scholarly journals Monophasic breast synovial sarcoma – case report

Mastology ◽  
2020 ◽  
Vol 30 (Suppl 1) ◽  
Author(s):  
Juliana Lopes de Aguiar Araujo ◽  
Ubiratan Wagner de Sousa ◽  
Fernanda Mabel Batista de Aquino ◽  
Luisa Gurgel de Lira ◽  
Iris Maria Rodrigues de Andrade Almeida

Introduction: Breast synovial sarcoma is extremely rare, with few cases described. It corresponds to 6–9% of soft tissue sarcomas and is more frequent in extremities (80%), trunk (8%), and abdomen (7%) in young adults. It usually does not affect the breast. Objectives: To report a rare case of monophasic breast synovial sarcoma and provide data for the global literature. Method/Case report: G.S.B., 97 years old, presented a 7 cm nodule in the left breast and negative axillary nodes. Ultrasound (US) revealed a heterogeneous nodule of 6.0 x 5.5 cm, BI-RADS 5. She did not have mammography. Core biopsy showed spindle cell neoplasm. Immunohistochemistry (IHC) indicated mesenchymal lesion, without differentiating stromal components of fibroepithelial tumor from the mesenchymal lesion. Rapid growth with ulceration and tumor bleeding were identified. Urgent mastectomy showed a malignant neoplasm of spindle cell pattern and high grade, with 12 cm, involved lateral margin, and 19 negative axillary lymph nodes. IHC of the surgical specimen indicated monophasic synovial sarcoma. Before the wide excision, she had a rapidly progressive recurrence in the sternum, making it non-resectable. During radiotherapy (RT), local progression was identified. She has been receiving chemotherapy (CT) with ifosfamide and adriamycin. No evidence of distant disease was found after 9 months of diagnosis. Results/Discussion: Synovial sarcoma corresponds to approximately 0.06% of all breast neoplasms, originating from their mesenchymal tissue, with variable epithelial differentiation. The term synovial sarcoma is inadequate, deriving from its frequent juxta-articular location. Its incidence is approximately 1.5 per 1 million individuals, with a mean age of 32 years and a male:female ratio of 1.2:1. The main histological subtypes are: classic biphasic and monophasic. Translocation t(X;18) (p11.2; q11.2) and expression of SYT/SSX gene fusion are present in more than 95% of cases. IHC shows an intense expression of vimentin and CD99, and focal of Bcl2, EMA, CKAE1-AE3, actin, and desmin, as well as negativity for S100, cytokeratins, hormone receptors, myosin, and caldesmon. The differential diagnosis is made with other spindle cell entities, such as fibromatosis, solitary fibrous tumor, myofibroblastoma, metaplastic carcinoma, and other sarcomas. Synovial sarcoma has a moderate response to chemotherapy with anthracyclines. The treatment includes wide surgical resection and RT. Metastases occur in about 50% of cases and are present at diagnosis in 16% to 25%; they are more frequent in the lung (75%), regional lymph nodes (15%), and bones (10%), tending to late recurrence and metastases. The 5-year disease-free survival is 60%. Conclusion: The heterogeneity of the disease and its low incidence hinder prospective studies addressing therapeutic options with better long-term results.

2017 ◽  
Vol 13 (01) ◽  
pp. 01
Author(s):  
Ignacio Mendoza ◽  
Ilson Sepúlveda ◽  
Geraldine Ayres ◽  
◽  
◽  
...  

Synovial sarcoma (SS) represents about 10% of all soft tissue sarcomas. It is believed that its origin would be found in cells that are related neither to ultrastructural nor to histological features of the synovial tissue. Head and neck is very rarely affected, with the lower extremities being most frequent. Complete resection with or without radiotherapy and chemotherapy is currently considered the best available therapy. This time we present the case of a patient with SS located in the infratemporal fossa, its diagnosis, treatment and evolution. According to our knowledge it is the first reported case in South America.


