
Introduction:The synchronous presentation of two distinct primary malignancies in the stomach, specifically gastric adenocarcinoma (GAC) and mucosa-associated lymphoid tissue (MALT) lymphoma, is an extremely rare clinical entity. These dual primary tumors challenge standard management protocols due to their differing biologies. Case Presentation:We present a case of a patient diagnosed with concurrent GAC and MALT lymphoma who was also found to have a pulmonary nodule, creating a complex staging dilemma. Surgical pathology revealed distinct neoplastic entities: gastroesophageal junction adenocarcinoma, gastric MALT lymphoma, and a pulmonary hamartoma. We provide a narrative review of selected cases from the literature to contextualize this finding. Conclusion:Our observations suggest that in such complex presentations, the prognosis is often driven by the higher-grade malignancy, necessitating a multidisciplinary approach to distinguish between metastatic disease and synchronous benign or indolent lesions.
Background:Intrathyroid thymic carcinoma (ITTC) is a low-grade malignant tumor that arises within the thyroid gland and represents a rare subtype of thyroid neoplasms. It exhibits histological and immunophenotypic features similar to those of thymic carcinoma . The first case was reported by Miyauchi et al. in 1985 . Methods:We present a case of a 60-year-old male patient with multiple bilateral cervical lymph node metastases. Results:The patient underwent an extended radical resection for bilateral thyroid cancer combined with mediastinal lymph node dissection following admission. As of the latest follow-up, 9 months have elapsed since the completion of treatment. Conclusions:This case represents a rare condition that is easily misdiagnosed as primary squamous cell carcinoma, poorly differentiated thyroid carcinoma, anaplastic carcinoma, medullary carcinoma, or other similar malignancies. Herein, we describe its imaging characteristics in detail and aim to facilitate diagnosis and treatment by integrating clinical and imaging findings.
Introduction:Pineal region tumors in children are rare and present significant diagnostic and therapeutic challenges due to their deep location, varied histology, and overlapping radiologic features. Mixed germ cell tumors (MGCTs) are particularly rare and may demonstrate complex clinical behavior. Case Presentation:We report the case of a 12-year-old boy who presented in August 2024 with progressive headache and nausea. Brain magnetic resonance imaging (MRI) revealed a large pineal mass. Biopsy with endoscopic third ventriculostomy revealed an MGCT composed of 90% immature teratoma and 10% germinoma, with a Ki-67 index of 25%. Serum alpha-fetoprotein (AFP) was mildly elevated (23.2 ng/mL), while beta-human chorionic gonadotropin levels were normal. The patient received six cycles of chemotherapy according to the ACNS1123 protocol for non-germinomatous germ cell tumors. The MRI after chemotherapy showed increased tumor size with cystic/necrotic components and normalization of AFP. Subsequent surgical resection revealed a mature teratoma without malignant elements, consistent with growing teratoma syndrome (GTS). Notably, the Ki-67 index had decreased from 25% at diagnosis to 5% after resection. Given the initial histology and lack of cerebrospinal fluid analysis, adjuvant craniospinal irradiation of 36 Gy with a 54-Gy boost to the tumor bed was delivered according to ACNS0122. Conclusion:The patient tolerated treatment well and remains in good clinical and radiological condition. This case underscores the importance of recognizing GTS and individualizing treatment in pediatric pineal MGCTs.
Background. The role of nab-paclitaxel in non-small cell lung cancer (NSCLC) maintenance therapy remains underexplored, particularly in combination with anti-angiogenic agents and immunotherapy. We present two cases of advanced NSCLC achieving prolonged progression-free survival (PFS) with nab-paclitaxel-based regimens. Case presentation. Case 1 (ALK-positive) received nab-paclitaxel+ bevacizumab every 4 weeks for more than 48 months. Case 2 (driver-negative) received nab-paclitaxel + bevacizumab + toripalimab every 6–8 weeks for 24 months. Both patients maintained partial regression disease, minimal toxicity (no grade ≥3 adverse events), and preserved quality of life. Conclusion. The literature review highlights synergistic mechanisms of chemotherapy, anti-angiogenesis, and immunotherapy, supported by trials such as IMpower150 and KEYNOTE-407. Low-dose, extended-interval nab-paclitaxel combined with anti-angiogenesis and immunotherapy may offer a feasible maintenance strategy, warranting validation in prospective trials.
