Sarcoma Presenting Research Articles

Interdigitating dendritic cell sarcoma presenting as retroperitoneal mass and obstructive jaundice. An uncommon entity of complex diagnosis.

A 60-year-old male was admitted for obstructive jaundice secondary to a 2 cm retroperitoneal lesion. Ultrasound endoscopy (UES) with fine needle biopsy (FNB) was performed, as well as endoscopic retrograde cholangiopancreatography (ERCP) with placement of a metal stent for bile duct drainage. Initially IgG4-related disease was suspected from FNB. After 8 months of treatment the patient attended with a progression evidenced in computed tomography (CT) with retroperitoneal adenopathies and liver metastasis, with involvement of duodenum and fistulation towards hepatic angle of colon. Upper endoscopy and colonoscopy was performed. The patient underwent a cephalic duodenopancreatectomy with antrectomy and right hemicolectomy. The histological study of the specimen revealed cells of fascicular distribution, fusiform aspect, with ovoid nuclei, accompanied by a polymorphic inflammatory infiltrate with mononuclear cells. Immunohistochemistry (IHQ) was positive for S100, CD21, CD23, D2-40 and Bcl-2 and negative for CD1a, CD2, CD3, CD4, CD5, CD8, CD20, CD79a, PD1, desmine, langherin, c-kit, PDGFR. These findings were suggestive of interdigitating dendritic cell sarcoma (IDCS). Systemic chemotherapy with R-CHOP was initiated and after the first 4 cycles of treatment, retroperitoneal adenopathies regressed, with stability of metastatic liver disease.

Rare case of low-grade fibro-myxoid sarcoma presenting as a lateral neck swelling

Low-grade fibromyxoid sarcoma (LGFMS) is an uncommon malignancy. LGFMS is more likely to affect the trunk and extremities in young adults. However, sporadic cases have been reported in many areas of the head and neck region, including the oral cavity, larynx, and oropharynx. LGFMS typically shows areas of collagenized, myxoid stroma with spindle cell appearance in a rolling pattern. Accurate diagnosis is challenging because it can be mistaken for a simple neoplastic entity of round cells. A few cases of LGFMS which have been reported in the head and neck showing that LGFMS is characterized by local recurrence and metastases. Because of the rarity of the head and neck region, there are no specific treatment guidelines or follow-up. Our study presents LGFMS in a 54-year-old patient in the neck region who underwent surgery and was followed up for one year.

A rare case Ewing’s sarcoma presenting as nasal mass

A rare case Ewing’s sarcoma presenting as nasal mass

Temporal Bone Myeloid Sarcoma Presenting as Necrotizing Otitis Externa: A Case Report and Literature Review.

Myeloid sarcoma (MS) is a proliferation of immature myeloid cells that occurs extramedullary, often accompanying acute myeloid leukemia (AML). It typically presents in areas such as lymph nodes, skin, and the head and neck regions. The emergence of MS within the temporal bone is rare, and it can manifest through nonspecific symptoms. In this paper, we reported a case of A 47-year-old female presented with right-sided otalgia, otorrhea, tinnitus, reduced hearing, and ear fullness, and developed facial asymmetry. She was initially diagnosed with necrotizing otitis externa and was treated accordingly. Further investigations were conducted, leading to the diagnosis of MS. She began treatment with induction chemotherapy followed by consolidation therapy. And we reviewed the literature and included 14 MS cases with the same anatomical localization, discussing the primary presentation, immunohistochemistry, and treatment approaches of these patients. In conclusion, temporal bone MS should be considered in patients with a history of AML presenting with nonspecific symptoms. Diagnostic modalities for MS include computed tomography and magnetic resonance imaging scans. Systemic chemotherapy remains the preferred treatment approach. The complexity and diversity of temporal bone MS presentations necessitate further comprehensive research to enhance understanding and improve management strategies for this rare condition.

