Rectal inflammatory myofibroblastic tumor: Case report and literature review

Rationale:Rectal inflammatory myofibroblastic tumor (IMT) is an extremely rare mesenchymal tumor characterized by a mixture of spindle-shaped myofibroblasts or fibroblasts and inflammatory infiltration of lymphocytes and plasma cells. To date, only 8 cases of rectal IMT have been reported. Herein, we report an additional case of rectal IMT in a 28-year-old woman.Patient concerns:A 28-year-old woman presented with abdominal pain and hematochezia.Diagnoses:Colonoscopy showed a 3.0-cm subepithelial tumor with central ulceration, covered by white exudate in the rectum. Rectal magnetic resonance imaging revealed a 4.0 × 3.0-cm-sized well-defined subepithelial tumor in the right wall of the rectum, with suspicious right perirectal fat infiltration.Interventions:Laparoscopic anterior resection was performed. Microscopic examination of the surgical specimen revealed bland-looking spindle cells intermingled with lymphoplasma cells. Immunohistochemistry and fluorescence in situ hybridization showed anaplastic lymphoma kinase positivity and anaplastic lymphoma kinase positivity rearrangement. Rectal IMT was confirmed based on histological, immunohistochemical, and fluorescence in situ hybridization findings. The patient was doing well without evidence of tumor recurrence 1 year after the surgery.Lessons:Rectal IMT, despite its rarity, should be considered in the differential diagnosis of rectal cancer. Second, an accurate histopathologic diagnosis and complete surgical resection can be the most important approaches to offer a chance for the cure of rectal IMT.     

Plexiform angiomyxoid myofibroblastic tumor treated by endoscopic submucosal dissection: A case report and review of the literature

BACKGROUNDPlexiform angiomyxoid myofibroblastic tumor (PAMT) is a rare mesenchymal tumor characterized by multiple nodular plexiform growth patterns and an immunophenotype with myofibroblasts. The pathological characteristics, immunohistochemistry, diagnostic criteria, differential diagnosis, and gene-level changes of PAMT have been reported in many studies. At present, the main treatment for PAMT in the reported cases is surgery; only eight cases were treated via endoscopy (excluding 1 thoracoscopic resection), and the lesions were all smaller than 5 cm. There are no reports on the prognosis and follow-up of young patients with lesion sizes reaching 5 cm who undergo endoscopic submucosal dissection (ESD). Herein, we present the first case of a young patient with a lesion size reaching 5 cm who was diagnosed with PAMT via endoscopic submucosal dissection.CASE SUMMARYA 15-year-old young man with upper abdominal pain for 2 years presented to the Gastroenterology Department of our hospital. Painless gastroscopy showed a semicircular bulge approximately 5 cm in size in the lesser curvature near the cardia of the fundus; the surface was eroded, and shallow ulcers had formed. The pathological manifestations of the biopsy were spindle cell proliferative lesions with interstitial mucinous changes, and the surface mucosa showed chronic inflammatory changes with active lesions; immunohistochemistry showed smooth muscle actin (SMA) (+), CD117 (-), CD34 (-), DOG-1 (-), S-100 (-), and Ki67 (LI: < 1%). We performed ESD on the patient. The lesion that we removed was 5 cm × 4 cm × 2 cm in size. Pathologically, the resected tissue displayed typical manifestations, such as fat spindle-shaped fibroblasts and myofibroblast-like cells showing irregular nodular hyperplasia. Immunohistochemistry staining of the tumor cells revealed the following: CD34 (partially +), SMA (weakly +), CD117 (-), DOG-1 (-), S-100 (-), SDHB (+), PCK (-), and Ki67 (labelling index: 2%). There was no recurrence or metastasis during the 3-mo follow-up after the operation, and the treatment effect was good. We also performed a review of the literature on the clinical manifestations, pathological features, immunohistochemistry, and differential diagnosis of PAMT.CONCLUSIONAt present, the diagnostic criteria for PAMT are relatively clear, but the pathogenesis and genetic changes require further study. PAMT is benign in nature, and these patients are less likely to experience local or metastatic recurrence. The main treatment is still surgery if the lesion is in the stomach. Partial gastrectomy and distal gastrectomy are the most frequently performed surgical treatments for PAMT, followed by local resection, subtotal gastrectomy, and wedge resection. But for comprehensive evaluation of the disease, ESD can be considered a suitable method to avoid excessive treatment.     

