scholarly journals Monophasic Synovial Sarcoma of the Ethmoid Sinus: An Uncommon Differential of a Nasal Mass.

2021 ◽  
Author(s):  
Stuti Chowdhary ◽  
Raghul Sekar ◽  
Sunil Kumar Saxena ◽  
Bheemanathi Hanuman Srini
Keyword(s):  
Synovial Sarcoma ◽  
Paranasal Sinuses ◽  
Surgical Excision ◽  
Ethmoid Sinus ◽  
Adjuvant Chemoradiotherapy ◽  
Mesenchymal Tumors ◽  
Wide Range ◽  
Synovial Sarcomas ◽  
Specific Symptoms

Abstract Sino-nasal synovial sarcomas are rare mesenchymal tumors, with most reports describing those arising primarily from the paranasal sinuses. Very few have been described with an extension to the nasal cavity. Due to a range of non-specific symptoms and local aggressiveness, there tends to be confusion in diagnosis. Here we describe a case of primary sino-nasal monophasic synovial sarcoma, diagnosed by histopathology and treated by excision and adjuvant chemoradiotherapy. On follow-up, the patient remains symptom-free to date for over two years. A wide range of diagnoses should be kept in mind for nasal masses since all tissue types coexist in this region. Initial definitive surgical excision can be considered a prudent plan of management in very limited nasal sarcomas confined to one or two paranasal sinuses.

scholarly journals Synovial sarcoma of the hand-wrist: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Serenella Serinelli ◽  
Lorenzo Gitto ◽  
Daniel J. Zaccarini
Keyword(s):  
Literature Review ◽  
Synovial Sarcoma ◽  
Malignant Tumors ◽  
Surgical Excision ◽  
Caucasian Woman ◽  
Review Of The Literature ◽  
Case Presentation ◽  
Synovial Sarcomas ◽  
Uncommon Case

Abstract Background Synovial sarcomas are infrequent malignant tumors occurring mostly in adolescents and young adults. The occurrence of synovial sarcoma in the hand-wrist area is rare (4 to 8.5% of all synovial sarcomas in different studies). Case presentation This report documents an uncommon case of synovial sarcoma occurring in the hand-wrist of a 69-year-old Caucasian woman. She was subsequently treated with surgical excision and radiotherapy without recurrence after follow up. Conclusions This paper aims to characterize the demographic, pathologic, and clinical features with a literature review. The present literature review confirms that hand-wrist synovial sarcomas are more frequent among males and subjects 10 to 40 years old. Most cases in this location are usually not larger than 5 cm in size. The five-year survival rate is higher than that reported in a previous review on hand synovial sarcomas, and this suggests an improved survival in recent decades.

scholarly journals A Case of Giant Primary Pleuropulmonary Synovial Sarcoma

2020 ◽  
pp. 1-4
Author(s):  
Wickii T. Vigneswaran ◽  
Brittany C. Fields ◽  
Vijayalakshmi Ananthanarayanan ◽  
Wickii T. Vigneswaran
Keyword(s):  
Synovial Sarcoma ◽  
Soft Tissue Sarcomas ◽  
Extrapleural Pneumonectomy ◽  
Ct Guided ◽  
Prosthetic Reconstruction ◽  
Pathologic Features ◽  
Synovial Sarcomas ◽  
Primary Pulmonary Synovial Sarcoma ◽  
Rare Group

Synovial sarcomas represent an extremely rare subtype of an already rare group of malignancies, soft tissue sarcomas. Among these, primary pulmonary synovial sarcomas comprise an even smaller number, though they have become more frequently reported in the literature. This case report details a case of giant primary pulmonary synovial sarcoma in a 44-year-old male patient who presented with left-sided chest pain and shortness of breath and was found to have a large left-sided pleural effusion. No malignant cells were demonstrated on cytology of pleural fluid after thoracentesis; however, CT-guided needle biopsy of pleural nodules seen on imaging demonstrated pathologic features consistent with monophasic type synovial sarcoma. He was treated with neoadjuvant chemotherapy with minimal response; thus, he was referred for surgical management. A left extrapleural pneumonectomy with resection of the left hemi-diaphragm and Gore-Tex prosthetic reconstruction was performed. Imaging at six-month follow-up demonstrated a new nodule in the contralateral lung, suggestive of metastasis at that time and the patient later developed ascites at nine months, consistent with further intra-abdominal metastasis. Perhaps early diagnosis and aggressive multimodality therapy may have a place in the treatment of this aggressive disease.

