Τρίτη 2 Μαρτίου 2021

Intrahepatic cholangiocarcinoma is more complex than we thought: A case report

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World J Clin Cases. 2021 Feb 26;9(6):1469-1474. doi: 10.12998/wjcc.v9.i6.1469.

ABSTRACT

BACKGROUND: Brain metastasis from intrahepatic cholangiocarcinoma is rare. To the best of our knowledge, only a few cases have been reported. The biological behavior was complex, and treatment requires further investigation.

CASE SUMMARY: A 62-year-old woman complained of left limb weakness. Abdominal computed tomography showed a 5.0 cm × 5.6 cm lesion in the left lobe of the liver. Tumor markers were normal. Serological analysis indicated absence of hepatitis virus. Brain magnetic resonance imaging revealed a 1.0 cm × 1.3 cm mass in the right frontal lobe. Intrahepatic cholangiocarcinoma with brain metastasis was diagnosed by our liver cancer multidisciplinary team. After sufficient preparation, the patient underwent partial frontal lobotomy and left hemihepatectomy. Histopathological results confirmed that both the lesions were cholangioca rcinoma. Six cycles of gemcitabine combined with S1 were administered. During a 39 mo postoperative follow-up, no sign of local recurrence or distant metastasis was observed.

CONCLUSION: This case expands our knowledge concerning the complex and heterogeneous nature of tumor metastasis.

PMID:33644217 | PMC:PMC7896692 | DOI:10.12998/wjcc.v9.i6.1469

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Congenital hepatic fibrosis in a young boy with congenital hypothyroidism: A case report

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World J Clin Cases. 2021 Feb 26;9(6):1475-1482. doi: 10.12998/wjcc.v9.i6.1475.

ABSTRACT

BACKGROUND: Congenital hepatic fibrosis (CHF) is a rare autosomal recessive disorder characterized by variable degrees of periportal fibrosis and malformation of bile ducts. CHF is generally accompanied by a variety of conditions or syndromes with other organ involvement.

CASE SUMMARY: We report a 5-year-4-month-old Chinese boy with congenital hypothyroidism (CH) diagnosed with CHF. The patient was diagnosed with CH by a newborn screening test and has since been taking levothyroxine. He has developed normally without neurocognitive deficits. Abnormal liver function was observed in the patient at the age of 4 years and 11 mo, and elevated levels of liver function indices were persistent for 5 mo. Radiological imaging indicated hepatospleno-megaly without narrowing of the portal vein but dilated splenic vein. A liver biopsy confirmed the patholo gical features of CHF. Genetic testing revealed two novel homozygous mutations, namely, c.2141-3T>C variant in PKHD1 related to CHF and c.2921G>A (p.R974H) in DUOX2 related to CH. The patient was treated with compound glycyrrhizin tablet, ursodeoxycholic acid, and levothyroxine after diagnosis. The patient achieved a favorable clinical outcome during a follow-up period of over 2 years.

CONCLUSION: Herein, we report the first case of a Chinese boy with comorbidity of CHF and CH, carrying both PKHD1 gene and DUOX2 gene novel mutations. Liver biopsy and genetic testing should be considered for the diagnosis of coexistent liver disease in CH patients with unexplained abnormal liver function.

PMID:33644218 | PMC:PMC7896672 | DOI:10.12998/wjcc.v9.i6.1475

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Venetoclax in combination with chidamide and dexamethasone in relapsed/refractory primary plasma cell leukemia without t(11;14): A case report

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World J Clin Cases. 2021 Feb 16;9(5):1175-1183. doi: 10.12998/wjcc.v9.i5.1175.

ABSTRACT

BACKGROUND: Conventional therapies for primary plasma cell leukemia (pPCL) are usually ineffective, with a short remission time with the use of multiple myeloma medications, showing aggressiveness of pPCL. B-cell lymphoma-2 inhibitor venetoclax is usually used for relapsed/refractory multiple myeloma (RRMM) with t(11;14). There are very few studies published on the use of venetoclax in pPCL without t(11;14). Similarly, histone deacetylase inhibitors are considered effective for the treatment of RRMM, but there are no reports on their use in pPCL.

CASE SUMMARY: A 57-year-old woman with severe anemia, thrombocytopenia, multiple bone destruction, impaired renal function, and 42.7% of peripheral plasma cells is reported. After multiple chemotherapy regimens and chimeric antigen receptor T-cell treatment, the disease progressed again. The patient h ad very good partial response and was maintained for a long time on venetoclax in combination with chidamide and dexamethasone therapy.

CONCLUSION: The success of venetoclax-chidamide-dexamethasone combination therapy in achieving a very good partial response suggested that it can be used for refractory/relapsed pPCL patients who have been exhausted with the use of various drug combinations and had poor survival outcomes.

PMID:33644182 | PMC: PMC7896656 | DOI:10.12998/wjcc.v9.i5.1175

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Polidocanol sclerotherapy for multiple gastrointestinal hemangiomas: A case report

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World J Clin Cases. 2021 Feb 26;9(6):1483-1489. doi: 10.12998/wjcc.v9.i6.1483.

