Case report: Remarkable response to sintilimab, lenvatinib, and nab-paclitaxel in postoperative metastatic chemotherapy-resistant combined hepatocellular-cholangiocarcinoma.

Background: Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a highly aggressive malignancy with a poor prognosis. However, there are no consensus treatment guidelines, and decisions are usually extrapolated from intrahepatic cholangiocarcinoma (ICC) or hepatocellular carcinoma (HCC). Given that cHCC-CCA owns the unequivocal presence of both hepatocytic and cholangiocytic differentiation, a combination regimen of anti-PD1 antibody, multikinase inhibitor, and chemotherapy targeting against both components might be an optimal choice. Case presentation: We present the case of a patient with postoperative metastatic chemotherapy-resistant cHCC-CCA who exhibited a durable response and reasonable tolerability to a combination therapy consisting of the anti-PD1 antibody sintilimab, multikinase inhibitor lenvatinib, and nab-paclitaxel, despite having a low tumor mutational burden (TMB-L), microsatellite stability (MSS), and negative programmed cell death 1 ligand 1 (PD-L1). Conclusion: The combination regimen of immune checkpoint inhibitor sintilimab, multikinase inhibitor lenvatinib, and chemotherapy with nab-paclitaxel, which targets both the HCC and ICC components, may represent a promising treatment option for patients with cHCC-CCA. Further research is warranted to validate these findings in larger patient cohorts.

Pathological complete response to neoadjuvant therapy with serplulimab and chemotherapy in stage IIIB small cell lung cancer: a case report and literature review.

Chemotherapy combined with immunotherapy has significantly improved survival in patients with extensive-stage small cell lung cancer (ES-SCLC), and neoadjuvant immunotherapy combined with chemotherapy has emerged as the standard treatment for those with resectable non-small cell lung cancer (NSCLC). However, the potential benefits of surgery following neoadjuvant immunotherapy combined with chemotherapy in locally advanced SCLC remain unclear. Herein, we report a patient diagnosed with stage IIIB SCLC, who was administered five cycles of neoadjuvant serplulimab combined with chemotherapy followed by surgery, and subsequently achieved a pathologic complete response (pCR). Within a follow-up duration of six months, the patient displayed neither recurrence nor metastasis and experienced no treatment-related adverse reactions of any grade. Based on this case, for locally advanced SCLC, neoadjuvant serplulimab combined with chemotherapy followed by surgery may present an effective, safe, and potentially curative treatment strategy. Nonetheless, further prospective studies are needed to verify our findings.

Early-onset colorectal cancer: A distinct entity with unique genetic features.

The aim of the present study was to elucidate the genetic features of early-onset colorectal cancer (CRC), particularly the genetic mutations that may be regarded as prognostic and/or predictive markers in CRC and other malignancies. In total, 40 patients with non-polyposis CRC aged 35 or younger were selected. The formalin-fixed, paraffin-embedded tumors acquired were subjected to mismatch repair (MMR) protein immunochemical staining and gene analysis with next-generation sequencing (44 exons, 17 genes; Ion Torrent Sequencing Platform). A total of 11 (27.5%) tumors presented with MMR protein deficiency (dMMR) and 26 (65%) tumors harbored one or more genetic mutations, including K-RAS proto-oncogene (35%), phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA; 20%), B-Raf proto-oncogene (5%), erb-b2 receptor tyrosine kinase 2 (5%), discoidin domain receptor tyrosine kinase 2 (5%), N-RAS proto-oncogene (2.5%), KIT proto-oncogene (2.5%), TSC complex subunit 1 (2.5%), DNA methyltransferase 3 alpha (2.5%) and ABL proto-oncogene 1 (2.5%). Of the dMMR tumors, 81.8% (9/11) of cases presented with mutations in the tested genes, while only 58.6% (17/29) of the MMR-proficient (pMMR) tumors presented with these (P=0.158). PI3KCA was frequently mutated in dMMR tumors compared to pMMR tumors (P=0.025). In a subgroup with a family history of CRC, the dMMR status (P<0.001) and PIK3CA genetic mutation status (P=0.01) were more frequently observed compared to the other two groups (with a family history of other cancer types or no malignancy). Almost all patients who had relatives with CRC presented with both dMMR and other genetic mutations, while this was not observed in the patients who had relatives with other types of carcinoma. Certain genetic mutations that are rarely reported in CRC were only identified in those patients with a family history of carcinoma. In conclusion, non-polyposis CRC in young adults presents as a distinct entity with a unique set of genetic features. However, investigation of more cases in further studies is required to verify the present results.

