecular or genetic association.     

Rapidly growing breast desmoid tumor with intra‐thoracic involvement after reconstructive surgery for breast cancer

Desmoid tumors are very rare soft tissue neoplasia that are slow growing and locally aggressive. They grow anywhere in the body and are rarely develop in the breast . Histopathologic examination confirms diagnosis. Recurrence rate is very high even after complete resection. We report the management of a rare case of rapidly growing breast desmoid with intra‐thoracic involvement causing cardiac compression.     

Calciphylaxis mimicking inflammatory breast cancer

Calciphylaxis is a rare disorder characterized by microcalcification of small- and medium-sized blood vessels causing cutaneous and soft tissue necrosis. Patients usually present with painful, violaceous skin discoloration in a livedo reticularis pattern. We present a case of a 59-year-old woman with end-stage renal disease (ESRD) who manifested signs and symptoms of inflammatory breast cancer. The patient underwent an open biopsy and subsequent mastectomy, with final pathology results of calciphylaxis. Due to recurrent hyperparathyroidism, she underwent a re-parathyroidectomy with reimplantation. To our knowledge, this is the first reported case of calciphylaxis recurrence after subtotal parathyroidectomy. We propose that those patients with ESRD who develop breast pathology consistent with inflammation and necrosis, and have no malignancy, be evaluated for secondary hyperparathyroidism. Total parathyroidectomy with reimplantation should be performed. Mastectomy should be performed for unresolving symptoms, necrosis or infection.     

隆突性皮肤纤维肉瘤的治疗进展

隆突性皮肤纤维肉瘤是一种罕见的皮肤恶性肿瘤,术后高复发率是其治疗的难题,彻底切除瘤体并尽可能多地保留正常组织是治疗该病的关键。术前三维重建技术已逐渐应用于肿瘤治疗领域,可用于辅助术前详细规划并指导术中精准切除瘤体。本文对隆突性皮肤纤维肉瘤的诊疗现状进行综述,并着重探讨了术前三维重建技术应用于隆突性皮肤纤维肉瘤诊疗中的价值,以期为该疾病的精准治疗提供新的思路。     

Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence; however, it seldom metastasizes. Most lesions occur over the trunk or proximal extremities, and there is slight male preponderance. DFSP is rare on the head and neck. Less than 5% tumors are located on the scalp. OBJECTIVE: To describe a case of giant fibrosarcoma arising in DFSP on the scalp during pregnancy. METHOD: In our patient, the tumor was on scalp and measured 15 x 11 x 7 cm. The recurrent tumor that developed in the same region was measured 33 x 30 x 15 cm. Histopathologic examination of this mass revealed fibrosarcoma. The mass was excised from the subperiosteal plane with a 3-cm free margin. Because of the high possibility of recurrence, the defect site was reconstructed with a split-thickness skin graft. RESULTS: After 1 year of follow-up, no recurrence or metastasis was detected. CONCLUSION: We present a unique case in which the person has a giant fibrosarcoma on her scalp arising from DFSP with fibrosarcomatous change during pregnancy.     

Dermatofibrosarcoma: a rare form of soft tissue. Management and review of the literature

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor of cutaneous origin of intermediate grade malignant potential. The incidence of DFSP is 0.1% of all cancers and 1% of all soft tissue sarcomas. We present the case of a 65years old female with a palpable, painful mass on the right thigh. A surgical excision of the lesion was done and the histopathology, as well as the immunohistochemical analysis with CD-34, confirmed the diagnosis of DFSP. Two years later, the patient is free of disease and no local recurrences or metastases have been found. Wide radical excision is the preferred surgical method for therapy of DFSP without distant metastasis. Furthermore, DFSP resists to conventional chemotherapy and radiation therapy, while, in cases of metastasis, therapy depends on cytogenesis and molecular biology of the tumor, so new therapeutic strategies are under research.     

Breast Hamartoma: a Case Report

Breast hamartoma is a benign and rare tumour consisting of glandular fat and fibrous tissue. A 36-year old woman was admitted to our clinic, complaining of a mass in her right breast since two months. Ultrasonographic and mammographic examinations revealed a 10 cm mass diagnosed as fibroadenolipoma. Total excision of the mass was performed without any complication. Histopathological examination revealed fibroadenomatous hamartoma.     

