An Unusual Case of Periosteal Glomus Tumor at the Metacarpal Base Presenting as Type II CRPS: Case Report

Background: An unusual case involving a middle-aged male with a 9-year history of presumptive chronic regional pain syndrome (CRPS) and ulnar neuropathy was referred for failure in treatment. Methods: On presentation, the patient was requesting an amputation of his arm. However, work-up uncovered a periosteal extra-digital glomus tumor on the base of the small finger metacarpal. Results: Surgical excision of the lesion resulted in rapid resolution of his pain and normal hand function was ultimately restored. Conclusions: Glomus tumors account for up to 5% of all soft tissue tumors of the upper extremity, occurring most frequently within or adjacent to the nail bed. Time from onset of symptoms to correct diagnosis may not be established for many years, especially with atypical tumor locations. Although glomus tumors have been widely reported, atypical locations of these tumors should be included in the differential diagnosis for patients with unusual chronic pain or neuropathy. Furthermore, when evaluating a chronic pain patient, our findings support the opinion that assignment of the diagnosis of CRPS should only be a diagnosis of exclusion.     

Predictive Value of Immune Cell Functional Assay for Non-Cytomegalovirus Infection in Lung Transplant Recipients: A Multicenter Prospective Observational Study

IntroductionImmune cell functional assay (ImmuKnow®) is a non-invasive method that measures the state of cellular immunity in immunosuppressed patients. We studied the prognostic value of the assay for predicting non-cytomegalovirus (CMV) infections in lung transplant recipients.MethodsA multicenter prospective observational study of 92 patients followed up from 6 to 12 months after transplantation was performed. Immune cell functional assay was carried out at 6, 8, 10, and 12 months.ResultsTwenty-three patients (25%) developed 29 non-CMV infections between 6 and 12 months post-transplant. At 6 months, the immune response was moderate (ATP 225–525 ng/mL) in 14 (15.2%) patients and low (ATP < 225 ng/mL) in 78 (84.8%); no patients had a strong response (ATP ≥ 525 ng/mL). Only 1 of 14 (7.1%) patients with a moderate response developed non-CMV infection in the following 6 months compared with 22 of 78 (28.2%) patients with low response, indicating sensitivity of 95.7%, specificity of 18.8%, positive predictive value (PPV) of 28.2%, and negative predictive value (NPV) of 92.9% (AUC 0.64; p = 0.043). Similar acute rejection rates were recorded in patients with mean ATP ≥ 225 vs. <225 ng/mL during the study period (7.1% vs. 9.1%, p = 0.81).ConclusionAlthough ImmuKnow® does not seem useful to predict non-CMV infection, it could identify patients with a very low risk and help us define a target for an optimal immunosuppression.     

基于纳米材料的肿瘤消融联合免疫治疗研究进展

影像学引导消融治疗已逐渐成为治疗不可切除实体肿瘤的重要方式,但消融治疗后存在肿瘤局部复发及全身转移风险。免疫治疗可通过增强机体自身免疫细胞功能间接清除肿瘤细胞,但多数实体肿瘤对免疫治疗的反应率较低。近年来,基于纳米材料的肿瘤消融技术发展迅速,为增强局部消融效果及提高系统免疫应答提供了新的可能。本文对基于纳米材料的消融联合免疫治疗肿瘤研究进展进行综述。     

肝细胞癌合并胆管癌栓多学科诊治中国专家共识(2020版)

肝细胞癌合并胆管癌栓是肝癌的一种特殊类型,发生率为0.5%~2.5%,疾病进展快、预后差,目前国内外尚无相关的诊断与治疗共识,造成该疾病的治疗极不规范。中国医师协会肝癌专业委员会基于国内外本领域研究获得的循证医学证据,并结合我国临床实践,制订《肝细胞癌合并胆管癌栓多学科诊治中国专家共识(2020版)》。该共识针对肝细胞癌合并胆管癌栓的临床表现、诊断及分型、外科治疗、辅助治疗以及其他局部、区域性和系统性治疗进行系统阐述,旨在规范、普及和提高对肝细胞癌合并胆管癌栓的诊断和多学科治疗水平,改善该疾病总体预后。     

Expanding Awareness of the Distribution and Biologic Potential of Ectomesenchymal Chondromyxoid Tumor

Ectomesenchymal chondromyxoid tumor is a rare neoplasm of uncertain histogenesis that typically occurs in the anterior dorsal tongue. Recent reports in the literature have described rare examples of gingival, palatal and tonsillar lesions. Histologically, ectomesenchymal chondromyxoid tumors are typically well-circumscribed, lacking overtly aggressive features. Herein we report a tumor arising in the right mandible that is morphologically and molecularly consistent with ectomesenchymal chondromyxoid tumor. This case furthers awareness of the extra-glossal distribution of this neoplasm; moreover, it suggests that a subset of these tumors have the potential for locally aggressive behaviour.Electronic supplementary materialThe online version of this article (10.1007/s12105-020-01169-5) contains supplementary material, which is available to authorized users.     

