International Cancer Conference Journal最新文献

Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma. EMPD can be associated with an underlying malignancy of an adjacent organ, in which case the skin lesion is known as the pagetoid spread (PS) of the underlying tumor. PS, also known as secondary EMPD, has a worse prognosis than primary EMPD. The standard treatment is surgery, but the efficacy of nonsurgical treatments is unknown in inoperable cases. We present 2 cases of anal canal adenocarcinoma with PS that were treated with definitive concurrent chemoradiation therapy (CCRT). Although both cases experienced recurrence outside of the radiation field, and one died, anal function was preserved in both. In conclusion, CCRT may be a viable treatment option for inoperable anal canal cancer with PS.

Nodular fasciitis (NF) is a benign, self-limiting myofibroblastic proliferation that often mimics soft tissue sarcoma because of its rapid growth, hypercellularity, mitotic activity, and local infiltration. NF typically arises in the forearm, trunk, or upper arm, whereas finger involvement is extremely rare, accounting for 0-2% of cases. Ulceration or protrusion is even more unusual and may further resemble aggressive malignancy. We report a rare case of ulcerated NF of the finger, clinically mimicking a fungating sarcoma. A 26-year-old Japanese woman presented with a rapidly enlarging ulcerated mass on the ulnar side of the right middle finger. MRI revealed an isointense lesion on T1-weighted images and hyperintense signal on T2-weighted and T2 fat-suppressed images. Core needle biopsy showed spindle-cell proliferation in a loosely storiform pattern within a fibromyxoid stroma, with vascular fibrinoid necrosis and neutrophilic infiltration. Immunohistochemistry demonstrated smooth muscle actin positivity and negativity for CD34 and S100. Fluorescence in situ hybridization confirmed USP6 gene rearrangement, supporting the diagnosis of NF. Because of progressive growth after biopsy, marginal excision with full-thickness skin grafting was performed under local anesthesia, achieving complete removal and functional preservation. Histopathological findings were identical to those of the biopsy specimen. The postoperative course was uneventful, and there was no recurrence at the final follow-up, with full finger mobility maintained. This case highlights a rare manifestation of NF with ulceration in the finger, an entity that can closely mimic high-grade sarcoma. Awareness of this presentation and the use of molecular confirmation of USP6 gene rearrangement are crucial for accurate diagnosis and avoidance of unnecessary radical surgery, particularly in functionally and cosmetically critical locations such as the hand.

The safety data on zolbetuximab, a new CLDN18.2-targeting antibody for advanced gastric cancer (AGC), are still exiguous. We report herein a 75-year-old, male patient with CLDN18.2-positive, HER2-negative AGC with weight loss, massive nodal disease, and peritoneal carcinomatosis-related obstructive uropathy. First-line zolbetuximab plus mFOLFOX6 triggered grade 2 nausea, which was followed on day 3 by tumor lysis syndrome (TLS) and 5-fluorouracil (5-FU)-induced hyperammonemic encephalopathy. Discontinuation of 5-FU and supportive care consisting of hydration and rasburicase led to rapid clinical improvement. Chemotherapy, which was resumed after a dosage adjustment, achieved tumor shrinkage and resolved the hydronephrosis. To the best of our knowledge, the present study is the first to describe concurrent TLS and 5-FU-induced encephalopathy during the administration of a zolbetuximab-based regimen and highlights the need for proactive prophylaxis against TLS and for controlling nausea in AGC patients with a high tumor burden, baseline renal impairment, and cachexia.

Small cell carcinoma of the urinary bladder (SCCB) is a rare and aggressive malignancy with limited treatment options and a poor prognosis. We present the case of a 63-year-old man who was initially diagnosed to have non-metastatic high-grade non-muscle invasive urothelial carcinoma with sarcomatoid subtype and later developed bone metastases. A bone biopsy confirmed small cell carcinoma, and retrospective review of the original tumor revealed mixed histology comprising small cell, sarcomatoid-like, and conventional urothelial carcinoma components. The patient was treated with six cycles of cisplatin and etoposide, during which genomic profiling identified a high tumor mutational burden (22 mutations/megabase). Based on this finding, pembrolizumab was administered sequentially as monotherapy. The patient achieved a complete response that lasted for more than 1 year, but subsequently developed lymph node metastases and recurrences in bone. This case highlights the role of genomic profiling test for clinical decision-making as tumor mutational burden predicts the efficacy of immune checkpoint inhibitor therapy in SCCB. This case also underscores the urgent need for novel treatment approaches for SCCB.