The unusual mammographic appearance of breasts augmented by autologous fat injection

We report the case of a woman presenting to our centre with two breast lumps, 12 months after undergoing breast augmentation with autologous fat injection. Mammography showed a very unusual appearance, with most of the breast parenchyma displaced anteriorly by a large amount of fatty tissue. This was associated with extensive bizarre microcalcification, highlighting the difficulty in mammographic evaluation of this rare type of breast augmentation.     

Dermatofibrosarcoma Protuberance of the Breast: a Diagnostic Challenge

Dermatofibrosarcoma protuberans (DFSP) is an uncommon slow growing neoplasm of the dermis with tendency to invade the subcutaneous tissues. It presents during the third to fourth decade of life and is commonly seen over the trunk, extremities and head and neck. DFSP presenting as a breast lump is rare but few cases have been reported in the literature. Pre-operative diagnosis with mammography, ultrasonography and FNAC is challenging. We report a case of a DFSP of the right breast in a middle aged lady with history of recurrent breast lumps excised and diagnosed in the past as benign. She presented with progressively increasing right breast lump of 2 months duration. She underwent wide local excision and histology revealed dermatofibrosarcoma protuberans. In view of its local aggressiveness with incomplete surgical margin, mastectomy was performed.     

Metaplastic spindle cell carcinoma of the breast in a patient with neurofibromatosis type 1

We report a unique case of a 67‐year‐old woman with neurofibromatosis type 1, who was also diagnosed with metaplastic spindle cell carcinoma of the left breast. She had many neurofibromatosis lesions on her body, as well as the mass in the left breast. After the breast mass was diagnosed as a malignant mesenchymal tumor by core needle biopsy, the patient underwent left modified radical mastectomy. Subsequently, the pathological analysis of the tumor showed it to be a metaplastic spindle cell carcinoma. The co‐occurrence of neurofibromatosis type 1 and breast cancer, in particular metaplastic spindle cell carcinoma, is very rare.     

Myoid hamartoma of the breast: a case report and review of the literature

Myoid hamartomas of the breast are extremely rare breast lesions, with a poorly understood pathogenesis. A 60-year-old woman presented with a palpable mass in the right breast. Breast ultrasonography and mammography showed the presence of a breast lesion with benign characters, but were not diagnostic. A local excision of the mass was performed; histology revealed that the tumor was composed of epithelial and stromal cells. Histological and immunohistochemical findings established the diagnosis of myoid hamartoma. The patient is well 4 years later without tumor recurrence. Breast myoid hamartomas are exceptionally rare benign lesions; complete surgical excision achieves cure.     

Screening mammogram microcalcification due to basal cell carcinoma of the skin

Basal cell carcinoma is the most common skin malignancy worldwide, typically presenting as a pearly or translucent papule. Histologically, approximately 21% contain areas of microcalcification. We report a case of a woman presenting with microcalcification on screening mammography that was suspicious. Repeated core biopsies did not obtain microcalcification, and a diagnostic excision of breast tissue and overlying skin revealed an intradermal basal cell carcinoma containing the microcalcification. No breast malignancy was present. We believe this is the second such reported case in the literature and the first to be reported in Europe.     

PET/CT-Detected myofibroblastoma of the breast with bizarre cells: A potential diagnostic pitfall of malignancy

Myofibroblastoma (MFB) is a rare benign mesenchymal tumor usually occurring in the breast parenchyma. This tumor can present as a palpable nodule or can be incidentally detected as a nonpalpable mass on routine screening mammogram. We first report a rare case of histologically proven MFB of the breast revealed by fluoro-deoxyglucose uptake on PET-CT examination in a patient with a lung nodule. Tumor exhibited an unusual morphology, being predominantly composed of polygonal, epithelioid, and deciduoid-like cells set in a myxoid stroma. The most striking feature was the multifocal presence of atypical/bizarre, mono/bi-nucleated cells that, in addition to diffuse myxoid stromal changes, were a concern of malignancy, especially on core biopsy. The final diagnosis of MFB was achieved on surgically resected specimen and, similarly to other benign soft tissue tumors (especially leiomyoma and schwannoma/neurofibroma), the term “bizarre cell MFB of the breast” is proposed to emphasize the degenerative/reactive nature of the atypia.     