scholarly journals Primary chordoma of the ethmoid sinus

2000 ◽  
Vol 114 (8) ◽  
pp. 627-629 ◽  
Author(s):  
S Loughran ◽  
L. Badia ◽  
V. Lund
Keyword(s):  
Paranasal Sinuses ◽  
Surgical Excision ◽  
Ethmoid Sinus ◽  
Review Of The Literature ◽  
Local Invasion ◽  
Rare Tumours ◽  
Ethmoid Sinuses ◽  
Local Surgical Excision

Primary chordoma of the paranasal sinuses are extremely rare tumours, with only a small number of cases verified and published in the literature. It appears that only five of these cases involved the ethmoid sinuses either as a primary or by local invasion, and of these documented cases only one other has been found to solely involve the ethmoid sinus. We present a case of primary ethmoid sinus chordoma treated by wide local surgical excision and present a review of the literature with regard to prevalence and treatment rationale.

Nasal haemangiopericytoma: report of two cases with literature review

1999 ◽  
Vol 113 (3) ◽  
pp. 199-206 ◽  
Author(s):  
Rémi Marianowski ◽  
Michel Wassef ◽  
Philippe Herman ◽  
Partice Tran Ba Huy
Keyword(s):  
Local Recurrence ◽  
Literature Review ◽  
Paranasal Sinuses ◽  
Surgical Excision ◽  
Lateral Rhinotomy ◽  
Vascular Tumour ◽  
Wide Surgical Excision ◽  
Incomplete Removal ◽  
Midfacial Degloving

AbstractHaemangiopericytoma (HPC) is a rare vascular tumour produced by proliferation of pericytes. One hundred and thirty-three cases of HPC have been reported in the nose and the paranasal sinuses. We present two more cases. Wide surgical excision via lateral rhinotomy, midfacial degloving, or endonasal removal is the treatment of choice. Radiotherapy has been used post-operatively in cases of incomplete removal. Life-long follow-up is required to evaluate local recurrence and late metastases.

scholarly journals Gastrointestinal Stromal Tumors of Small Intestine

2019 ◽  
Vol 05 (03) ◽  
pp. e92-e95 ◽  
Author(s):  
Tanweerul Huda ◽  
Mahendra Pratap Singh
Keyword(s):  
Gastrointestinal Tract ◽  
Interstitial Cells Of Cajal ◽  
Surgical Excision ◽  
Biological Behavior ◽  
Pacemaker Cells ◽  
Mesenchymal Tumors ◽  
Stromal Tumors ◽  
Cells Of Cajal ◽  
Oncogene Protein

AbstractGastrointestinal stromal tumor (GIST) is defined as mesenchymal tumors of the gastrointestinal tract expressing proto-oncogene protein CD117. They are the most common sarcomatous tumors of the gastrointestinal tract. GISTs are presumed to arise from interstitial cells of Cajal or gastrointestinal pacemaker cells which control gut motility. They have unpredictable biological behavior. Prognosis is dependent on tumor size as well as mitotic count. Radical surgical excision is the treatment of choice. They rarely metastasize to lymph nodes. Imatinib therapy is used as an adjuvant therapy. The follow-up of patients postsurgery is not standardized.

SPEARHEAD-1: A phase 2 trial of afamitresgene autoleucel (Formerly ADP-A2M4) in patients with advanced synovial sarcoma or myxoid/round cell liposarcoma.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11504-11504
Author(s):  
Sandra P. D'Angelo ◽  
Brian Andrew Van Tine ◽  
Steven Attia ◽  
Jean-Yves Blay ◽  
Sandra J. Strauss ◽  
...  
Keyword(s):  
T Cells ◽  
Synovial Sarcoma ◽  
Phase 1 ◽  
Low Grade ◽  
Phase 2 ◽  
Round Cell ◽  
Cytokine Release ◽  
Cytokine Release Syndrome ◽  
Wide Range

