scholarly journals Manifestation of Pleomorphic Undifferentiated Aortic Sarcoma with Splenic Infarction: A Case Report

2020 ◽  
Vol 13 (2) ◽  
pp. 1002-1005
Author(s):  
Varun Kaushal ◽  
Behzad Amoozgar ◽  
Nabonita Barua ◽  
Shankar Chhetri ◽  
Bhaveshkumar Garsondiya
Keyword(s):  
Poor Prognosis ◽  
Spindle Cell ◽  
Tumor Resection ◽  
Splenic Infarction ◽  
Spindle Cell Sarcoma ◽  
Shortness Of Breath ◽  
Major Vein ◽  
Aortic Sarcoma ◽  
Ct And Mri

Aortic spindle cell sarcoma is a rare neoplasm with poor prognosis that is often found incidentally due to its adverse effects. CT and MRI with contrast are useful imaging modalities, but a tissue biopsy is the gold standard for diagnosis. Tumor resection is the ultimate treatment followed by chemotherapy. Our case was an adult female who presented mainly for shortness of breath, and further imaging workup demonstrated a soft tumor juxtaposed to a major vein with compressive effect. The patient’s tumor was resected, and the pathology result confirmed undifferentiated aortic sarcoma. The patient’s condition improved and she was discharged with outpatient oncology follow-up and possible treatment.

scholarly journals Well-Differentiated Extraskeletal Osteosarcoma Arising from the Retroperitoneum That Recurred as Anaplastic Spindle Cell Sarcoma

2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Hiromasa Arai ◽  
Yasushi Rino ◽  
Teppei Nishii ◽  
Norio Yukawa ◽  
Nobuyuki Wada ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Tumor Resection ◽  
Large Mass ◽  
Japanese Woman ◽  
Spindle Cell Sarcoma ◽  
Histopathological Diagnosis ◽  
High Grade ◽  
Extraskeletal Osteosarcoma ◽  
Cell Sarcoma ◽  
Well Differentiated

Extraskeletal osteosarcoma is an uncommon high-grade malignant soft tissue sarcoma. Well-differentiated extraskeletal osteosarcoma is thought to have a better prognosis than classical extraskeletal osteosarcoma, but dedifferentiation after recurrence has also been reported. We present a case of a primary retroperitoneal extraskeletal osteosarcoma in a 62-year-old Japanese woman. Abdominal CT revealed a large mass with diffuse calcification in the right retroperitoneal space and tumor resection was performed. The histopathological diagnosis was well-differentiated retroperitoneal extraskeletal osteosarcoma. She was followed up by CT every 6 months without adjuvant radiotherapy and chemotherapy for 31 months until anaplastic high-grade spindle cell sarcoma recurred in the retroperitoneum. Our case is the seventh reported description of well-differentiated extraskeletal sarcoma, and the first to arise in the retroperitoneum and recur as an entirely dedifferentiated spindle cell sarcoma.

Spindle cell hemangioendothelioma of the sacrum

2014 ◽  
Vol 21 (2) ◽  
pp. 275-278 ◽  
Author(s):  
Andrea Winter ◽  
Alan Siu ◽  
Aria Jamshidi ◽  
Martin Malawer ◽  
Jonathan H. Sherman
Keyword(s):  
Spindle Cell ◽  
Bladder Dysfunction ◽  
Percutaneous Biopsy ◽  
Vascular Tumor ◽  
Radicular Pain ◽  
Preoperative Embolization ◽  
Low Back ◽  
Open Biopsy ◽  
Ct And Mri

Spindle cell hemangioendothelioma is a rare benign vascular tumor that is not known to involve the sacrum. The authors describe the case of a 31-year-old woman presenting with low-back and radicular pain without weakness or bowel or bladder dysfunction. Admission CT and MRI studies revealed a large S1–3 lytic sacral lesion. The patient initially underwent a nondiagnostic percutaneous biopsy. She subsequently underwent an open biopsy, during which the lesion was found to be highly vascular. Histological investigation revealed a vasoformative lesion consistent with spindle cell hemangioendothelioma. Preoperative embolization followed by resection via intralesional currettage resulted in resolution of symptoms up to 9 months postintervention. Despite the authors' recommendation, the patient became pregnant at that time and requested no additional follow-up imaging. The authors present the first reported case of a spindle cell hemangioendothelioma of the sacrum and review the current literature.