High-grade glioma (HGG) arising from a mature ovarian teratoma is extremely rare and its genetic alterations remain largely unknown. We report a case of WHO Grade 4 HGG (HGG-G4) developing 3 years after cystectomy for ovarian mature teratoma, where a WHO Grade 3 HGG (HGG-G3) was identified upon pathological reevaluation. Whole-exome sequencing confirmed the clonal relationship between HGG-G3 and HGG-G4, revealing genome-wide copy-neutral loss of heterozygosity, copy-number alterations, and whole-genome doubling in both HGGs. Genomic and epigenetic analyses have suggested multistep tumorigenesis and clonal alteration during the clinical course, particularly in response to chemotherapy, in HGGs arising from ovarian teratomas.

Supplementary information: The online version contains supplementary material available at 10.1007/s13691-025-00790-x.

The prognosis of sarcoma is poor; only radical resection with a negative margin has curative potential. We report a case of liposarcoma with complete surgical resection including the abdominal aorta. A 70-year-old man visited his doctor with the chief complaint of left abdominal distention. A computed tomography scan showed a mass of 15 cm, and the patient was referred to our hospital. The inferior mesenteric artery ran through the center of the tumor. The tumor surrounded the abdominal aorta, in close to the inferior vena cava, left iliopsoas muscle, left kidney, duodenum, and marginal arteries of left colon. We diagnosed the tumor as a sarcoma originated from the mesentery membrane, with invading the retroperitoneum, but with no unresectable factors. Radical surgical resection combined with aortic resection was performed. The tumor was histopathologically diagnosed as dedifferentiated liposarcoma, and was in close to surgical margin but not exposed. The patient could be discharged without major complications. Combined resection including the abdominal aorta was considered an acceptable procedure. On the other hand, the patient had a recurrence at 10 months postoperatively and died at 12 months, and techniques to ensure surgical margin and perioperative treatment may need to be considered to improve outcomes.

Anaplastic large cell lymphoma (ALCL) accounts for 10-15% of pediatric and adolescent non-Hodgkin lymphoma cases, with lung involvement observed in 14% of cases. We present a case of primary isolated ALCL in the endobronchial region of a 14-year-old boy. He presented with fever, dyspnea on exertion, cough, and left chest pain. A chest X-ray revealed a complete cut-off of the left main bronchus, which was further evaluated by bronchoscopy, showing complete occlusion of the left main bronchus by an endoluminal mass. Bronchoscopy with electrocautery snaring was performed, and immunomorphological findings confirmed the diagnosis of primary ALCL of the left main bronchus. Clinical and radiological evaluation revealed no evidence of extra-thoracic disease. A standard chemotherapy protocol was initiated. ALCL is an uncommon neoplasm in the pediatric population and rarely presents as a primary lung tumor. However, when unusual pulmonary lesions are encountered, especially in children, ALCL should be part of the differential diagnosis.

Soft-tissue sarcoma (STS) is a rare malignancy that accounts for less than 1% of all cancers, and recent advances in molecular biology have led to its classification based on genomic information. Some RET-rearranged neoplasms have been reported to present pathological features similar to Neurotrophic Tyrosine Kinase Receptor-rearranged spindle cell neoplasms. Here, we report the first case of head and neck spindle cell sarcoma with a GOLGA5-RET fusion that demonstrated a sustained clinical response to selpercatinib, identified through targeted next-generation sequencing (NGS). The patient was a 43 year-old man with a tumor in the arytenoid region that was resected and diagnosed as a malignant spindle cell tumor. Despite initial treatment with surgical resection alone, local recurrence was confirmed, requiring salvage therapy with total laryngectomy and bilateral cervical dissection. Surgical specimen revealed a spindle tumor with a patternless pattern and collagenous stroma. Immunohistochemistry (IHC) with positivity for CD34, bcl-2 (focally), S100, and weak nuclear staining for STAT6, with absence of expression of CK AE1/3, desmin, c-kit, smooth muscle actin, myogenin, synaptophysin, and SOX10. Trk A/B/C were also negative on IHC. Following confirmation of multiple lung metastases, the patient was treated with doxorubicin monotherapy. Targeted NGS identified GOLGA5-RET rearrangement, FGF14 amplification (equivocal), CDKN2B loss, and CDKN2A loss. GOLGA5-RET rearrangements were validated through fluorescence in situ hybridization. The patient subsequently was enrolled in a phase 1/2 trial for the selective RET inhibitor selpercatinib, resulting in a sustained partial response over 5 years. Although solitary fibrous tumor (SFT) was initially considered as a differential diagnosis based on immunohistochemical findings, the lack of strong and diffuse STAT6 expression made this diagnosis unlikely. Subsequent next-generation sequencing (NGS) revealed a RET fusion, leading to the diagnosis of an RET-rearranged spindle cell neoplasm. This case highlights the importance of genomic testing for certain spindle cell sarcomas and the potential benefit of RET-specific inhibitors against RET-altered sarcomas.