Spinal extradural angiolipoma: a report of two cases and review of literature.

Spinal extradural angiolipomas (SEALs) are extremely extraordinary benign extradural lesions. They are infrequently encountered in normal clinical practice although several authors have report single cases or case series. We present two cases of SEAL which we successfully surgical resected with no further neurological deficits. Our cases comprise of a male and a female with ages ranging from 30 to 60 years. Their principal presenting complains were numbness and pain at the lower extremity with associated fecal and urinary incontinence. In all our cases, MRI revealed extradural spinal lesions that exerted compressive effect on the spinal cord. The male patient had an infiltrating type while the female had non-infiltrating type. We attained total resection in both cases without any further neurological complication. The diagnosis of SEALs initially can be challenging radiologically since they may mimic other spinal lesions. The gold standard treatment modality should always be surgery although total resection may not be achievable in some cases.

Assessment of programmed cell death ligand-1 expression with multiple immunohistochemistry antibody clones in non-small cell lung cancer.

BACKGROUND: Programmed cell death-1 (PD-1) and programmed cell death ligand-1 (PD-L1) have been identified as novel targets for immunotherapy, with PD-L1 as a potential predictive biomarker. However, a specific antibody for PD-L1 expression is an immediate requirement. Meanwhile, the clinicopathological identification of patients with positive PD-L1 remains unclear. METHODS: The present study adopted three anti-PD-L1 IHC antibodies, SP142, SP263, and UMAB228 to test PD-L1 expression in 84 non-small cell lung cancer (NSCLC) specimens. The concordance among antibodies was examined by analytical comparison, and the association between PD-L1 expression and clinicopathological factors was assessed. RESULTS: The samples from 41 (48.8%), 51 (60.7%), and 50 (59.5%) patients were detected as PD-L1 positive evaluated by antibody SP142, SP263, and UMAB228, respectively. The kappa coefficient was 0.53, 0.58, and 0.46 for SP263 vs. SP142, SP263 vs. UMAB228, and SP142 vs. UMAB228, respectively. On the other hand, the univariate analysis of consensus cases indicated that the PD-L1 expression was significantly correlated with tobacco use (χ2=4.25, P=0.04). CONCLUSIONS: The analytical comparison showed moderate concordance between SP142, SP263 and UMAB228, whereas SP263 exhibited higher overall positive rate. Moreover, PD-L1 positive rate was significantly higher in patients with smoking history, which might help in identifying patients who would benefit from PD-1/PD-L1 checkpoint inhibitors.

Thoracolumbar spinal neurenteric cyst with tethered cord syndrome and extreme cervical lordosis in a child: A case report and literature review.

RATIONALE: Neurenteric cysts, are rare benign tumors of the central nervous system that are mostly located in the spinal cord and predominantly seen in male children although adult form of the disorder also occurs. The etiology and treatment of this disorder is still a matter of debate. Our case further throws more light on the pathogenesis and treatment of this disorder. PATIENT CONCERNS: A 4-year-old boy presented with 5-month history of cervical lordosis and bilateral lower extremity pain that progressed to his abdomen and upper body. The pain was general, recurrent, non-persistent and progressive in nature with no paralysis. The pain was aggravated by trunk stretching and relieved when he assumed opisthotonos position so he preferred sleeping in this position at night. DIAGNOSES: Magnetic resonance imaging (MRI) revealed a cystic lesion at the thoracolumbar spine with tethering of spinal cord and cervical lordosis. INTERVENTIONS: He was operated on successfully and the cervical lordosis and pain resolved. OUTCOMES: The child recovered well with no tumor recurrence and massive improvement of his life. LESSONS: The gold standard treatment for this disorder is surgery although the precise surgical approach is still a matter of debate. We are of the view that surgical approach should be individualized and aim at total excision of the cyst.