Primary Endothelial Sarcoma of the Thoracic Aorta

Vascular ◽  
2004 ◽  
Vol 12 (2) ◽  
pp. 140-144
Author(s):  
Ralph I. Rückert ◽  
Birgit Rudolph ◽  
Patrik Rogalla ◽  
Michael Walter
Keyword(s):  
Thoracic Aorta ◽  
Poor Prognosis ◽  
Malignant Tumors ◽  
Polypoid Lesion ◽  
Aortic Tumor ◽  
Tumor Extirpation ◽  
Time Of Operation ◽  
Aortic Sarcoma ◽  
Primary Aortic Tumor

Primary malignant tumors of the aorta are extremely rare. The case of a 64-year-old woman who presented with peripheral embolism to both femoropopliteal arteries is reported. The search for a source revealed a polypoid lesion severely narrowing the lumen of the distal thoracic aorta. Differential diagnosis included thrombus and primary aortic tumor. Extirpation of the tumorous lesion was performed. Histologic examination revealed intimal aortic sarcoma of endothelial cell origin. Although the liver was the only site of suspected metastases at the time of operation, during the 18-month follow-up until the patient's death, generalized metastatic spread had developed. This case report thus demonstrates the generally poor prognosis of this rare variety of aortic sarcoma, in particular when symptoms have already occurred.

scholarly journals Diagnostic Enigma: Spindle Cell Sarcoma of the Aorta Presenting as Pulmonary Embolism and Chronic Anaemia

2020 ◽  
Author(s):  
Gabriella A Conte ◽  
Marjan Alidoost ◽  
Mitchel S Devita ◽  
Jonathan S Harmon ◽  
Jake W Schuler ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Spindle Cell ◽  
Signs And Symptoms ◽  
Aortic Aneurysms ◽  
Spindle Cell Sarcoma ◽  
Li Fraumeni Syndrome ◽  
Peripheral Embolization ◽  
Clinical Presentations ◽  
Chronic Anaemia ◽  
Aortic Sarcoma

Primary aortic sarcoma is a rare and aggressive malignancy with only approximately 190 cases reported in the literature. While angiosarcoma and intimal sarcomas represent an estimated 67.7% of malignant aortic tumours, spindle cell sarcomas are even more exclusive, consisting of only 0.9% of malignant aortic tumours. Differentiated from other malignant aortic tumours, spindle cell sarcomas are of mesenchymal origin and usually express vimentin and osteopontin. Clinical presentations are variable and nonspecific, ranging from back pain, abdominal pain or elevated blood pressure, misleading to differentials like pulmonary emboli or aortic aneurysms such as in our case here. In this article, we discuss the finding of an extremely rare aortic sarcoma masquerading as a pulmonary embolism. The patient underwent surgical resection; however, the course was complicated by the development of brain metastases and intracranial haemorrhage. The literature is expanding regarding the evolution of adjuvant chemotherapy and radiation therapy in the treatment of these patients. The exact pathogenesis of spindle cell sarcomas is unknown but thought to be related to the MDM2-p53 pathway. The development of spindle cell sarcomas may be related to Li-Fraumeni syndrome, which should be on the differential for these patients. This case highlights the importance of identifying aortic sarcomas in patients who present with signs and symptoms of peripheral embolization as the diagnosis can be easily misconstrued for thrombus or aortic aneurysm, leading to a delay in proper and timely management. We herein emphasize that aortic sarcomas should be included in the clinician’s working differential due to the poor prognosis and outcomes that these aggressive tumours carry.