Unprecedented presentation of pelvic synovial sarcoma: A compelling case report

Synovial sarcoma (SS) is a rare tumor that typically arises in various body regions, with its manifestation in the abdominal-pelvic area being exceptionally uncommon. However, despite its rarity in this location, it often metastasizes to the thorax. Despite its slow growth, it can be misdiagnosed as benign in less than 10% of cases. Diagnosis necessitates identification of the SYT-SSX fusion transcript, alongside immunohistochemical and cytogenetic assessments. Treatment involves surgical resection followed by radiotherapy for localized tumors, while chemotherapy is necessary for metastatic cases. Despite treatment, synovial sarcomas have a high recurrence rate, with about half recurring within two years. We report the case of a 73-year-old patient from sub-Saharan Africa who presented to the emergency room with obstructive symptoms, revealing a mass near the anus compressing the rectum upon imaging, causing an obstructive syndrome. Despite initial tumor resection, histological analysis identified a neurofibromatous origin. Twenty months later, recurrence prompted a hospital visit, revealing thoracic metastases necessitating surgery and chemotherapy. Subsequent analysis confirmed perianal synovial sarcoma. This case underscores the rarity of pelvic-anal synovial sarcoma and emphasizes the importance of early detection and proper management. Synovial sarcoma should figure prominently on the list of differential diagnoses of high-grade pelvic masses due to the importance of adjuvant chemotherapy.

Case Report : Undifferentiated Synovial Sarcoma Presenting as a Large Thoracic Mass: A Rare and Challenging Diagnosis

Background: Undifferentiated synovial sarcoma (USS) is a rare and aggressive subtype of soft tissue sarcoma. Its presentation as a large thoracic mass poses significant diagnostic and therapeutic challenges.  Case Presentation: A 34-year-old male presented with a three-month history of progressive chest pain and hemoptysis. Imaging studies revealed a large, cystic thoracic mass with pleural effusion. Initial serological testing suggested hydatid cyst, but subsequent testing revealed negative Echinococcus (Hydatid) IgG antibodies.  Diagnostic and Therapeutic Interventions: Repeat fine-needle aspiration cytology (FNAC) was deferred due to revised ultrasound findings indicating a solid fibrocystic vascular lesion. Bronchial artery embolization (BAE) was performed to control life- threatening hemorrhage. Thoracotomy and en-bloc mass excision were subsequently undertaken.  Histopathological Diagnosis: Histopathological examination of the resected specimen revealed undifferentiated synovial sarcoma and spindle cell sarcoma.  Clinical Implications: This case highlights the importance of correlating imaging and serological findings, managing vascular complications, and the efficacy of BAE in controlling hemorrhage. The significance of thoracotomy and mass excision in achieving diagnostic clarity and therapeutic success is underscored.  Conclusion: Undifferentiated synovial sarcoma presenting as a large thoracic mass poses significant diagnostic and therapeutic challenges. A multidisciplinary approach, incorporating imaging, serological testing, and surgical intervention, is crucial for achieving optimal patient outcomes.

Sarcoma or haematoma? If only it was that simple! Part 2.

Imaging appearances and clinical presentation of soft tissue sarcoma and soft tissue haematomas are similar. It is imperative that sarcoma is differentiated from benign soft tissue lesions due to the poor outcomes and high morbidity associated with sarcoma. Part 2 of this pictorial review summarises the key ultrasound appearances of soft tissue sarcoma and soft tissue haematomas and some differential diagnoses. Ultrasound is the first line test in the investigation of soft tissue masses. With the overlapping ultrasound appearances of soft tissue sarcoma and soft tissue haematomas, it is essential that practitioners are aware of key ultrasound appearances and understand when to escalate cases for further investigation. Sound knowledge of the clinical and sonographic features of soft tissue haematomas and soft tissue sarcoma as well as recognising potential differential diagnoses is fundamental to ensuring an accurate diagnosis, timely management and improved patient outcomes.

