Case Report: Adult NTRK-Rearranged Spindle Cell Neoplasm: Early Tumor Shrinkage in a Case With Bone and Visceral Metastases Treated With Targeted Therapy

BackgroundNTRK (neurotrophic tyrosine receptor kinase)-rearranged spindle cell neoplasms are a new group of tumors included in the new 5th edition of the World Health Organization (WHO) classification of soft Tissue and Bone Sarcomas. These tumors are characterized by NTRK gene fusions and show a wide spectrum of histologies and clinical behavior. Several targeted therapies have recently been approved for tumors harboring NTRK fusions, including STS.Case PresentationA 26-year-old male with advanced, pretreated NTRK rearranged spindle cell neoplasm and liver, lung and bone metastases was treated with larotrectinib on a continuous 28-day schedule, at a dose of 100 mg twice daily. An 18FDG-PET/CT scan performed after 7 days of treatment showed tumor shrinkage in both visceral and bone lesions. There was no drug-related toxicity. Subsequent evaluations confirmed continued tumor regression in disease sites. The patient is well and continues treatment.ConclusionThe clinical and radiological response of our patient with an uncommon TPM4 (exon 7)-NTRK1 (exon 12) gene fusion tumor treated with a first-generation TRK inhibitor could contribute to a better understanding of the biology of this new STS entity and help to improve patient management.

Solitary Fibrous Tumor of the Prostate: A Case Report and Literature Review

Solitary fibrous tumors (SFTs) usually occur in the pleura and account for two-thirds of all cases; however, SFTs occurring in the prostate are extremely rare. Approximately 25 cases have been reported in the literature to date. This study reports the case of a 43-year-old man referred to our hospital with the chief complaint of a pelvic tumor after careful examination. The tumor marker levels were within normal limits. T2-weighted magnetic resonance imaging revealed a tumor, demonstrating primarily low signal intensity. It showed a capsule-like rim at the left lobe of the prostate, suggesting that the tumor was partially invading the rectal wall. Histopathological examination of needle-core biopsies showed spindle cell neoplasm with small and fusiform cells, strongly expressing signal transducer and activator of transcription 6 (STAT6) with a ramifying vascular network. Therefore, the clinical diagnosis of the patient was SFT of the prostate and robot-assisted radical prostatectomy was performed. Histopathological examination revealed that the tumor was composed of spindle cells with patternless and staghorn patterns. Immunohistochemical analysis showed a strong expression of STAT6. Furthermore, the tumor was partially positive for CD34. Therefore, the patient was diagnosed with SFT of the prostate. Two years after the initial diagnosis, the patient was alive with normal erectile function, continence status, and no evidence of the disease.

Mesonephric Like-Carcinoma (MLCA) of the Uterus with a Predominant Spindled Morphology: A Case Report

Introduction/Objective Mesonephric-like carcinoma of the uterus is an increasingly recognized carcinoma with mesonephric differentiation, but without association with mesonephric remnants. We present a case of a 60-year-old woman presented with postmenopausal bleeding. Methods/Case Report Pelvic MRI showed possibly a cervical lobulated lesion (4.4cm) extending into the endocervical canal. Initial biopsy of this mass showed a spindle cell neoplasm raising possibility of an endometrial stromal sarcoma. On subsequent radical hysterectomy, there was a mass arising in the lower uterine segment (LUS) with circumferential cervical involvement. The tumor was comprised of sheets of epithelioid to spindle cells with scant cytoplasm and indistinct cell borders. Abundant mitotic figures and foci of necrosis were identified. Focal areas showed dense sclerosis with cords of cells, and only rare areas showed tubule formation with scant secretions. By immunohistochemistry (IHC), the tumor cells were positive for keratin AE1/AE3 (strong, diffuse), TTF-1, p63, p16, CD10 (with luminal accentuation); PAX8, desmin and caldesmon showed focal/rare positivity. Other markers were negative, including GATA3 (patchy, weak), ER and PR. Mismatch repair proteins were intact. Next-generation sequencing (NGS) revealed a KRAS mutation. Considering strong expression of epithelial markers, focal tubule formation with positive TTF-1 and negative GATA-3 labeling, and absence of identified mesonephric remnants, the tumor was classified as a high-grade mesonephric-like carcinoma of the uterus (LUS). Pelvic lymph nodes were negative (pT2 N0), and the patient is receiving cisplatin and external beam radiation. Results (if a Case Study enter NA) NA Conclusion MLCA with a prominent spindled/sarcomatoid component can be difficult to diagnose. Ancillary testing including a broad IHC panel with TTF-1, GATA-3 and NGS may be useful to aid in the diagnosis.