scholarly journals Pancreatic Leiomyosarcoma With Schistosomiasis Hematobia: A Case Report and Literature Review

2021 ◽  
Vol 11 ◽  
Author(s):  
Qiang Li ◽  
Daniel Staiculescu ◽  
Yurong Zhou ◽  
Jiang Chen
Keyword(s):  
Case Report ◽  
Tumor Resection ◽  
Clinical Manifestations ◽  
Malignant Neoplasm ◽  
The Body ◽  
Imaging Characteristics ◽  
Magnetic Resonance Imaging Mri ◽  
The Relationship ◽  
Ct And Mri

Pancreatic leiomyosarcoma (PL) is a very rare, malignant neoplasm with a very poor prognosis. Here, we examine a novel case of PL with schistosomiasis hematobia. The patient had been initially misdiagnosed by the first magnetic resonance imaging (MRI). The second imaging examination demonstrated an enlarged heterogeneous tumor mass in the body-tail of pancreas. Following image analysis, the patient underwent a pancreatectomy, splenectomy and lymph node dissections. Sixteen months after the tumor resection, follow-up computed tomography (CT) and MRI revealed tumor metastasis in the liver and lung. PL has non-specific clinical manifestations and imaging characteristics, making early diagnosis very challenging. When it is difficult to distinguish between benign and malignant pancreatic lesions, short-term imaging follow-up is preferred. In this case report, we discuss the relationship between PL and schistosomiasis hematobia.

Malignant spindle cell tumors of the posterior fossa in children: case series and review of management

2021 ◽  
pp. 1-11
Author(s):  
Michael J. Gigliotti ◽  
Christine Mau ◽  
Charles S. Specht ◽  
Cynthia Lawson ◽  
Sarah McNutt ◽  
...  
Keyword(s):  
Literature Review ◽  
Posterior Fossa ◽  
Ewing Sarcoma ◽  
Spindle Cell ◽  
Spindle Cell Sarcoma ◽  
High Grade ◽  
Cell Sarcoma ◽  
Presenting Symptoms ◽  
Spindle Cell Tumors

OBJECTIVE The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population. METHODS The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with “posterior fossa” or “spindle cell tumors” or “Ewing sarcoma” or “high-grade” or “spindle cell sarcoma” or “leptomeningeal melanocytoma” as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome. RESULTS The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease. CONCLUSIONS A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.

scholarly journals Complete resection of a cavoatrial metastatic dedifferentiated liposarcoma under hypothermic circulatory arrest

2021 ◽  
Author(s):  
Ryumon Matsumoto ◽  
Koki Maekawa ◽  
Toshiki Fujiyoshi ◽  
Kentaro Kamiya ◽  
Jun Matsubayashi ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Poor Prognosis ◽  
Rare Case ◽  
Circulatory Arrest ◽  
Tumor Resection ◽  
Complete Resection ◽  
Hypothermic Circulatory Arrest ◽  
Tumor Mass ◽  
The Poor

A patient had undergone surgical resection twice for primary and metastatic dedifferentiated liposarcomas. Follow-up computed tomography revealed a new tumor mass located at the cavoatrial junction. Prompt surgical resection of the tumor with thrombectomy was successfully performed using cardiopulmonary bypass with hypothermic circulatory arrest. We report a rare case of a patient surviving for 8 years after the first tumor resection despite the poor prognosis of metastatic or recurrent liposarcoma.

scholarly journals High-Grade Leiomyosarcoma Arising in a Previously Replanted Limb

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Tiffany J. Pan ◽  
Liron Pantanowitz ◽  
Kurt R. Weiss
Keyword(s):  
Viral Infections ◽  
Spindle Cell ◽  
Spindle Cell Sarcoma ◽  
High Grade ◽  
Traumatic Amputation ◽  
Chronic Injury ◽  
First Case ◽  
Grade 3 ◽  
Prior Trauma