8365 Bilateral Adrenal Hemorrhage Unveiling Adrenal Metastases In A Patient With Retroperitoneal Sarcoma

Abstract Disclosure: R.J. Shah: None. A.C. Goldberg: None. S. Jasim: None. Introduction: Metastatic disease to the adrenal gland is the most commonly occurring malignancy of the adrenal glands. Adrenal hemorrhage (AH) however is a rare but potentially fatal manifestation of malignancy. This case report highlights the unusual presentation of retroperitoneal sarcoma, manifesting as bilateral adrenal hemorrhage in the context of underlying metastases. Case Presentation: A 53-year-old man presented with a 4-week history of fatigue, intermittent abdominal pain radiating to the back, and vomiting. CT revealed bilateral adrenal masses measuring up to 8.5cm on the left side, with Hounsfield units (HU) 27, and 5.9cm on the right, HU 27, consistent with large areas of bilateral adrenal hemorrhage later confirmed on MRI. Biochemical evaluation included ACTH-stimulated cortisol, which was borderline at 17.4mcg/dl (recommended above 18-20mcg/dl), with a significantly elevated ACTH level of 103.8pg/nl (reference range 7.0-63.0pg/ml). Plasma metanephrines, electrolytes, aldosterone, and DHEA-S were normal. Although hemodynamically stable, he was started on prednisone 5mg daily as he was at high risk for primary adrenal insufficiency (AI). He was referred to endocrine surgery and further workup with PET/CT demonstrated a new FDG-avid periaortic retroperitoneal mass, with an interval increase in the size of the adrenal masses and development of hypoattenuating lesions in the kidneys and pancreas favoring aggressive metastatic disease. He underwent biopsy of the retroperitoneal mass, and pathology was consistent with high-grade sarcoma. Oncology started treatment with dexrazoxane and doxorubicin. His adrenal masses remained unchanged in size, and the patient died related to rapid progression of disease. Discussion: Spontaneous bilateral adrenal hemorrhage is rare with a reported incidence of 0.14 to 1.8%. Although most cases have been associated with sepsis, anticoagulation, hematologic disorders and trauma, it is important to maintain a high level of suspicion for underlying adrenal tumors and metastatic disease. AH can be a diagnostic challenge because of its non-specific presentation, and imaging is vital for timely recognition. Patients with bilateral AH should be managed with corticosteroids to avert AI-related fatalities. Presentation: 6/1/2024

Primary ileal myeloid sarcoma presenting with bowel obstruction: a case report

BackgroundMyeloid sarcoma (MS) is an extramedullary tumor constituted by myeloid blasts or immature myeloid cells. It frequently occurs in conjunction with acute myeloid leukemia (AML); however, it can exceptionally manifest in patients without leukemia. Here, we present a rare case of primary MS originating in the small bowel without evidence of bone marrow involvement.Case representationA 33 year-old female with no relevant medical history was admitted to our hospital with recurrent abdominal pain. Computed tomography (CT) revealed bowel obstruction due to thickening of the ileum wall, which was suspected to be an ileal tumor. Initially, ectopic endometriosis was suspected because of abdominal pain associated with the menstrual cycle and changes observed on a follow-up CT scan. The lesion could not be detected by double-balloon endoscopy. Despite conservative treatment, the obstruction persisted, and laparoscopic partial ileal resection was performed, which revealed extensive involvement of the ileum and mesentery. Additionally, the mesentery of the resected ileum was extremely thickened. Histopathological and immunohistochemical examinations of the surgical specimen indicated ileal MS. Bone marrow aspiration after discharge was negative for cytological findings of leukemia, leading to a final diagnosis of primary ileal MS. Her postoperative course was uneventful, and she is currently undergoing systemic chemotherapy tailored to AML at another hospital.ConclusionsEven though MS of the small bowel is rare and may not be considered preoperatively, similar surgical treatment to that of other small bowel malignancies can ensure proper postoperative diagnosis and appropriate chemotherapy. Given the potential need for chemotherapy, ensuring surgical safety that allows for its rapid initiation is critical.