Hepatic inflammatory pseudotumor-like follicular dendritic cell tumor with hepatic lymphoma history: A case report and literature review

Rationale:Hepatic inflammatory pseudotumor (IPT)-like follicular dendritic cell (FDC) sarcoma is a very rare disease. Till now, only 19 cases were reported in the English literature. However, the coexistence of IPT-like sarcoma and non-Hodgkin lymphoma (NHL) in the same patient has never been reported. In this report, we presented a case of hepatic IPT-like FDC with hepatic NHL history of which both were successfully resected.Patient concerns:We reported a case of a 47-year-old male patient who presented with right upper abdominal discomfort. Nineteen years ago, he underwent liver resection of segment VII for hepatic NHL (B-cell lymphoma). He had a history of chronic hepatitis B virus infection. Serum alpha fetoprotein level was normal. However, imaging studies revealed a well-circumscribed, solid mass in the right hepatic lobe, he came back to the clinic because he was worried about a recurrence of the B-cell lymphoma.Diagnoses:Based on the patient''s past medical history and magnetic resonance imaging results, and he was diagnosed as hepatocellular carcinoma or hepatic NHL preoperatively.Interventions:Right hemi-hepatectomy was performed on this patient.Outcomes:Histological report showed features of a mixture of chronic inflammatory cells and variable amounts of spindle cells. Also, immuno-histo-chemical studies demonstrated that all the tumor cells showed strong nuclear in situ labeling for EBV-encoded small RNAs and strongly positive stainings with CD21 and CD35. The patient tolerated the surgery well, recovered smoothly and he was discharged on postoperative day 7 (day 7). The patient is still disease free after a follow-up of over 50 months.Conclusions:To our knowledge, this is the first report demonstrating hepatic IPT-like FDC sarcoma in a patient with primary hepatic NHL history. In regards to treatment, complete surgical resection should be performed and would acquire excellent long-term outcomes.     

Pediatric inflammatory myofibroblastic tumor of the trachea: a case report and review of the literature

Inflammatory myofibroblastic tumors (IMTs) are relatively rare entities of a distinct histologic appearance and benign clinical course. Although these lesions have been described in virtually every anatomic location, there are few documented reports with tracheal localization. We add to the short list of pediatric IMTs of the trachea, a 13-year-old boy that was referred to our pediatric respiratory unit for evaluation of his respiratory distress. In particular, we describe the clinical and pathologic features of this patient and present a review of all reported lesions in the available literature.     

Novel treatment of endobronchial inflammatory myofibroblastic tumor in a child

Isolated endobronchial inflammatory myofibroblastic tumors (IMT) are rare, accounting for about 1% of primary endobronchial tumors in children. The mainstay of treatment for this tumor has been surgical resection. Recently, the identification of anaplastic lymphoma kinase (ALK) gene mutations in half of IMTs and promising results of treatment with ALK inhibitors in other ALK-positive tumors have opened the possibility of alternative approaches. We present a 4-year-old child with an ALK-positive endobronchial IMT, treated with endoscopic resection and neoadjuvant therapy with crizotinib, without evidence of tumor recurrence 2 years after the initial resection.     

Mesenteric inflammatory myofibroblastic tumors in an elder patient with early recurrence： A case report

Inflammatory myofibroblastic tumor （IMT） of the alimentary tract often occurs in children or young adults, but may occur at any age. Symptoms are nonspecific and depend on the location of the tumor. The most often involved sites are small bowel mesentery especially the distal ileum, mesotransverse colon, or great omentum. Recurrence appears to be more frequent in the extrapulmonary lesion. Herein we demonstrate a 63-year-old male patient with mesenteric INT, with an early recurrence after his first operation. We should be aware that if the tumor is larger than 8 cm, multinodular, omental, with ill-defined margin, with pathologically atypia, or ganglion-like cells, a close surveillence after primary surgery with image study might be necessary to detect the tumor recurrence early. Tumor recurrence may be asymptomatic, and it may act like a malignant tumor with a poor prognosis.     

Successful treatment with lorlatinib in a patient with meningeal carcinomatosis of ALK-positive non-small cell lung cancer resistant to alectinib and brigatinib: A case report