Gastric cancer remains a major cause of cancer-related mortality worldwide. Systemic chemotherapy is the primary treatment strategy for unresectable or metastatic gastric cancer, but long-term survival remains poor. Zolbetuximab, a monoclonal antibody targeting claudin-18 isoform 2 (CLDN18.2), has demonstrated efficacy in combination with chemotherapy for CLDN18.2-positive gastric and gastroesophageal junction (GEJ) adenocarcinomas. In select cases, systemic therapy can lead to a significant reduction in tumor burden, enabling conversion surgery, which has been associated with favorable outcomes. However, reports on zolbetuximab-based regimens facilitating conversion surgery remain scarce. A 27 year-old man presented with progressive dysphagia, nausea, and weight loss. Endoscopy revealed a type 3 GEJ adenocarcinoma (Siewert type II), and imaging confirmed liver and lymph-node metastases. Pathological analysis identified CLDN18.2 positivity, HER2 negativity (score 1 +), mismatch repair proficiency, and CPS 1 ≤ 5. The patient was treated with six cycles of zolbetuximab-CAPOX (zolbetuximab, capecitabine, and oxaliplatin), resulting in complete regression of liver metastases and significant tumor shrinkage. PET-CT showed no distant metastases, and endoscopic evaluation confirmed tumor regression with resolution of treatment-related gastritis. Given the disappearance of non-curative factors, conversion surgery was performed. The patient underwent robot-assisted proximal gastrectomy with lower esophagectomy, D2 lymphadenectomy, and esophagogastrostomy with Delta anastomosis. Histopathological examination revealed a moderately differentiated adenocarcinoma (tub2 > por2) with residual tumor cells, classified as ypT3N1. Significant fibrosis and necrosis were observed within the tumor, with a histological response grade of 2a, indicating viable tumor cells with extensive treatment-induced changes. Surgical margins were negative, and no lymphovascular invasion was detected. The patient recovered uneventfully and was able to resume chemotherapy early, on postoperative day 20. This case demonstrates the potential of zolbetuximab-based chemotherapy in facilitating conversion surgery for CLDN18.2-positive GEJ adenocarcinoma. While clinical trials suggest reduced efficacy of zolbetuximab in GEJ cancer compared to gastric cancer, our case highlights the possibility of exceptional responses in select patients. Additionally, transient treatment-related gastritis observed during therapy raises questions about the effects of CLDN18.2 inhibition on gastric mucosal integrity. Further research is warranted to refine patient selection for CLDN18.2-targeted therapy and investigate its broader physiological effects.

Papillary tumors of the pineal region (PTPR) are rare neoplasms arising from pineal gland and occurring in less than 1% of adult patients. Surgery is reported as the main treatment option, although the role of radiotherapy, especially in the adjuvant setting, remains controversial. A young adult woman of 24 years received adjuvant radiotherapy after a subtotal resection of a PTPR. The patient was treated with helical tomotherapy for a total dose of 59.4 Gy delivered in 33 fractions with no concurrent chemotherapy. After initial follow-up MRI exams documented stable disease, later imaging reported a slight decrease in size of the treated lesion. After 2 years of follow-up the patient is in good general conditions, with no evidence of recurrence, and no major side effects from the treatment. This descriptive case highlights the role of adjuvant radiotherapy as a safe and effective treatment option to reduce the risk of local recurrence. Modern radiotherapy techniques may easily allow the delivery of higher doses to the target to reduce the incidence of disease relapse.