Unusual Presentation of Myeloid Sarcoma as Thigh Mass

IntroductionMyeloid sarcoma (MS) is a tumor containing myeloid blasts, with or without maturation, involving any anatomical site other than the bone marrow. It can occur de novo or following a hematological malignancy. CaseWe report a 6-year-old female who presented with fever and a rapidly enlarging left thigh mass. The initial provisional diagnosis considered was tubercular hip arthritis with an abscess, but imaging investigations suggested neoplastic swelling. A biopsy of the mass showed a malignant small-round cell tumor. Bone marrow aspiration revealed the diagnosis of acute myeloid leukemia without the presence of blasts in the peripheral smear. ConclusionMS is a rare malignancy. The diagnosis is often delayed due to a lack of clinical suspicion and the absence of blasts in peripheral blood. This report aims to enhance the awareness of pediatric nurse practitioners that MS should also be considered as a differential for an extremity mass.

Sarcoma or haematoma? If only it was that simple! Part 1.

Imaging appearances and clinical presentation of soft tissue sarcoma and soft tissue haematomas are similar. It is imperative that sarcoma is differentiated from benign soft tissue lesions due to the poor outcomes and high morbidity associated with sarcoma. Part 1 of this pictorial review will summarise the paucity of guidance in management of suspected haematomas, the clinical features and ultrasound techniques used in the assessment of soft tissue masses. Ultrasound is the first-line test in the investigation of soft tissue masses. With the overlapping ultrasound appearances of soft tissue sarcoma and soft tissue haematoma, thorough and methodical clinical examination and scanning technique is fundamental so that practitioners understand when to escalate cases for further investigation. The clinical assessment and clinical history taking into consideration the intensity of trauma and ecchymosis must correlate with the ultrasound appearances. This will facilitate an accurate diagnosis, timely management and improved patient outcomes.

Gastric Undifferentiated Pleomorphic Sarcoma Presenting as a Rapidly Growing Subepithelial Tumor

A 48-year-old man presented to our hospital for evaluation of melena. Abdominal computed tomography (CT) revealed an endophytic contrast-enhanced mass (approximately 9 cm) in the gastric fundus and upper body. CT showed no evidence of metastasis to other organs. Endoscopic examination revealed a large mass accompanied by an exudate and signs of surface hemorrhage. The shape of the mass resembled that of a subepithelial tumor, and >50% of the mucosa was peeled off. Endoscopic ultrasonography revealed hyperechoic spreading and dotor rod-shaped anechoic lesions arranged within an overall hypoechoic mass. The patient underwent total gastrectomy with lymph node dissection and was diagnosed with undifferentiated pleomorphic sarcoma without lymph node metastasis. Undifferentiated pleomorphic sarcoma, a high-grade aggressive soft tissue sarcoma, predominantly involves the extremities and rarely the stomach. It is usually asymptomatic but tends to grow rapidly. Endoscopic imaging performed a year prior to presentation was unremarkable. We report a case of a gastric undifferentiated pleomorphic sarcoma that presented as a rapidly growing subepithelial tumor. Malignancies other than a gastrointestinal stromal tumor should be considered in the differential diagnosis in patients presenting with a rapidly growing gastric subepithelial tumor.

Rare presentation of primary intra-articular synovial sarcoma of knee joint with insights from literature review

ABSTRACT Synovial sarcoma is a rare and malignant soft tissue tumor, comprising 5%–10% of such cases. This tumor primarily afflicts adolescents and young adults, with slight male bias, often near large joints like the knee and hip. Intra-articular synovial sarcoma, an even rarer subset, can delay diagnosis due to its slow growth and vague clinical signs. We present a unique case of primary knee intra-articular synovial sarcoma in a 24-year-old female. Imaging revealed a distinct lesion adjacent to the medial femoral condyle and patellofemoral joint, initially suggestive of synovial haemangioma. However, histopathology confirmed biphasic synovial sarcoma, characterized by spindle and gland-like epithelial components. Immunohistochemistry further supported the diagnosis. Reviewing documented cases, we find that synovial sarcoma can affect a wide age range but often occurs in the knee joint, emphasizing the need for vigilance. Early detection and intervention are crucial for improved treatment outcomes in this rare but challenging malignancy.

