Spindle Cell Sarcoma

Surgical margins were negative, and one-year follow-up showed that the patient remained disease-free. This case highlights the importance of immunohistochemistry and molecular testing for accurate diagnosis and appropriate management.

Current literature indicates the widespread use of pancreaticoduodenectomy; while the role of chemotherapy remains unclear. The online version contains supplementary material available at 10.1007/s13691-026-00852-8.

Histopathology showed a monomorphic spindle-cell infiltrate without atypia; immunohistochemistry (IHC) confirmed a CD34-positive, S100-negative spindle-cell neoplasm consistent with a myxoid variant of dermatofibrosarcoma protuberans. This case highlights the diagnostic utility of multimodal dermoscopy integrated with histopathology and supportive IHC in precisely evaluating atypical cutaneous tumours.

Histologically, given its morphological diversity, it needs to be differentiated from spindle cell tumors such as pancreatic gastrointestinal stromal tumors, fibromatosis, inflammatory myofibroblastic tumors, and pancreatic sclerosing epithelioid fibrosarcoma. Nuclear expression of STAT6 and the presence of NAB2: : STAT6 fusion are important characteristics of SFT.

Multiple chemotherapy regimens (ifosfamide, carboplatin, and etoposide; and doxorubicin, vincristine, cyclophosphamide, and cisplatin) demonstrated limited efficacy. Subsequent treatment with alternating albumin-paclitaxel, gemcitabine, ifosfamide, and etoposide/cyclophosphamide, irinotecan, and vincristine chemotherapy combined with anlotinib and cranial radiotherapy achieved disease stabilization, with no subsequent progression observed during follow-up. This case highlights the aggressive nature and chemoresistance of the mesenchymal components of HB, emphasizing the need for novel therapeutic approaches that incorporate targeted agents.

PI3K alterations were more frequent in Spindle cell carcinoma and squamous carcinoma This study identified high frequency of stem cell marker and targetable genetic alterations in the PI3K signalling pathway. Thus, Inhibitors of the PI3K signalling pathway are ideal candidates for targeted therapy for MBC irrespective of histological subtype.

The origin of this ganglioneuroma was likely neural crest cells of the adrenal gland. It is unknown whether the dermal masses were related to the ganglioneuroma or were unrelated sarcomas.

While the observation raises a hypothesis regarding potential systemic or hormonal influences, no receptor, molecular, or serum studies were performed to support a causal relationship. Overall, this case underscores the importance of thorough intraoperative exploration, continuous surveillance, and prompts further investigation into systemic or hormonal influences on SFT growth.

The patient was treated with VAC chemotherapy (vincristine, actinomycin D, cyclophosphamide), local radiotherapy (60 Gy), cranial prophylactic radiotherapy (12 Gy), and subsequent debulking surgery. Multimodal treatment incorporating chemotherapy, radiotherapy, surgery, and targeted maintenance therapy can achieve meaningful disease control in aggressive craniofacial MCS. To our knowledge, this represents one of the very few reported pediatric cases of maxillary MCS with confirmed HEY1::NCOA2 fusion managed with sirolimus-based maintenance therapy.

This case illustrates the diagnostic utility of MDM2 and CDK4 as specific biomarkers for cardiac intimal sarcoma. It reinforces the necessity of a multidisciplinary approach involving advanced molecular pathology to ensure accurate classification and appropriate management of rare cardiac malignancies.

Thus, although rare, synovial sarcoma occurs in the stomach and hence it should be included in the list of differential diagnoses of spindle cell sarcomas affecting stomach. A review of literature with treatment implications are discussed herewith.