PEDICLE ALT FLAP FOR RECONSTRUCTION OF HUGE ANTERIOR ABDOMINAL WALL DEFECT WITH USE OF CONTINUOUS LIGNOCAINE DRIP – CASE REPORT

Summary – A 83 year old male presented with lump in right lower quadrant of abdomen since 1 year. It was associated with pain since 3 months and was of pricking type. No other associated complaints. On HPE the lump was Spindle cell neoplasm. After thorough evaluation he was subjected to chemotherapy and then underwent surgery and the huge defect was reconstructed using pedicled ALT flap.

A unique case of classic pleomorphic sarcoma restricted to the toes

Over 50% of soft tissue sarcomas occurring in older adults are histologically pleomorphic and high grade. Most have traditionally been classified as malignant fibrous histiocytoma (MFH). MFH was originally defined as a malignant pleomorphic spindle cell neoplasm showing fibroblastic and histiocytic differentiation. More recently, pathologists have accepted that this morphology may be shared by a wide range of malignant neoplasms. Many sarcomas that were previously classified as pleomorphic MFH, on careful immunohistochemical and histopathologic analyses, revealed a specific line of differentiation and could be reclassified as myxofibrosarcoma (30%), myogenic sarcoma (30%), liposarcoma (4%), malignant peripheral nerve sheath tumor (2%), or soft tissue osteosarcoma (3%), whereas about 30% had no specific line of differentiation or were myofibroblastic. The term undifferentiated pleomorphic sarcoma (UPS) is now reserved for pleomorphic sarcomas that show no definable line of differentiation by current technology. The majority of extremity sarcomas occur in the lower extremity (74 vs. 26% in the upper limb). According to one of the studies conducted on 315 patients, non-metastatic soft tissue sarcoma of the lower extremity who were treated at one institution over a ten-year period. Sixty-six percent of the lesions were above the knee, and 60% were high grade. This case had a 3x3 cm ulcer at the 3rd toe in a 30-year-old male patient who subsequently underwent midfoot ampuatation.

Solitary Fibrous Tumor of Internal Jugular Vein: An Extremely Rare Entity with Review of Literature

Solitary fibrous tumor (SFT) is an unusual spindle cell neoplasm that commonly arises from pleura. In the last decade, multiple case reports have described its diverse occurrence in extrapleural locations involving almost every anatomic site. Intravascular SFT is extremely rare and has been reported in inferior vena cava and renal vein only, to the best of our knowledge. SFT of the internal jugular vein has never been reported. We present a case of a SFT arising from internal jugular vein with extraluminal exophytic component extending into supraclavicular fossa. It should also be considered as a differential diagnosis for neoplasm arising from the internal jugular vein.

Clinicopathological Characteristics of Three Cases with Recurrent Orbital Solitary Fibrous Tumors: A Retrospective Study and Literature Review

Purpose Solitary fibrous tumor (SFT) is a spindle cell neoplasm that rarely occurs in orbit. This study aimed to report clinical, imaging, and pathological features of three patients with recurrent orbital SFTs.Methods Clinical, imaging, and pathological data of the three patients were retrospectively reviewed, and the results were compared with those of previously reported cases with recurrent orbital SFT. Results One female and two male patients (mean age, 54 years old) were included in this study. The present cases and literature review showed that the average time to recurrence in patients who aged under 50 years old was shorter than that in those who aged over 50 years old. The most common site for recurrent orbital SFT was the retrobulbar area of the orbit (23.8%). Imaging examinations showed consistent intensity of MRI signals before and after recurrence. Immunohistochemical results of all cases revealed the expressions of CD34. The mitotic rate increased in 4/8 cases, and the percentage of Ki-67-positive cells was elevated in 5/16 cases. Conclusion These results suggested that young patients were more likely subjected to recurrent orbital SFT. The postoperative pathological diagnosis revealed that patients with recurrent orbital SFT had more nuclear abnormalities and mitotic activity, as well as a higher percentage of Ki-67-positive cells, indicating that orbital recurrent SFT tended to be malignant according to both morphological features and immunohistochemistry results.