Ewing sarcoma presenting in the lung: a case report

BackgroundEwing sarcoma is a malignant round-cell tumor that primarily affects bones in children. It can also arise in extraosseous tissues, such as the lung, kidneys, and liver. The presentation symptoms of Ewing sarcoma may include cough, dyspnea, and chest pain.Case presentationThis report details the history of a 15-year-old Syrian boy with a previous diagnosis of Hodgkin lymphoma who presented with chronic shoulder pain. Imaging studies revealed an 80 mm mass in the apex of the left lung, which was confirmed through histopathological examination to be Ewing sarcoma following a computed-tomography-guided biopsy. The patient received multiple cycles of chemotherapy and subsequently underwent surgical resection of the remaining mass.ConclusionsThis case highlights the rare occurrence of Ewing sarcoma in the lung and the unusual clinical presentation of shoulder pain without other accompanying symptoms.

Unusual Clinical Presentation of Clear Cell Sarcoma Masquerading as Cutaneous Lymphoma, in a Young Male

Background: Clear cell sarcoma of soft tissue is an exceedingly rare tumor that originates from the neural crest cells. These tumors are histologically characterized by the presence of clear cells that contain accumulated glycogen. Clear cell sarcomas share histological and immunohistochemical features with malignant melanoma. They commonly arise from tendinous sheaths and aponeuroses, with the lower limbs, particularly around the ankles, being the most common site. Tumors arising in the upper extremities are rare, and very few primary clear cell sarcoma cases have been described in the chest wall and scapular soft tissues. They usually present as a solitary mass. Materials and Methods: Here we are reporting a case of clear cell sarcoma in a young boy, presenting with multifocal lesions involving the trunk, both upper and lower extremities, lymph nodes, bone marrow, and many internal viscera. Results: The case described an unusual presentation of clear cell sarcoma with multifocal involvement, which is rare compared to the typical presentation as a solitary mass. Conclusion: The reported case demonstrates a very unusual presentation of clear cell sarcoma involving multiple sites, highlighting the importance of considering a wide differential diagnosis when encountering multifocal soft tissue lesions.

Retroperitoneal soft tissue sarcoma, a rare mimic and important differential of iliopsoas abscess

This case report highlights the diagnostic challenges posed by retroperitoneal soft tissue sarcoma (STS) presenting as an iliopsoas abscess, mimicking common symptoms and radiological findings. This patients’ symptoms recurred despite multiple percutaneous drains and revisions over a period of months. Further investigation revealed a partly solid, partly cystic mass consistent with sarcoma. Surgical excision confirmed the diagnosis, but recurrence necessitated palliation. This case emphasizes the importance of differential diagnoses of iliopsoas collection and the need for research into alternative imaging modalities to aid clinicians and radiologists in differentiating benign retroperitoneal collections from malignancy.

Clear cell sarcoma metastasizing to the pancreas

In this editorial based on a case report, we delve into a seldom-seen occurrence of clear cell sarcoma featuring pancreatic metastasis in a 47-year-old male patient. Recognized for its typical tendency to metastasize to the lungs, bones, and brain, clear cell sarcoma rarely extends its reach to the pancreas. Despite the initial absence of discernible abnormalities during the patient's physical examination, the manifestation of abdominal pain prompted further investigation. Subsequent abdominal computed tomography brought to light the presence of a pancreatic tumor, culminating in the definitive diagnosis of clear cell sarcoma with pancreatic metastasis. The successful management of this atypical presentation involved a series of surgical interventions, including distal pancreatectomy and splenectomy. This report not only sheds light on the infrequent manifestation of clear cell sarcoma within the pancreas but also underscores the pivotal role of vigilant postoperative follow-up in addressing this rare sarcoma. The emphasis on postoperative care serves as a crucial aspect of the broader narrative, acknowledging the need for ongoing monitoring and management to ensure a comprehensive and successful treatment trajectory for patients with this unique presentation of clear cell sarcoma.