Selecting a suitable surgical treatment for hepatic angiomyolipoma: a retrospective analysis of 92 cases.

BACKGROUND: Hepatic angiomyolipoma (HAML) is a rare and difficult-to-diagnose liver tumour. The aim of this study was to summarize experiences in the management of HAML and to recommend a practical treatment strategy. METHODS: We retrospectively studied 92 patients who were diagnosed with HAML and analysed the clinical presentation, histopathological features and treatment of the tumours encountered at our institute from May 2009 to June 2016. RESULTS: The patients included 67 females and 25 males who underwent at least one radiographic examination. Sixty-eight patients underwent radical hepatectomy, two patients underwent liver biopsy, and 22 patients were treated with radiofrequency ablation after liver biopsy. The tumour cells correspondingly expressed both melanoma cell markers (HMB45, MART-1) and smooth muscle cell markers. Two patients were found to have tumour recurrence (2/92, 2.2%) after radical hepatectomy, and none of the patients died. CONCLUSION: Diagnosis of HAML depends on pathological findings. The treatment strategy for HAML should be selected according to the tumour size, liver biopsy, location and clinical symptoms of HAML. Patients should be followed closely after surgery because of the malignant potential of HAML.

Extrarenal teratoma with nephroblastoma in the retroperitoneum: Case report and literature review.

RATIONALE: Teratoma with nephroblastoma is a rare disease. The most common site at which teratoma with nephroblastoma occurs is the kidney. The mechanisms underlying the development of teratoma with nephroblastoma have not been fully elucidated. PATIENT CONCERNS: In the current report, we describe the clinical characteristics of a 3-year-old girl with a complaint of a painless abdominal mass in the upper right side of the body. Ultrasonography and computed tomography revealed a cystic-solid mass with a clear boundary. DIAGNOSIS: Surgical resection and a subsequent pathological examination confirmed that the mass contained teratoma tissues and renal blastemal components, which supports the diagnosis of teratoma with nephroblastoma. INTERVENTIONS: The patient underwent an exploratory laparotomy through a transverse abdominal incision. Complete resection of the mass was performed in this patient. OUTCOMES: The patient's postoperative course was uneventful and she was discharged on the 8th postoperative day. The girl had no complaints during the 2 years follow-up period. LESSONS: Teratoma with nephroblastoma is a rare entity that typically presents in childhood. Due to its rarity, no standardized criteria have been established for the categorization and treatment of these lesions. However, a complete excision of this tumor allows the diagnosis to be confirmed and lowers the risk of recurrence.

Hepatic paragonimiasis in a 15-month-old girl: a case report.

BACKGROUND: Paragonimiasis, particularly hepatic paragonimiasis (HP), is a type of zoonotic parasitic disease rarely encountered in infants. There have been only a few reports of HP, and no case of HP has been reported in an infant. CASE PRESENTATION: A 15-month-old girl presented with persistent mild fever with a duration of 1 month, hepatomegaly, and low-density lesions in the right hepatic lobe on abdominal ultrasound and computer tomography. Pathological examination and serum antibody detection were performed to verify HP. The diagnosis of HP was established based on findings of Charcot-Leyden crystals on liver lesion biopsy and antibodies against paragonimus westermani detected by enzyme-linked immunosorbent assay. After initiation of praziquantel (75 mg/kg/day for 3 days), all clinical findings promptly improved and the patient was discharged. CONCLUSION: It is very important to consider paragonimiasis in the clinical examination of infants from an area with paragonimiasis epidemic presenting with fever, hepatomegaly, low-density lesions in the liver.

Giant solitary primary intracranial lymphoma masquerading as meningioma: a case and review of literature.