Rationale:Although anaplastic lymphoma kinase (ALK) inhibitors are effective treatment options for ALK-positive non-small cell lung cancer (NSCLC) with central nervous system (CNS) metastasis, achieving long-term survival in patients with NSCLC with meningeal carcinomatosis resistant to ALK inhibitors is difficult. Lorlatinib, a third-generation ALK inhibitor, was designed for selective CNS penetration, and exerts potent antitumor activity against tumors resistant to first- and/or second-generation ALK inhibitors. However, there is limited information about the activity of lorlatinib in ALK inhibitor-resistant meningeal carcinomatosis. Here, we report a case of ALK-positive lung adenocarcinoma with meningeal carcinomatosis in which lorlatinib was used after resistance to alectinib and brigatinib.Patients concerns:A 55-year-old woman with no history of smoking presented to our hospital with a swelling on the left neck. Clinical imaging and histopathological examination revealed a tumor of adenocarcinoma histology in the left upper lung with no CNS metastasis.Diagnoses:The patient was diagnosed with ALK-positive lung adenocarcinoma (cT3N3M1b: stage IVA).Interventions:She received the second-generation ALK inhibitors, alectinib and brigatinib, in the first and second-line settings, respectively. However, she developed meningeal carcinomatosis. Hence, treatment with lorlatinib was initiated in the third-line setting.Outcomes:The symptoms associated with meningeal carcinomatosis, such as disturbance of consciousness and diplopia, improved dramatically. At 8 months from the initiation of lorlatinib, the patient remained well without disease progression.Lessons:Lorlatinib is an effective treatment option for patient with ALK-positive NSCLC who develop meningeal carcinomatosis resistant to second-generation ALK inhibitors. Therefore, lorlatinib should be considered in such cases, even when patients exhibit serious symptoms associated with meningeal carcinomatosis.     

肝脏神经内分泌肿瘤报道1例并文献回顾

背景肝脏可疑恶性肿瘤行手术切除后病理诊断肝脏神经内分泌肿瘤(hepatic neuroendocrin neoplasmas,HNENs)病例,此病例易与肝癌混淆,了解转移性和原发性HNENs的特性,对临床治疗手段有很好的指导意义.病例简介一名56岁男性患者体检发现肝脏占位性病变复查腹部增强CT提示肝脏转移性恶性肿瘤可能;胃部包块考虑良性.手术切除胃部包块并行术中冰冻病理检查提示梭形细胞肿瘤;完整切除肝脏包块术后石蜡病理检查结果提示NENs(G2),患者术后恢复佳.结论本文报道了一例HNENs,考虑转移性.术前诊断不能排除肝脏转移性恶性肿瘤可能.通过对患者病情的详细分析,最终行手术切除治疗并行病理检查确诊.同时我们对HNENs相关文献进行了分析,进一步了解原发和转移性HNENs的临床特征、治疗方案以及预后,以期对此类疾病有更深入的了解,对临床治疗方案决策做证据支撑作用.     

Duodenal neuroendocrine tumor: A rare case report

Rationale:Duodenal neuroendocrine tumor (d-NET) is a rare tumor originating in the neuroendocrine system. The clinical manifestations of d-NET are similar to those of other digestive tract tumors, resulting in a lack of specificity and complex clinical symptoms.Patient concerns:A 55-year-old female patient was admitted to our hospital with a chief complaint of an abdominal mass that had been present for more than 4 months.Diagnoses:The upper abdomen enhanced computed tomography scan showed an uneven density mass across the upper abdomen, and the tumor size was approximately 6.2 × 5.8 cm with obvious visible enhancement present in 1 area and a cystic nonenhanced area. The postoperative pathology showed the tumor cells to be positive for chromogranin, synaptophysin, cytokeratin, CD56 (partial weak), negative for vimentin, CD117, DOG-1, CD34, S-100, SMA, desmin, and Ki-67 approximately 2%, which confirmed the diagnosis of d-NETs.Interventions:We preferred laparoscopic surgical exploration, but the tumor started at the ascending part of the duodenum and involved the mesenteric artery. As the branches of the superior mesenteric artery were intertwined with the tumor, it was difficult to operate with the endoscope, so we converted to open laparotomy. The postoperative pathology revealed the presence of d-NET.Outcomes:The patient recovered uneventfully and was discharged after the operation. One-month and 3-month follow-up after surgery, showed no evidence of recurrence.Lessons:Radiological imaging studies are insufficient for the differential diagnosis of abdominal mass from other diseases, whereas surgery is the only radical treatment method, and the preferred surgical method is still active radical resection of the tumor.     

An inflammatory myofibroblastic tumor of the ampulla of vater successfully managed with endoscopic papillectomy: report of a case

Inflammatory myofibroblastic tumors (IMTs) are solid neoplastic mesenchymal proliferations composed of myofibroblastic spindle cells admixed with inflammatory infiltrates. The documented sites in the gastrointestinal tract include the esophagus, small intestine, colon, appendix, rectum, pancreas, spleen, liver, and Meckel's diverticulum. Biliary IMTs are rare, and IMTs arising from the ampulla of Vater have not been reported previously. Herein we report the case of a 65-year-old woman with an extrahepatic biliary obstruction due to IMT of the ampulla of Vater, and a successful therapeutic approach using endoscopic ultrasonography and endoscopic papillectomy.     