BACKGROUND: Hepatocellular carcinoma (HCC) is the most common primary liver malignancy. Over the last decade, the therapeutic landscape of advanced HCC has substantially evolved following the introduction of immune checkpoint inhibitor-based combinations, including anti-programmed death ligand 1 (PD-L1) plus anti-vascular endothelial growth factor (VEGF) agents and dual immune checkpoint blockade strategies targeting programmed death 1 (PD-1) and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4). Nevertheless, the optimal timing and integration of locoregional therapies following systemic treatment remain poorly defined, particularly in patients achieving major radiological responses. In this setting, multidisciplinary evaluation is essential to guide individualized therapeutic strategies. CASE SUMMARY: Herein we present the case of a 68-year-old male with advanced HCC. First-line systemic treatment with atezolizumab plus bevacizumab was administered for six cycles, resulting in a major radiological and biochemical response. Following multidisciplinary discussion, selective transarterial embolization (TAE) was performed as consolidative locoregional treatment. At the last follow-up evaluation at 42 months, the patient remained alive with no evidence of disease recurrence. CONCLUSION: Our case highlights the potential role of a multidisciplinary therapeutic strategy in selected patients with advanced HCC. The integration of systemic immunotherapy-based treatment with consolidative locoregional therapy may enable successful downstaging and durable disease control, suggesting a possible role of leftward treatment stage migration in advanced unresectable HCC.
Objective: To investigate the clinical efficacy and safety of integrated traditional Chinese and Western medicine in the treatment of severe cutaneous immune-related adverse events (irAEs) induced by tislelizumab, to describe the diagnosis and treatment process and the thinking of traditional Chinese medicine syndrome differentiation, and to provide clinical experience and research hypotheses for the clinical management of such severe adverse events.Methods: A 71-year-old male patient with squamous cell carcinoma of the right lung was diagnosed with severe tislelizumab-induced cutaneous irAE (syndrome of intermingled damp-heat, stasis, and toxin in TCM) and treated with modified TCM formulas (oral and external application) combined with comprehensive Western medicine (glucocorticoids, antihistamines, etc.). The clinical course, TCM syndrome differentiation, treatment plan, and follow-up data were systematically analyzed, and the potential synergistic mechanism was discussed. Results: After 12 days of hospitalization and 3 months of follow-up, the patient's severe rash, pain, and pruritus were completely relieved without recurrence. The duration of glucocorticoid use was shortened (7 days of intravenous administration followed by oral tapering), and no obvious adverse reactions were observed. Conclusion:Integrated traditional Chinese and Western medicine demonstrates synergistic efficacy in the treatment of severe tislelizumab-induced cutaneous immune-related adverse events (irAEs) by regulating immune balance and eliminating pathogenic factors. It provides a valuable therapeutic option for such patients and warrants further validation in larger sample studies.
Introduction: Patients undergoing maintenance hemodialysis exhibit a unique immunological state characterized by chronic inflammation and T-cell dysfunction. Evidence regarding the efficacy and safety of combination chemoimmunotherapy in dialysis patients remains extremely limited. Case Report: We report a case of extensive-stage small cell lung cancer (ES-SCLC; performance status 1, cT1cN2M1c, stage IVB) in a patient undergoing maintenance hemodialysis who received four cycles of carboplatin (300 mg/m², day 1), etoposide (50 mg/m², days 1 and 3), and durvalumab (1,500 mg/body, day 1). Treatment was safely continued after adjusting the dosing schedule and dose levels in accordance with the dialysis schedule. Although new brain metastases developed during treatment and whole-brain radiotherapy was administered, computed tomography after four cycles demonstrated a partial response, and durvalumab maintenance therapy was subsequently continued. Grade 4 thrombocytopenia and grade 3 anemia and neutropenia were observed as adverse events; however, these were manageable with transfusion support and dose reduction. Disease progression was subsequently observed. Conclusion: Carboplatin, etoposide, and durvalumab can provide meaningful therapeutic benefit even in patients undergoing maintenance hemodialysis when adverse events are appropriately managed.