11504 Background: This phase 2, open-label trial (SPEARHEAD-1; NCT04044768) is designed to evaluate the efficacy, safety, and tolerability of afamitresgene autoleucel in 45 patients (pts) with advanced/metastatic synovial sarcoma or Myxoid/Round Cell Liposarcoma (MRCLS). Methods: Eligible pts are HLA-A*02 positive with MAGE-A4-expressing tumors. Pts undergo leukapheresis for collection of autologous T-cells for processing and manufacture into afamitresgene autoleucel cells. Pts were treated with afamitresgene autoleucel doses between 1–10 × 109 transduced T-cells after receiving lymphodepleting chemotherapy. The primary endpoint is overall response rate per RECIST v1.1 by independent review. An independent Data Safety Monitoring Board reviews ongoing safety and benefit: risk during the interventional phase. Results: As of Feb 4, 2021, 32 pts received afamitresgene autoleucel. Of these pts, 59% were male, 87.5% had synovial sarcoma, the median age was 43 yrs (range: 24–73), and they had a median of 3 prior systemic lines of therapy. The MAGE-A4 antigen expression level (histoscore) ranged from 112–300, and the transduced cell dose ranged from 2.7–9.9 x 109. At the data cutoff, 25 pts were evaluable for preliminary efficacy (23 with synovial sarcoma and 2 with MRCLS) and 7 pts (5 with synovial sarcoma and 2 with MRCLS) had insufficient follow-up (<8 weeks follow-up and/or awaiting first scan). Of the 25 evaluable pts, the investigator-assessed responses were: complete response (2 pts), partial response (8 pts), stable disease (11 pts), and progressive disease (4 pts). All responses were confirmed. Nine of the 10 responders had ongoing response at the data cutoff and 3 responders had MAGE-A4 antigen histoscores <200. The most common AEs of any grade (>30% pts) were neutropenia, lymphopenia, nausea, cytokine release syndrome, leukopenia, fatigue, pyrexia, and anemia. Cytokine release syndrome of any grade occurred in 19/32 pts; 95% of those events were ≤Grade 2. No immune effector cell-associated neurotoxicity syndrome (ICANS) has been reported to date. Cytopenia (≥G3) at 4 wks post-infusion was observed in 6 pts (anemia 3 pts, neutropenia 2 pts, and thrombocytopenia 1 pt). Conclusion: These preliminary data demonstrate afamitresgene autoleucel is efficacious and well tolerated in heavily pre-treated pts. Objective responses are reported across a wide range of MAGE-A4 antigen levels and deep responses have been observed. Initial durability data is encouraging. Preliminary response data in SPEARHEAD-1 is comparable to the findings of the prior Phase 1 trial [1]. To date, the safety profile of afamitresgene autoleucel has been favorable, with mainly low-grade cytokine release syndrome and tolerable/reversible hematologic toxicities. [1]. Van Tine BA, et al. CTOS; November 18-21, 2020; Virtual. Clinical trial information: NCT04044768.

Monophasic spindle cell myxoid synovial sarcoma of the hand: promising results with marginal resection

2021 ◽  
Vol 14 (1) ◽  
pp. e238366
Author(s):  
Sandeep Vijayan ◽  
Afsal P Mohammed ◽  
Mahesh Suresh Kulkarni ◽  
Sharath Kumar Rao
Keyword(s):  
Synovial Sarcoma ◽  
Elderly Woman ◽  
Spindle Cell ◽  
Normal Function ◽  
Malignant Neoplasms ◽  
High Incidence ◽  
Male Preponderance ◽  
Synovial Sarcomas ◽  
Prompt Diagnosis

Synovial sarcomas are deep-seated, genetically distinct, malignant neoplasms seen in young adults, with a male preponderance. They have unusual clinical and pathological presentation and mimic many other sarcomas and carcinomas, making the diagnosis quite challenging. Although four variants are identified, occurrence in the hand is extremely rare and leads to significant morbidity. There is a high incidence of local recurrence and distant metastasis within the first 2 years. We report the case of an elderly woman with monophasic spindle cell synovial sarcoma who presented with painless swelling in her palm and underwent local excision of the neoplasm. At 2-year follow-up, she remains totally asymptomatic with normal function of the hand. We also attempt to give an overview about monophasic spindle cell synovial sarcoma with the differentials, which would help surgeons in prompt diagnosis and appropriate management.

