Giant Soft Tissue Sarcoma of Scalp with Skull and Cerebral Invasion

Scalp soft tissue sarcomas (STS) are very rare accounting for less than 0.1% of all malignancies. We report a rare clinical image of advanced stage soft tissue sarcoma of the scalp. A 65 year woman had presented to the surgical department with complaints of a rapidly growing swelling over the scalp for three months. On examination there was huge 20 x 20 cm swelling over the scalp in the left temporoparietal region with variegated consistency. Computed tomography of head revealed a large soft tissue mass with necrosis invading the bone and underlying brain parenchyma. Histopathological finding from core needle biopsy revealed pleomorphic sarcoma. STS are highly malignant tumors which should be diagnosed and treated using multimodality approach. Recurrences are common even after complete resection and prognosis is poor.

Multiple Undifferentiated Pleomorphic Sarcoma (Malignant Fibrous Histiocytoma) with Extradural Involvement in a 7-Year-Old Labrador Retriever

A 7-year-old castrated male Labrador retriever was referred for evaluation of progressive hind limb paresis of 4 weeks’ duration. On computed tomography and magnetic resonance imaging examination, masses were found in several regions including the lung, right kidney, and peritoneum. Additionally, an extradural mass at the region of T13–L1 was identified, which is assumed to related to the chief complaint, progressive hind limb paresis. With the consent of the owner, a dorsal laminectomy was performed to remove the mass and surrounding tissues for the palliation of the hind limb paresis. Hematoxylin and eosin staining and immunohistochemical examination revealed the mass to be consistent with an undifferentiated (high-grade) pleomorphic sarcoma. The patient presented with recurrence of the hind limb paresis, respiratory discomfort, and urinary incontinence. The owner declined treatment and the dog was euthanized. Systemic metastasis was confirmed on postmortem microscopic examination. To the authors’ knowledge, this is the first report describing multiple undifferentiated high-grade pleomorphic sarcoma with extradural involvement developing into the vertebral canal through the intervertebral space, resulting in spinal damage, in a dog.

Comparative oncology reveals DNMT3B as a therapeutic vulnerability in soft-tissue sarcoma

Undifferentiated pleomorphic sarcoma (UPS), an aggressive subtype of soft-tissue sarcoma (STS), is exceedingly rare in humans and lacks effective, well-tolerated therapies. In contrast, STS are relatively common in canine companion animals; thus, incorporation of veterinary patients into studies of UPS offers an exciting opportunity to develop novel therapeutic strategies for this rare human disease. Genome-wide studies have demonstrated that UPS is characterized by aberrant patterns of DNA methylation. However, the mechanisms and impact of this epigenetic modification on UPS biology and clinical behavior are poorly understood. Leveraging cell lines and tissue specimens derived from human and canine patients, we discovered that the DNA methyltransferase DNMT3B is overexpressed in UPS relative to normal mesenchymal tissues and associated with a poor prognosis. Consistent with these findings, genetic DNMT3B depletion strongly inhibited UPS cell proliferation and tumor progression. However, existing hypomethylating agents, including the clinically approved drug 5-aza-2'-deoxycytidine and the DNMT3B-inhibiting tool compound nanaomycin A, were ineffective in UPS due to cellular uptake and toxicity issues. Thus, further development of DNMT3B-targeting strategies for these patients is critical.

413 Challenges in the field of cardiac tumours: the surgical experience of Trieste

Aims Cardiac tumors are rare and heterogeneous entities which still remain a diagnostic and therapeutic challenge. The treatment for most cardiac tumors is prompt surgical resection. We sought to provide an overview of surgical results from a series of consecutive patients treated at our tertiary centre during almost a 20-year experience. Methods and results In this single centre study, 55 consecutive patients with cardiac tumor underwent surgical treatment from January 2002 to April 2021. Of these, 42% of patients were male and the mean age was 62 ± 12 years. Almost 27% of patients were symptomatic at the time of the diagnosis, mostly for dyspnoea and palpitations. The most frequent benign cardiac tumor was myxoma (58% of cases), occurring mainly in the left atrium (97%). Pleomorphic sarcoma was the most frequent primary malignant cardiac tumour (7.2% of cases), mainly located in the ventricles (25% left ventricle; 50% right ventricle). In all cases of benign tumors surgery was successful with no relapses. In 50% of cases of pleomorphic sarcoma relapses were observed during follow-up. After a median follow-up of 44 months, 15 (27%) patients died. While malignant tumors presented a limited survival, benign tumours showed a very good prognosis. Conclusions Cardiac tumours require a multidisciplinary work-up to guarantee a prompt diagnosis and appropriate treatment. In our surgical experience, the prognosis of benign tumours was excellent, while malignant tumours had poor outcomes despite radical surgery.

Undifferentiated Pleomorphic Sarcoma of a Chronic Burn Scar of the Knee With Lymph Node Metastasis

Malignant transformation of chronic burn scars are usually toward cutaneous lineages, including squamous cell carcinoma, basal cell carcinoma, and malignant melanoma. Sarcomas are less common. Undifferentiated pleomorphic sarcoma(UPS) is a subtype of soft tissue sarcoma with storiform-pleomorphic cells of uncertain origin, and has sparingly been reported to arise from burn scars. The majority are localized lesions probably due to the spatial distance of mesenchymal cells from the epithelium. The authors describe a rare case of UPS of the chronic burn scar of his knee with ipsilateral femoral and external iliac lymph node metastasis.

Primary soft palate biphasic synovial sarcoma - case report and literature review

BACKGROUND. Synovial sarcomas of the soft tissue are a particular type of sarcomas that rarely appear in the head and neck region. CASE REPORT AND COMMENTS. We present the case of a 27-year-old patient diagnosed in 2017 with soft palate biphasic synovial sarcoma who presented with recurrent microepistaxis, nasal obstruction, left cephalalgia and aural fullness in the left ear. The clinical examination showed a tumor with approximately 4/6 cm in diameter, covered with sero-sanguinolent secretions, pulsating in nature, completely obstructing the left choana. The surgical treatment consisted of complete removal of the tumor under endoscopic guidance with electrocauterization of the insertion area, without further postoperative radiotherapy. The histopathological aspect was suggestive for pleomorphic sarcoma, poorly differentiated, confirming the local recurrence of the tumor. The patient also presented lung metastasis from undifferentiated malignant tumor. CONCLUSION. The particularity of this case is represented by the extremely rare occurrence of synovial sarcoma in the head and neck region, especially at the level of the soft palate. Complete resection of the tumor with negative margins represent the mainstay of treatment, associated with adjuvant radiotherapy, with an important role in improving disease-specific survival.