Primary mediastinal large B-cell lymphoma (PMBCL) is a rare, aggressive lymphoma with limited therapeutic options in the relapsed or refractory setting. Axicabtagene ciloleucel (axi-cel), an anti-CD19 chimeric antigen receptor (CAR) T-cell therapy, has shown promising efficacy; yet treatment decisions remain challenging in patients with rapidly progressive disease and high tumor burden. We report a 29-year-old woman with primary chemotherapy-refractory PMBCL who developed fulminant extranodal disease progression despite radiotherapy and systemic bridging therapy (BT) prior to planned axi-cel infusion. Following CAR T-cell therapy, she experienced grade 2 cytokine release syndrome (CRS) and grade 3 immune effector cell–associated neurotoxicity syndrome (ICANS), alongside clinical deterioration and radiological findings suggestive of disease progression. Notably, pronounced CAR T-cell expansion preceded biochemical improvement and subsequent radiological tumor regression, consistent with pseudoprogression rather than true treatment failure. Despite initially misleading clinical and imaging findings in the context of suboptimal baseline conditions, the patient achieved a durable complete remission and remains disease-free 23 months post-CAR T-cell therapy, with excellent quality of life. This case highlights the critical importance of recognizing immune-mediated flare phenomena to guide clinical decision-making and avoid premature treatment discontinuation or misclassification of therapeutic failure.
Introduction: The synchronous presentation of chronic myeloid leukemia (CML) and papillary thyroid carcinoma (PTC) is rare and may create important diagnostic and therapeutic challenges. Case Presentation: A 36-year-old woman presented with leukocytosis and was diagnosed with chronic-phase CML. During the initial workup, ^18F-FDG PET/CT showed diffuse marrow activity together with mild to moderate uptake in selected cervical and right paratracheal lymph nodes. Neck imaging demonstrated a nodular thyroid gland and cervical lymphadenopathy with suspicious features including cystic change and calcifications, raising concern for a synchronous second primary malignancy. Fine-needle aspiration confirmed papillary thyroid carcinoma, and nodal sampling established metastatic PTC. Hematologic stabilization with nilotinib was initiated before surgery. The patient subsequently underwent total thyroidectomy with bilateral neck dissection. Histopathology revealed multifocal PTC with lymphatic invasion, extensive nodal metastases (59/67 lymph nodes positive), and extranodal extension. Final staging was pT2N1bM0, corresponding to AJCC 8th edition Stage I. Conclusion: This case highlights the importance of careful evaluation of atypical imaging findings in patients with newly diagnosed CML and the critical role of tissue confirmation in distinguishing leukemic disease from a synchronous second primary malignancy. It also illustrates the complexity of treatment sequencing and the importance of multidisciplinary management in patients with dual primary cancers.
Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic plasma cell disorder with potential for malignant transformation, but it rarely presents with marked leukocytosis. We report a unique case of MGUS presenting as a persistent neutrophilic leukemoid reaction (LR), initially raising concern for a myeloproliferative neoplasm. A 48-year-old man was found to have sustained neutrophilic leukocytosis exceeding 30 × 10³/µL, with peripheral smear and bone marrow findings suggestive of granulocytic hyperplasia. Extensive evaluation excluded infectious, inflammatory, autoimmune, and common myeloproliferative causes, including BCR-ABL1, JAK2, and CALR mutations. Serum protein electrophoresis and immunofixation revealed an IgA lambda monoclonal gammopathy, and bone marrow biopsy demonstrated 5% lambda-restricted plasma cells, consistent with MGUS. Next-generation sequencing showed no pathogenic myeloid mutations, including CSF3R, effectively ruling out chronic neutrophilic leukemia. The neutrophilia was attributed to a paraneoplastic leukemoid reaction, likely mediated by cytokine production from clonal plasma cells. Over three years of follow-up, the patient demonstrated stable neutrophilia and gradual increases in IgA and free light chain levels without progression to myeloid malignancy. This case highlights the importance of considering plasma cell disorders in the differential diagnosis of unexplained neutrophilia and underscores the critical role of CSF3R mutational analysis in distinguishing reactive leukemoid reactions from true chronic neutrophilic leukemia.