Giant hepatic extra-gastrointestinal stromal tumor treated with cytoreductive surgery and adjuvant systemic therapy: A case report and review of literature

BACKGROUNDPrimary extra-gastrointestinal stromal tumors (E-GIST) of the liver are rare. The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect. Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care. However, under specific circumstances, a cytoreductive approach may represent a therapeutic option. We describe herein the case of an 84-year-old woman who presented with a tender, protruding epigastric mass. Abdominal computed tomography scan revealed a large, heterogeneous mass located across segments III, IV, V, and VIII of the liver. The initial approach was transarterial embolization of the tumor, which elicited no appreciable response. Considering the large size and central location of the tumor and the advanced age of the patient, non-anatomic complete resection was indicated. Due to substantial intraoperative bleeding and hemodynamic instability, only a near-complete resection could be achieved. Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver. Considering the risk/benefit ratio for therapeutic options, debulking surgery may represent a strategy to control pain and prolong survival.CASE SUMMARYHere, we present a case report of a patient diagnosed with E-GIST primary of the liver, which was indicated a cytoreductive surgery and adjuvant therapy with imatinib.CONCLUSIONE-GIST primary of the liver is a rare conditional, the treatment is with systemic therapy and total resection surgery. However, a cytoreductive surgery will be necessary when a complete resection is no possible.     

A case report of a rare nasopharyngeal myoepithelial dominant pleomorphic adenoma

IntroductionPleomorphic adenoma is the most common benign salivary gland neoplasm. The majority of cases occur in the major salivary glands; however, they can also originate from the minor salivary glands. The nasopharynx is an uncommon site, but it has been reported in the literature. The characteristic difference of our report from the literature is the young age of our patient and his previous unremarkable medical or surgical history.The objective of this report is to present a case of salivary gland pleomorphic adenoma, discuss radiological and histopathological findings and treatment options.Case presentationHerein we report a case of a 25-year-old male patient presenting with a long-term history of snoring, mouth breathing, and progressive left nasal obstruction. After examining the patient and confirming the diagnosis, patient successfully underwent tumor resection using a combined transnasal/transoral endoscopic approach with no complications following surgery and significant improvement of the previously reported symptoms.DiscussionPleomorphic adenoma of the minor salivary glands can occur anywhere throughout the distribution sites of these glands such as: along the upper aerodigestive tract, parapharyngeal fat spaces, soft palate, the sinonasal, and nasopharyngeal areas.ConclusionThe occurrence of pleomorphic adenoma in uncommon sites has been reported in the literature, and the nasopharynx is considered to be one of these uncommon sites. The mainstay of treatment for nasopharyngeal pleomorphic adenoma is surgical excision as they can grow to giant sizes if left untreated.     

Retrorectal cystic hamartoma: A case report

Retrorectal tumors are a rare group of tumors that can be of benign or malignant origin, the differential diagnosis concerns all retrorectal tumors. We report a case of a huge retrorectal cystic hamartoma in which surgical excision was performed.A 58 years-old female presented with a low back pain and constipation. Digital rectal examination found a renitent cystic mass compressing the posterior wall of the rectum. Colonoscopy showed a tumor compressing the rectum. Magnetic resonance imaging (MRI) scan showed a presacral cystic formation. Surgical resection using laparotomy was performed. The patient made a full recovery and was released eight days after the surgery. Histological examination of the mass revealed a retrorectal cystic hamartoma.     

Functional paraganglioma with tumor thrombus in the inferior vena cava,first case report

Pheochromocytoma (PHEO) is a rare neuroendocrine that tumor originated from the adrenal medulla that secrets catecholamines. Tumors from extra-adrenal chromaffin tissues are called extra-adrenal PHEO or paraganglioma (PGL). To our knowledge, adrenal PHEO and subclinical PGL with inferior vena cava (IVC) invasion had been sporadically reported, while functional PGL with IVC tumor thrombus has not been publicly reported yet. Perioperative management of those diseases is less well established because of their multidisciplinary nature and rarity. We herein present a case of primary malignant PGL with IVC invasion. A 16-year-old female patient with a history of severe paroxysmal hypertension was admitted to Peking Union Medical College Hospital on suspicion of retroperitoneal mass. In-house diagnostic work-up revealed a malignant PGL with IVC invasion, inferior mesenteric artery encasement and, aorta engagement. Multi-disciplinary discussions were held and careful preoperative preparation plans were made. After everything was ready, the functional PGL and tumor thrombus were completely resected, then a reconstruction of IVC was performed. The patient was discharged on postoperative day 14 and all her clinical symptoms disappeared afterward. No evidence of tumor residual or metastasis was found in the subsequent six months of follow-up. Gene tests were made for her and her family. Albeit its rarity, functional PGL with IVC invasion is not unresectable, a multi-disciplinary task force should be established to settle down every detail. We recommended 3-dimensional imaging reconstruction for gaining a better anatomic understanding. Literature reviews showed that complete resection is the premise of a good prognosis. In particular cases, complementary or alternative therapy like chemotherapy and ¹³¹I-metaiodobenzylguanidine might help, family hereditary genetic tests are advised as well.