Combined Robotic and Vaginal Surgery for Pelvic Exenteration Due to Vaginal Sarcoma Relapse in an Obese Woman

Pelvic exenteration (PE) is an aggressive surgical procedure that implies a large hard-to-fill pelvic defect. Different reconstruction techniques were proposed to improve abdominal organ support and reduce complications (infections, pelvic organs herniation, vaginal stump dehiscence, bowel prolapse and obstruction) [1], with conflicting results [2]. Because of young age and survival greater than 50% at 5 years in patients with no residual tumor after surgery [3], a new approach with better clinical results to pelvic reconstruction is needed. The aim of this surgical film is to present an unusual presentation of vaginal sarcoma, successfully managed with a minimally invasive approach, and to illustrate our contextual multilayer technique of pelvic reconstruction using a combination of pedicled omental flap (POF) and human acellular dermal matrix (HADM). Tertiary level academic hospital. A 42-year-old obese patient with recurrent and symptomatic myxoid leiomyosarcoma, previously underwent vaginal-assisted laparoscopic surgery at a primary care center for the removal of a vaginal swelling. The multidisciplinary board determined anterior PE as the optimal therapeutic approach. Given the patient's body mass index (33 kg/m2), young age, and the favorable outcomes of robotic surgery in obese patients compared with other approaches [3,4], we proposed a combined robotic and vaginal surgery for both exenteration and reconstructive procedures [5]. During surgery, we initially explored the abdominal cavity to exclude macroscopic metastasis, followed by anterior PE. Urinary diversion was achieved with a Bricker ileal conduit by means of an ileoileal laterolateral anastomosis and an uretero-ileo-cutaneostomy. The pelvic dead space was partially filled with a POF on the left gastroepiploic artery. Subsequently, the pelvic defect was covered by a 15 × 10 mm HADM inlay inserted circumferentially at the pelvic brim, fixed with a barbed thread suture on residual pelvic structures. The final pathology confirmed the recurrence of myxoid leiomyosarcoma and indicated tumor-free resection margins. The intraoperative and postoperative periods were uneventful. The patient was discharged 14 days after surgery and underwent adjuvant doxorubicin- and dacarbazine-based chemotherapy, which was initiated 45 days after the surgery. Currently the patient is asymptomatic and disease free at the sixth month of follow-up. Robotic PE proves to be a feasible technique in obese patients, reducing postoperative hospital stay and complications. The contextual pelvic floor reconstruction with a POF and HADM supports abdominal viscera, diminishing interorgan adhesions and bowel prolapse. VIDEO ABSTRACT.

A case of a patient with embryonal sarcoma presenting with abdominal pain

A case of a patient with embryonal sarcoma presenting with abdominal pain

An unusual cystic presentation of pelvic skeletal Ewing sarcoma: a case series.

Ewing sarcoma (ES) is the second mostcommon primarymalignant bone tumour in children and adolescents. About 14.5% of primary malignancies develop in pelvic bones, wherethey typically have worse prognoses than extremity or acral sarcomas. It usually presents with aggressive features onradiology scans, but may also present with different radiological characteristics. In this series, we describe rare appearancesof pelvic skeletal Ewing sarcoma, with large extraosseous cystic component on imaging, defined by the presence of fluid-filledspaces in the extraosseous tumour lesion, which distinguishes it from the solid nature of conventional ES. We report3 cases of cystic presentation of ES, with imaging features supporting diagnosis of a primary malignant bone tumourarising from the superior pubic ramus with associated massive intrapelvic solid and cystic mass. CT-guided biopsy provideddiagnosis of ES, with large intrapelvic soft tissue and cystic component. These patients underwent neo-adjuvantchemotherapy and proton beam therapy with significant reduction in size of the solid components, while the cysticcomponents remained relatively unchanged. Two patients underwent surgical resection of the tumour (navigated P3internal hemipelvectomy and hemipelvis P2/P3 resection, respectively), and one patient died while on treatment. In both whounderwent surgery, histology showed ES with margins clear and more than 99% of treatment-induced necrosis. To theauthors' knowledge, this unusual presentation of pelvic ES is described for the first time in the literature as a case series,with particular reference to atypical extraosseous cystic changes, along with the clinical and radiological characteristics,and their treatment.