Mastology ◽  
2020 ◽  
Vol 30 (Suppl 1) ◽  
Author(s):  
Paula Clarke ◽  
Carolina Nazareth Valadares ◽  
Douglas de Miranda Pires ◽  
Nayara Carvalho de Sá

Introduction: Occult breast carcinoma is a rare presentation of breast cancer, with histological evidence of axillary lymph node involvement and clinical and radiological absence of malignant breast lesions. Its survival is similar to that of the usual presentation. The treatment consists of modified radical mastectomy or axillary drainage with breast irradiation, resulting in similar survival, associated with systemic therapy according to the staging. Neoadjuvant therapy should be considered in N2-3 axillary cases. Differential diagnoses of axillary lymphadenopathies include: non-granulomatous causes (reactive, lymphoma, metastatic carcinoma) and granulomatous causes (infectious – toxoplasmosis, tuberculosis, sarcoidosis, atypical mycobacteria). Objectives: To report the case of a patient who needed a differential diagnosis among the various causes of axillary lymphadenopathy. Methods: This is a literature review conducted in the PubMed database, using the keywords "granulomatous lymphadenitis", "breast sarcoidosis", "occult breast cancer". Inclusion and exclusion criteria were applied. Case report: V.F.S., female, 51 years old, was referred to an evaluation of axillary lymphadenopathy in May 2019. She was followed by the department of pulmonology due to mediastinal sarcoidosis since 2017. Physical examination indicated breasts without changes. Axillary lymph nodes had increased volume and were mobile and fibroelastic. Mammography revealed only axillary lymph nodes with bilaterally increased density, and the ultrasound showed the presence of atypical bilateral lymph nodes. Neither presented breast lesions. Axillary lymph node core biopsy was compatible with granulomatous lymphadenitis. This result corroborates the diagnosis of sarcoidosis affecting peripheral lymph nodes. The patient was referred back to the department of pulmonology, with no specific treatment since she is oligosymptomatic. Discussion: Despite the context of benign granulomatous disease, malignancy overlying the condition of sarcoidosis must be ruled out. The biopsy provided a safe and definitive diagnosis, excluding the possibility of occult breast carcinoma. The patient will continue to undergo breast cancer screening as indicated for her age and usual risk. Conclusion: In the presentation of axillary lymphadenopathy, the mastologist must know the various diagnoses to be considered. The most feared include lymphoma and carcinoma metastasis with occult primary site. A proper workup can determine the diagnosis and guide the appropriate treatment.


Rare Tumors ◽  
2020 ◽  
Vol 12 ◽  
pp. 203636132097702 ◽  
Author(s):  
April Choi ◽  
Philip M Carpenter ◽  
Shefali Chopra ◽  
Kristi M Lara ◽  
William W Tseng ◽  
...  

Spindle cell carcinoma (SpC), also known as metaplastic carcinoma—spindle cell type, is a subtype of metaplastic carcinoma. Metaplastic carcinomas of the breast are rare but are thought to be more aggressive than invasive ductal carcinomas. Due to their rarity, there are few randomized trials that can inform any standardized approaches to treatment. Treatment is instead extrapolated from other types of breast cancer or metaplastic carcinomas of different locations. Here we present the first known case report of a patient with spindle cell carcinoma of the breast successfully treated with a standard sarcoma neoadjuvant regimen of doxorubicin, ifosfamide, and mesna (AIM) that resulted in >99% necrosis of the tumor and negative margins at the time of resection.


2015 ◽  
Vol 9 (1) ◽  
Author(s):  
Mohamed T. Hafez ◽  
Basel Refky ◽  
Khaled Abd Elwahab ◽  
Mohammad Arafa ◽  
Islam Abdou ◽  
...  

2020 ◽  
Vol 4 ◽  
pp. 11-11
Author(s):  
Wai Peng Lee ◽  
Spoorthi Sudhakar Shetty ◽  
Victor Weng Leong Ng ◽  
Su-Ming Tan

2012 ◽  
Vol 15 (5) ◽  
pp. 297 ◽  
Author(s):  
Osman Nuri Tuncer ◽  
Ozan Erbasan ◽  
İlhan Golbasi

Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 ◊ 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.


2012 ◽  
Vol 12 (2) ◽  
pp. 147-150 ◽  
Author(s):  
Rita Nonnis ◽  
Panagiotis Paliogiannis ◽  
Daiana Giangrande ◽  
Vincenzo Marras ◽  
Mario Trignano

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