Introduction: Fibroblast growth factor receptor 1 (FGFR1) amplification is associated with endocrine therapy resistance and a poor prognosis in hormone receptor-positive breast cancer. However, effective therapeutic options for FGFR1 amplification remain limited. We report a patient with metastatic breast cancer who was treated with pazopanib for FGFR1 amplification in the BELIEVE trial. Case Presentation: A 71-year-old woman was diagnosed with bilateral luminal-HER2 type breast cancer (pT2N1M0) 18 years previously, and underwent bilateral mastectomy and axillary lymph nodes dissection followed by adjuvant therapy. She had no family history. She developed bone and liver metastases 4 years after the operation and her condition became worse, despite multiple lines of endocrine and cytotoxic therapies. She was treated with pazopanib under the trial because comprehensive genomic profiling showed FGFR1 amplification. After 3 months, computed tomography demonstrated necrosis and shrinkage of liver metastases, consistent with a partial response. Serum tumor markers (CEA and CA15-3) showed a sustained decline, reaching their lowest levels at approximately 5 months, consistent with radiologic response. Treatment was continued for 8 months until tumor markers increased and imaging revealed disease progression. Adverse events were manageable. Conclusion: Pazopanib is likely to have a clinical benefit for patients with FGFR1-amplified breast cancer using genomic profiling in personalized oncology at the early stage of metastasis.
Introduction:Abemaciclib, a cyclin-dependent kinase 4/6 inhibitor that has the ability to penetrate breast tissue and the blood-brain barrier, has been used as an addition to endocrine therapy in breast cancer. Adverse effects like infections, nausea and vomiting, diarrhoea, fatigue, cytopenia (including neutropenia) have been mentioned quite frequently. Case Presentation:Here, we present the first report of a possible temporal association in a patient who has been treated with abemaciclib due to an unresectable breast carcinoma and developed cerebral amyloid angiopathy-related inflammation (CAA-ri). Two months after start of treatment with abemaciclib, progressive cognitive impairment was seen (with dyscalculia, behavioural changes, apraxia, and eventually bradyphrenia). Although abemaciclib dosage was reduced and eventually discontinued, the patient experienced three tonic-clonic seizures a week later and was unresponsive afterwards. A CT scan and a subsequent MRI scan showed the imaging characteristics of probable CAA-ri. Furthermore, alternative diagnoses were excluded, and after immunosuppressive therapy, clinical and radiological improvement occurred. Conclusion:Considering this clinical course, a temporal association with abemaciclib has been proposed.
Introduction:Glioblastoma (GBM) is an aggressive primary brain tumor that rarely metastasizes outside the central nervous system, with reported rates of extracranial dissemination below 2%. Increasing survival and improved diagnostic capabilities may contribute to more frequent detection of this phenomenon. Case Presentation:We describe a retrospective case series of 3 consecutive patients with histopathologically confirmed IDH-wild-type GBM who developed extracranial metastases at our institution. Clinical, radiological, pathological, and molecular data were extracted from medical records. Histopathological confirmation of metastatic disease was achieved in 2 of three patients, and an independent second-opinion pathology review was obtained for 1 case. Patient 1 (55-year-old man) developed cervical lymph node metastasis within 6 months of initial resection despite MGMT promoter methylation; the recurrent tumor contained a primitive neuroectodermal tumor-like component, immunophenotypically supported by diffuse Ki-67 positivity (∼100%) and expression of synaptophysin, chromogranin, CD56, and NSE. Patient 2 (56-year-old man) developed rapid systemic dissemination to bone, lung, liver, and the interatrial septum approximately 18 months after diagnosis; the metastatic tumor displayed mesenchymal (gliosarcoma-like) differentiation with co-expression of GFAP, SMA, and h-caldesmon, PD-L1 SP263 CPS 10/TPS 10%, retained mismatch-repair proteins, and a clinically significant TP53 c.833C>G (p.Pro278Arg) mutation identified by NGS. Patient 3 (48-year-old woman) survived 61 months despite unfavorable molecular markers, with late leptomeningeal and multiorgan dissemination. Overall survival was 8, 22, and 61 months for patients 1, 2 and 3, respectively. Conclusions:This series illustrates three distinct biological trajectories of extracranial GBM dissemination and supports the concept that prolonged survival, repeated surgical interventions, and specific molecular features (mesenchymal differentiation, PD-L1 expression, TP53 mutation) may unmask systemic metastatic potential in IDH-wild-type GBM. Clinicians should maintain a high index of suspicion for extracranial dissemination in long-term GBM survivors and in patients with atypical systemic symptoms; targeted use of whole-body 18F-FDG PET/CT and histopathological confirmation of suspicious lesions are essential for accurate diagnosis.