Non-Hodgkin's lymphomas (NHL) with intracranial origin are very rare and constitutes about 1-2% of primary central nervous system lymphomas (PCNSL). Diffuse large B cell lymphoma (DLBCL) is the most common subtype of NHL and mostly seen in immunocompromised patients. Therefore, the occurrence of giant solitary DLBCL in an immunocompetent patient is puzzling. We present a case of 68-year-old man who was admitted at our facility with a history of "hypomnesia of two (2) months" duration. Magnetic resonance imaging (MRI) revealed a space occupying lesion in the bilateral frontal lobe and corpus callosum measuring about 5.4cm * 4.6cm * 3.8cm with mixed signal intensities and vasogenic edema around the mass. Radiological, this mass was mistaken for meningioma until histopathological studies revealed DLBCL. Giant solitary primary intracranial lymphomas are very rare and can be mistake for meningioma even with very experience radiologist or neurosurgeon since the radiological features of PCNSL can be very unspecific. We achieved to total resection because of the giant and solitary nature of our case. The prognosis of PCNSL is general very poor when the patient is immunocompromised. In immunocompetent patients, who are well managed with surgery and chemotherapy, the overall survival and quality of life can very encouraging.

Contrast-enhanced ultrasound in diagnosis of primary hepatic angiosarcoma.

Primary hepatic angiosarcoma (PHA) is a rare malignant tumor that occurs mainly in the elderly, with almost no specific symptoms or tumor markers. Information on the imaging characteristics of this tumor is limited due to its rarity. Therefore, it is difficult to diagnose PHA. So far, its definite diagnosis depends on histopathologic examination combined with immunohistochemical results. Patients with PHA have a poor prognosis in spite of surgical resection of this tumor. In this case report, we present a 72-year-old woman with PHA, focusing on the imaging features of this tumor, especially its enhancement pattern on contrast-enhanced ultrasound images. Contrast-enhanced ultrasound provided helpful information for diagnosis.

Feasibility of laparoscopic major hepatectomy for hepatic paragonimiasis: two case reports.

BACKGROUND: Though accumulated evidence proved that laparoscopic major hepatectomy was technically feasible, it remains a challenging procedure and is limited to highly specialized centers. Paragonimiasis is one of the most important food-borne parasitic zoonoses caused by the trematode of the genus Paragonimus. Although hepatic paragonimiasis is rare, the previous studies had investigated hepatic paragonimiasis from different perspectives. However, the safety and feasibility of laparoscopic major hepatectomy for hepatic paragonimiasis have not yet been reported in the literature. METHODS: We here present 2 cases of hepatic paragonimiasis at the deep parts of the liver with treatment by laparoscopic major hepatectomy. One case is a 32-year-old male patient who was admitted to the hospital due to upper abdominal discomfort without fever for 1 month. The clinical imaging revealed that there was a lesion about 5.9 × 3.7 cm in the boundary of right anterior lobe and right posterior lobe of the liver with rim enhancement and tract-like nonenhanced areas. The other one is a 62-year-old female patient who was referred to the hospital for 1 month of right upper abdominal pain and fever. The ultrasonography showed that there was a huge hypoechoic mass (about 10.8 × 6.3 cm) in middle lobe of the liver with tract-like nonenhanced areas. Both patients were from an endemic area of paragonimiasis and the proportion of eosinophil in the second case was increased. RESULTS: The preoperative diagnosis of the first case was ambiguous and the hepatic paragonimiasis was considered for the second case. The first case underwent laparoscopic extended right posterior lobe hepatectomy and the other case underwent laparoscopic extended left hemihepatectomy. Both operations went very well and the operation times for the 2 cases were 275 minutes and 310 minutes, respectively. The 2 patients' postoperative recovery was smooth without major postoperative complications (such as, bleeding, bile leakage, and liver failure). Moreover, the 2 patients were discharged on the 6th day and 7th day after surgery, respectively. The postoperative histopathological examination manifested hepatic paragonimiasis in both patients. CONCLUSION: This study suggests that the laparoscopic approach may be safe and technically feasible for hepatic paragonimiasis.