Inflammatory myofibroblastic tumor successfully treated with chemotherapy and nonsteroidals: A case report

Inflammatory myofibroblastic tumor (IMT) occurring at retroperitoneal sites has rarely been reported. We report the case of a previously well 14-year-old girl with no history of abdominal disease whose past medical history and family tumor history were unremarkable. She complained of intermittent abdominal pain for one month. An abdominal mass was found on physical examination and abdominal contrast-enhanced computed tomography (CT) showed a hypodense soft mass, the size and location of which suggested a well delineated retroperitoneal tumor surrounding the superior mesenteric vessels measuring 3.3 cm × 4.5 cm × 4.5 cm with enlarged lymph nodes. The patient underwent an exploratory laparotomy followed by biopsy and was subsequently diagnosed with retroperitoneal IMT. She was successfully treated with postoperative chemotherapy and oral diclofenac sodium. Following completion of therapy the mass was no longer palpable and no longer visible on CT scanning. The use of methotrexate and cisplatin for aggressive myofibroblastic tumors is also reviewed.     

Hepatic inflammatory pseudotumor presenting in an 8-year-old boy: A case report and review of literature

Hepatic inflammatory pseudotumors are uncommon benign lesions. Accurately diagnosing hepatic inflammatory pseudotumor can be very challenging because the clinical presentation and radiological appearances are nonspecific and cannot be certainly distinguished from malignant neoplastic processes. Herein, we present a case of hepatic IPT in an 8-year-old boy who presented to clinic with a 3-mo history of a tender hepatic mass, fever of unknown origin, and 9-kg weight loss. The physical examination was notable for tender hepatomegaly. Laboratory investigations were notable for a normal hepatic profile and elevated erythrocyte sedimentation rate and C-reactive protein. A T2-attenuated magnetic resonance imaging scan of the abdomen showed a 4.7 cm × 4.7 cm × 6.6 cm, contrast-enhancing, hyper-intense, well-defined lesion involving the right hepatic lobe. In view of the unremitting symptoms, tender hepatomegaly, thrombosed right hepatic vein, nonspecific radiological findings, and high suspicion of a deep-seated underlying infection or malignancy, a right hepatic lobectomy was recommended. Microscopically, the hepatic lesion exhibited a mixture of inflammatory cells (histiocytes, plasma cells, mature lymphocytes, and occasional multinucleated giant cells) in a background of dense fibrous tissue. Immunohistochemically, the cells stained negative for SMA, ALK-1, CD-21 and CD-23, diffusely positive for CD-68, and focally positive for IgG4. The final histopathological diagnosis was consistent with hepatic IPT. At the postoperative 4-mo follow-up, the patient was asymptomatic without radiological evidence of recurrence.     

右半结肠炎性肌纤维母细胞瘤一例

目的通过本病例报道,提高对该病的认识、诊断和治疗。 方法就我院经病理证实右半结肠肌纤维母细胞瘤的病例1例,结合患者临床病例资料与相关文献报道进行回顾性诊断分析。 结果该患者为老年女性,临床表现为腹部肿块,无其他特异性表现,诊断难度大。IMT多发生在儿童及青少年,常见累及器官是肺,右半结肠炎性肌纤维母细胞瘤在临床上罕见。 结论炎性肌纤维母细胞瘤发病率低,呈低度恶性,临床表现不典型,影像学检查无特异性,易误诊,确诊仍依赖于病理及免疫组化检查。手术为其有效的治疗手段。有复发倾向,术后随访有重要意义。     

Primary intravascular large B-cell lymphoma of the lung: a review and case report

Objective

To investigate the clinicopathological features of primary intravascular large B-cell lymphoma (IVLBCL) of the lung.

Methods

Histopathological and clinical data based on lung biopsy were analyzed and used to diagnose a patient with IVLBCL of the lung.

Results

Fever and respiratory symptoms were the main presentations, lung biopsy revealed lymphoma cells in the lumen of small blood vessels. Tumor cells expressed Bcl-2, the Bcl-6, CD20, Ki67, MUM-1, Pax5, CD, CD30, and vascular endothelial CD34.

Conclusions

Primary pulmonary IVLBCL of the lung is extremely rare, on chest CT it manifests as diffuse ground glass shadow, or nodular consolidations in the lung, lactate dehydrogenase and C-reactive protein was found to increase, fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) is an important and significant diagnostic modality in its early diagnosis. Also, bronchial lung biopsy has the advantage of less trauma and high sensitive rate. R-CHOP is the main treatment for lung primary pulmonary IVLBCL of the lung; however, its prognosis is relatively poor.