Introduction: Metastases to oral and maxillofacial regions are uncommon and may mimic odontogenic infection or primary intraosseous malignancy. Mandibular involvement in patients with gastric cancer is particularly rare, and delayed recognition may postpone definitive diagnosis. Case Presentation: A 61-year-old man presented with numb-chin syndrome and a nonhealing mandibular extraction socket. Panoramic radiography and CT revealed an ill-defined osteolytic lesion in the left mandible with suspected extraosseous extension. Although primary intraosseous carcinoma was initially suspected, histopathological reevaluation of the mandibular biopsy specimen suggested metastatic adenocarcinoma. 18F-FDG PET/CT demonstrated uptake in the mandible and a suspected primary lesion extending from the lower thoracic esophagus to the stomach, with multiple nodal and distant metastases. Upper gastrointestinal endoscopy revealed an ulcerated, bleeding mass at the gastroesophageal junction, and biopsy demonstrated adenocarcinoma. Based on the combined clinicopathologic, radiologic, and endoscopic findings, the lesion was diagnosed as mandibular metastasis from an advanced upper gastrointestinal adenocarcinoma, clinically managed as gastric cancer. The patient received four cycles of FOLFOX chemotherapy and achieved a partial radiologic response, but treatment was discontinued because of gastrointestinal perforation. He was transitioned to best supportive care and died 7 months after the initial diagnosis. Conclusion: Metastatic disease should be considered for patients with ill-defined mandibular osteolysis accompanied by numb-chin syndrome and a nonhealing extraction socket, even in cases without a history of malignancy. To avoid diagnostic delay, early tissue diagnosis and prompt systemic evaluation are critical.
Introduction:Cytokine release syndrome (CRS) is a rare but potentially severe immune-related adverse event associated with immune checkpoint inhibitor (ICI) therapy. CRS is characterized by high fever, hemodynamic instability, and potential multi-organ dysfunction. Pembrolizumab, an ICI, is increasingly used as a standard perioperative treatment for triple-negative breast cancer (TNBC). However, reports of CRS in TNBC remain scarce. Case Presentation:We report the case of a 63-year-old woman diagnosed with TNBC (T3N2M0, Stage IIIA) who received pembrolizumab plus chemotherapy. On day 1 of the third cycle, she developed a high fever and headache within hours of pembrolizumab administration. On day 2, hypotension, tachypnea, hypoxia, and hepatic and renal dysfunction were observed. Suspecting CRS, the patient was admitted to the intensive care unit (ICU) and treated with tocilizumab and steroid pulse therapy, resulting in rapid clinical improvement. On day 4, the patient was discharged from the ICU. She was started on oral prednisolone with gradual tapering; however, she experienced CRS recurrence. The patient underwent surgery rather than resuming chemotherapy. At 20 months postoperatively, neither breast cancer relapse nor CRS recurrence was observed. Conclusion:Early diagnosis and treatment of CRS are crucial to prevent severe multi-organ failure.
Introduction:Empagliflozin is a widely used medication for type 2 diabetes mellitus (T2DM) that can increase the risk for diabetic ketoacidosis (DKA) among susceptible patients. Pembrolizumab cancer immunotherapy can lead to immune-related adverse events such as diabetes mellitus (DM), with most of the reported cases presenting with hyperglycemic DKA. However, euglycemic DKA (eDKA) can also occur and is particularly dangerous because of the potential for delayed or misdiagnosis of DKA. Case Presentation:We report a breast cancer patient with DM receiving empagliflozin and basal insulin therapy, presenting with eDKA during neoadjuvant chemotherapy and pembrolizumab immunotherapy. The patient's condition deteriorated into a comatose state, which necessitated intubation and mechanical ventilation. She improved under intravenous fluids and insulin, returned to full consciousness, was extubated and discharged from the intensive care unit. She underwent surgery and radiotherapy and was most recently seen in the oncology unit with an excellent performance status again. Conclusion:To the best of our knowledge, we report the first case of fulminant eDKA in a breast cancer patient treated with empagliflozin, neoadjuvant chemotherapy and pembrolizumab immunotherapy according to the Keynote 522 regimen, despite basal insulin therapy initiation, highlighting the need to use this combination with caution.