Intraneural Synovial Sarcoma of the Median Nerve in a 15-Year-Old Male: A Case Report and Review of Literature

Hand ◽  
2021 ◽  
pp. 155894472097513
Author(s):  
Arthur Samia ◽  
Joshua Scarcella ◽  
Richard Zeri ◽  
Yifan Guo
Keyword(s):  
Median Nerve ◽  
Synovial Sarcoma ◽  
Thenar Eminence ◽  
Right Hand ◽  
History Of ◽  
Synovial Sarcomas ◽  
Poorly Differentiated ◽  
Web Space ◽  
The Right

There have been 8 synovial sarcomas of the median nerve reported. We report a case of a 15-year-old male with synovial sarcoma of the right-hand median nerve. Patient presented with a 2-month history of enlarging mass at the base of the right thenar eminence associated with numbness in the median nerve distribution. Physical examination revealed a soft mass over the thenar eminence and paresthesia in the median nerve distribution. He underwent excision of the tumor, which revealed a well-encapsulated lesion encompassing the median nerve, involving the first, second, and radial aspect of the third web space as well as recurrent branches of the median nerve. Following excision of the tumor, a thorough metastatic workup was negative for metastatic disease. He was staged as III, T2b, N0, M0—poorly differentiated monophasic synovial sarcoma of the right median nerve. Postoperatively the patient was started on chemotherapy and radiation. Intraneural synovial sarcoma is extremely rare. Our case is the youngest with the longest follow-up. He is currently at a status of 3 years posttreatment with no signs of recurrence and excellent use of his right hand. This case is of particular interest due to the rarity of the disease along with this being the best outcome reported in the literature to-date.

Neurilemmoma of the Nasal Cavity and Paranasal Sinuses

2021 ◽  
pp. 014556132110079
Author(s):  
Xindi He ◽  
Ying Wang
Keyword(s):  
Nasal Cavity ◽  
Paranasal Sinuses ◽  
Benign Tumor ◽  
Postoperative Radiotherapy ◽  
Surgical Excision ◽  
Complete Resection ◽  
Sinus Surgery ◽  
Complete Surgical Excision ◽  
The Mean

Objectives: Neurilemmoma in the nasal cavity and paranasal sinuses is very rare. The study aimed to improve the understanding of neurilemmoma in the nasal cavity and paranasal sinuses. Materials and Methods: The clinical data of 10 patients with neurilemmoma in the nasal cavity and paranasal sinuses treated from January 2014 to June 2019 in our hospital were retrospectively studied. Results: There were 6 females and 4 males patients in our study. The mean age was 49.5 years (range 37-77 years), and the most common clinical symptom was unilateral nasal obstruction. The site of tumor included the nasal cavity, maxillary sinus, ethmoid sinus, and sphenoid sinus. There were 2 cases with malignant neurilemmoma. Nine patients underwent functional endoscopic sinus surgery (FESS); however, 1 patient underwent FESS combined with the lateral rhinotomy for complete resection of the tumor. Two patients with malignant neurilemmoma received postoperative radiotherapy. The mean follow-up was 3.82 years (range 2-7 years). There were no incidences of tumor recurrence during the study period. Conclusions: Neurilemmoma in the nasal cavity and paranasal sinuses is a mainly benign tumor. Complete surgical excision by FESS is the only treatment option for neurilemmoma in the nasal cavity and paranasal sinuses; while malignant neurilemmoma needs postoperative radiotherapy.

scholarly journals Intraarticular Synovial Sarcoma Causing Chronic Wrist Pain: A Rare Case Report

2020 ◽  
Vol 7 (3) ◽  
pp. 141-146
Author(s):  
Farid Najdmazhar ◽  
◽  
Tina Shooshtarizadeh ◽  
Seyed Matin Sadat Kiaei ◽  
◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Synovial Sarcoma ◽  
Rare Case ◽  
Surgical Excision ◽  
Wrist Pain ◽  
Joint Involvement ◽  
Rare Case Report ◽  
Chronic Wrist Pain

Synovial Sarcoma (SS) is a rare and aggressive sarcoma that usually occurs around the knee joint; it rarely involves the hand. Joint involvement by SS is extremely rare. We report a case of wrist intraarticular SS presenting with chronic wrist pain. The patient underwent surgical excision alongside with local radiotherapy and chemotherapy. After 24 months of follow up, there was no recurrence or metastasis. Intraarticular SS should be in a differential diagnosis list of chronic wrist pain even though it is extremely rare.

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