Sarcoma development has been associated with genetics, irradiation, viral infections, and immunodeficiency. Reports of sarcomas arising in the setting of prior trauma, as in burn scars or fracture sites, are rare. We report a case of a leiomyosarcoma arising in an arm that had previously been replanted at the level of the elbow joint following traumatic amputation when the patient was eight years old. He presented twenty-four years later with a 10.8 cm mass in the replanted arm located on the volar forearm. The tumor was completely resected and pathology examination showed a high-grade, subfascial spindle cell sarcoma diagnosed as a grade 3 leiomyosarcoma with stage pT2bNxMx. The patient underwent treatment with brachytherapy, reconstruction with a free flap, and subsequently chemotherapy. To the best of our knowledge, this is the first case report of leiomyosarcoma developing in a replanted extremity. Development of leiomyosarcoma in this case could be related to revascularization, scar formation, or chronic injury after replantation. The patient remains healthy without signs of recurrence at three-year follow-up.

scholarly journals Primary renal synovial sarcoma

2012 ◽  
Vol 2 (2) ◽  
pp. 44 ◽  
Author(s):  
Girish D. Bakhshi ◽  
Arshad S. Khan ◽  
Aftab S. Shaikh ◽  
Mohammad Ashraf A. Khan ◽  
Mohammad Adil A. Khan ◽  
...  
Keyword(s):  
Synovial Sarcoma ◽  
Poor Prognosis ◽  
Spindle Cell ◽  
Review Of Literature ◽  
Soft Tissue Tumours ◽  
Renal Tumours ◽  
Spindle Cell Tumours ◽  
Synovial Sarcomas ◽  
Sarcoma Patient

Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

Pediatric Non-vestibular Schwannoma

2017 ◽  
Vol 20 (3) ◽  
pp. 232-239 ◽  
Author(s):  
Cory Broehm ◽  
Alyaa Al-Ibraheemi ◽  
Karen J Fritchie
Keyword(s):  
Spindle Cell ◽  
Neurofibromatosis Type ◽  
Mitotic Rate ◽  
Vestibular Schwannomas ◽  
Spindle Cell Sarcoma ◽  
Age Group ◽  
Clinicopathologic Features ◽  
Age Range

While the clinicopathologic features of pediatric vestibular schwannomas, often in the context of neurofibromatosis type 2 (NF2), have been well studied, there is less data regarding the characteristics of pediatric non-vestibular schwannomas (NVS). Additionally, the rate of loss of SMARCB1/INI1 expression in this population has not been systematically evaluated. Our institutional archives were searched for cases of NVS arising in patients 18 years or younger. Clinicopathologic features including SMARCB1/INI1 status were assessed for each case. Twenty-three NVS from 9 males and 13 females (age range, 2 months to 18 years) were identified, and sites included paraspinal (n = 10), head and neck (n = 6), extremities (n = 4), trunk (n = 1), mediastinum (n = 1), and retroperitoneum (n = 1); 22 cases were Antoni A predominant with 6 cases comprising solely Antoni A tissue. The mitotic rate of the tumors ranged from 0 to 10/10 high-power fields (HPFs), and 3 tumors had mitotic rates of ≥4 mitoses/10 HPFs. Two tumors showed plexiform architecture. No NVS showed diffuse atypia, calcifications, microcystic/reticular architecture, epithelioid morphology, pseudoglandular change, neuroblastoma-like features, or necrosis. All tumors tested (23/23) showed retained nuclear expression of SMARCB1/INI1. Follow-up was available in 21 patients (range 1 week to 194 months), and 5 tumors recurred. Pediatric NVS have a relatively homogeneous appearance with a predominance of Antoni A areas. Pathologists should be aware that schwannomas in this age group may be cellular with mitotic rates of ≥4/10 HPFs to avoid misclassification as a spindle cell sarcoma.

Sign in / Sign up

Export Citation Format

Share Document