Introduction:Management of recurrent high-grade serous ovarian cancer (HGSOC) remains a challenge. This case presents a successful multimodal treatment plan in a BRCA-mutated patient with repeated recurrences. Case Presentation:A 60-year-old woman diagnosed with FIGO stage III HGSOC received initial debulking surgery followed by chemotherapy at another hospital in December 2017. The patient presented to our institution in March 2019 for further management because of a suboptimal response to 2nd-line chemotherapy. A disseminated disease extent was identified. She progressed (new bone metastasis) during the 3rd- to 5th-line platinum-based chemotherapy, but achieved complete remission after changing to pembrolizumab and weekly paclitaxel. She had a 3rd relapse at the anterior mediastinum (proven by thoracoscopic resection in April 2020, and the tumor was found to harbor a BRCA2 heterozygous deletion), which was controlled by adjuvant radiotherapy combined with nivolumab and nab-paclitaxel, followed by maintenance therapy nivolumab plus olaparib, and eventually olaparib monotherapy. A 4th recurrence at the perigastric lymph nodes was found through periodic positron emission tomography surveillance with normal CA-125 in September 2021. Resection of metastasis and hyperthermic intraperitoneal chemotherapy (HIPEC) were performed, and maintenance therapy was again with olaparib. She has maintained remission since then. Conclusion:A durable remission is possible by multimodality management, including the strategic use of chemo-immunotherapy, localized resection plus HIPEC/adjuvant radiotherapy, and poly (ADP-ribose) polymerase (PARP) inhibitor maintenance, in BRCAm-HGSOC with multiple recurrences.
Introduction: Metastatic squamous cell carcinoma of the anus (SCCA) is a rare cancer with very limited treatment options. Early-phase trials of anti-PD-1 immunotherapy have demonstrated durable complete responses in a very small subset of patients. Case presentation: We report the case of a 53-year-old female presenting with heavily pretreated advanced SCCA with brain, lymph node, and lung involvement and having a complete response to single-agent pembrolizumab. Monitoring the circulating tumor tissue modified viral (TTMV) HPV DNA throughout treatment allowed for the early detection of treatment response. Conclusion: This case highlights the potential of anti-PD-1 immunotherapy in resolving advanced anal squamous cell carcinoma and the value of circulating tumor DNA monitoring as a biomarker for the early determination of treatment response.
Introduction: Hematological immune-related adverse events associated with immune checkpoint inhibitors (ICIs) are rare, and autoimmune hemolytic anemia (AIHA) is particularly uncommon. Case Presentation: A 71-year-old woman with stage IIA triple-negative breast cancer received perioperative pembrolizumab with carboplatin and paclitaxel. During the first treatment cycle, she developed acute severe anemia with laboratory findings consistent with hemolysis. Despite a negative direct antiglobulin test (DAT), major alternative causes of hemolysis were considered unlikely, leading to a clinical diagnosis of suspected ICI-associated AIHA. Oral prednisolone (1 mg/kg) promptly resolved hemolysis without recurrence. Pembrolizumab was discontinued; however, imaging demonstrated a clinical complete response, and subsequent surgery confirmed a pathological complete response (pCR). Conclusion: This case demonstrates that AIHA during ICI therapy may occur despite a negative DAT and underscores the importance of prompt recognition and corticosteroid therapy. Favorable oncologic outcomes, including pCR, may still be achieved despite early discontinuation of ICI therapy.
Introduction:Muir-Torre syndrome (MTs) is a rare autosomal dominant disorder characterized by sebaceous neoplasms and internal malignancies, most commonly colorectal and urothelial cancers. Prostate cancer is exceptionally rare in MTs, with only thirteen cases reported to date. Case Presentation:We present the case of a 65-year-old male initially diagnosed with MTs following the development of multiple sebaceous tumors and identification of a heterozygous MSH6 mutation. Immunohistochemistry demonstrated isolated loss of MSH6 protein expression, with preserved microsatellite stability - an unusual finding in MTs. Despite two negative prostate biopsies and a transurethral resection, choline positron emission tomography imaging revealed hypermetabolic pelvic and cervical lymph nodes. Excisional biopsy confirmed metastatic prostate adenocarcinoma (NKX3.1-positive). The patient received first-line androgen deprivation therapy with an LH-RH analog and apalutamide, achieving a sustained biochemical response. He remained alive and on treatment with undetectable PSA 3 years later, at the age of 74. Conclusion:This case highlights the diagnostic challenges of prostate cancer in MTs and the need to consider metastatic disease in atypical presentations, especially when MMR deficiency is suspected.