Isolated spontaneous breast haemorrhage in a patient on anticoagulation therapy: Breast biopsy or not?

A 62‐year‐old woman presented with a hard, well circumscribed, painless breast lump of 2 weeks duration. Past history showed an episode of deep vein thrombosis 6 weeks earlier, for which she was on warfarin. Mammogram, ultrasound and needle biopsy confirmed spontaneous haemorrhagic necrosis of breast tissue, a rare site for spontaneous bleeding for patients on anticoagulants. Though concurrent use of warfarin or heparin is generally considered a contraindication to carrying out core breast biopsy, recent evidence suggests it is unnecessary to discontinue anticoagulants in patients undergoing such procedures, which is crucial in ruling out incidental malignancy, especially in elderly patients.     

Nodular fasciitis of the breast: a case report

Nodular fasciitis is a soft tissue lesion that in rare instances occurs in the breast. It can clinically and radiologically mimic malignant tumor. We describe a case of nodular fasciitis of the breast in a young woman that was misdiagnosed as phyllodes tumor. The histologic features and a review of the literature are presented. Awareness of such an entity in the breast obviates the need for unnecessary surgical intervention.     

ALK‐Positive Inflammatory Myofibroblastic Tumor of the Nipple During Pregnancy—An Unusual Presentation of a Rare Disease

Inflammatory myofibroblastic tumors (IMT) is a benign to low‐grade malignant neoplasm most commonly occurring in the viscera and soft tissues of children and young adults. Involvement of the breast is very rare. This report presents the first case of IMT of the nipple and highlights the histologic features and differential diagnosis at this unusual anatomical site. The patient was a 31‐years‐old pregnant woman with a palpable mass at the upper half of the left nipple. The lesion appeared after breastfeeding of her first child and increased in size during her second pregnancy. A conservative, incomplete surgical excision was performed in the 24th week of the second pregnancy. The residual tumor subsequently underwent spontaneous regression. There was no evidence of disease 5 years after surgery. FISH and immunohistochemical analyses revealed rearrangement and overexpression of the ALK gene, a typical feature of both pulmonary and extrapulmonary IMT.     

Mohs micrographic surgery and dermatofibrosarcoma protuberans: a multidisciplinary approach in 44 patients

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor with a high recurrence rate. The goal of this study was to review patients with DFSP treated by Mohs micrographic surgery (MMS) and to compare demographics and recurrence rates with cases in the literature treated by standard excision methods. Medical charts were used to evaluate patient and tumor characteristics, number of stages for tumor clearance, reconstructive methods used, and recurrence rate after MMS. Data were compared with published recurrence rates in the literature. Our results indicate that there were no recurrences in 44 of 44 patients with DFSP resected by MMS after a mean follow-up of 3.3 years. This compares with recurrence rates in the literature of 10% to 60% using standard excision methods. We conclude that MMS provides recurrence-free treatment of DFSP tumors and promotes a collaborative approach between dermatologic and plastic surgeons.     

Bilateral Orbital Metastases as the Presenting Finding in a Male Patient with Breast Cancer: A Case Report and Review of the Literature

Abstract: Breast cancer in men has traditionally been thought to be substantially different from that in women. As more becomes known about this relatively rare entity, the similarities between genders become more striking than the differences. Carcinoma of the male breast is an uncommon disease occurring in less than 1% of all breast cancers. Male breast carcinoma is staged similarly to female breast cancer using the American Joint Committee Clinical Staging System. As in women, axillary nodal status is the strongest predictor of outcome. Distant metastasis to bones, soft tissue, lungs, and liver have been widely reported in men with breast cancer. This case report provides a rather rare presentation of a man with breast carcinoma with bilateral orbital metastasis as an initial clinical presentation.