ABSTRACT

BACKGROUND: Gastrointestinal (GI) hemangioma has a low incidence among systemic hemangiomas, and some GI hemangiomas occur in the intestine, stomach, and esophagus. Polidocanol has been increasingly used in sclerotherapy. However, this paper reports that minimally invasive treatment of multiple hemangiomas with large diameters can achieve satisfactory results by multipoint injection.

CASE SUMMARY: A 46-year-old female patient was hospitalized in another hospital for cough. We accidentally found thickening of the lower esophagus by chest computed tomography. The patient was eventually diagnosed with multiple GI hemangiomas and underwent a series of examinations including esophagogastroduodenoscopy (EGD), endoscopic ultrasound, and magnetic resonance imaging. We calculated the dose of polidocanol according to the volumes of the hemangiomas, fixe d the target vein with the help of a transparent cap, and then administered polidocanol via multipoint injection into the hemangiomas under endoscopic guidance. EGD and endoscopic ultrasound showed that the hemangiomas disappeared. The color of the esophageal mucosa returned to normal 1 mo after sclerotherapy.

CONCLUSION: Sclerotherapy may be a safe and effective method for treating multiple hemangiomas of the alimentary canal.

PMID:33644219 | PMC:PMC7896683 | DOI:10.12998/wjcc.v9.i6.1483

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Imaging characteristics of a rare case of monostotic fibrous dysplasia of the sacrum: A case report

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World J Clin Cases. 2021 Feb 16;9(5):1111-1118. doi: 10.12998/wjcc.v9.i5.1111.

ABSTRACT

BACKGROUND: Fibrous dysplasia (FD) is a common benign intramedullary fibro-osseous lesion. Involvement of the spine is rare, with the literature including only case reports, and cases of monostotic FD (MFD) in the sacrum are extremely rare. A correct preoperative diagnosis of spinal MFD is important for clinicians to select proper treatment.

CASE SUMMARY: We retrospectively assessed a case report of MFD in the sacrum. This patient was examined by computed tomography (CT) and magnetic resonance imaging (MRI), and the diagnosis was confirmed by pathology. A review of the literature was performed to analyze the imaging characteristics and differential diagnoses of spinal MFD. For our patient, the CT scan showed the lesion to be expansile, with ground glass opacity and a sclerotic rim. On MRI, the lesion showed iso-low signal intensity on T1WI and iso-high signal intensity on T2WI. A low signal rim was found on T1WI and T2WI. Our patient was treated by posterior focal excision, decompression, bone grafting, fusion and pedicle screw fixation. A satisfactory result was achieved, with pain disappearance. No complications had occurred at the 1-year follow up.

CONCLUSION: MFD is an expansile osteolytic change. Ground glass opacity and a sclerotic margin are obvious characteristics. The lesion often involves the vertebral body and posterior element. Knowledge of these imaging characteristics of spinal FD could be helpful for diagnosis and prevent unnecessary procedures.

PMID:33644174 | PMC:PMC7896654 | DOI:10.12998/wjcc.v9.i5.1111

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Bilateral musculocutaneous neuropathy: A case report

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World J Clin Cases. 2021 Feb 16;9(5):1237-1246. doi: 10.12998/wjcc.v9.i5.1237.

ABSTRACT

BACKGROUND: Isolated musculocutaneous nerve injury is a rare condition. Herein, we report the first case of bilateral musculocutaneous neuropathy after vigorous stretching of both upper extremities with normal results of sensory nerve action potential. Clinicians should be aware of this rare condition that can appear bilaterally. In addition, the interpretation of the aberrant electrodiagnostic study results of this case was discussed.

CASE SUMMARY: A 29-year-old male complaining of bilateral forearm tingling and upper extremity weakness visited the outpatient clinic. The symptoms began 6 mo prior, and he visited another hospital before visiting our department. The diagnosis was not made even after cervical spine magnetic resonance imaging, electrodiagnostic study, brain magnetic resonance imaging, and arteriography were conducted. The patient performed unique exercises that stretched the pectoralis minor and coracobrachialis muscles. On the follow-up electrodiagnostic study, abnormal spontaneous activities in the bilateral biceps and brachialis muscles were observed. The patient was diagnosed with bilateral musculocutaneous neuropathy. Steroid pulse therapy was administered for approximately 6 wk. After treatment, his muscle strength returned to the predisease condition.

CONCLUSION: Clinicians should be aware of this condition, have adequate understanding of anatomy, and advise to correct inappropriate exercises.

PMID:33644190 | PMC:PMC7896669 | DOI:10.12998/wjcc.v9.i5.1237

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Δευτέρα 1 Μαρτίου 2021

Prognostic value of olfactory evoked potentials in patients with post-infectious olfactory dysfunction

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Eur Arch Otorhinolaryngol. 2021 Mar 1. doi: 10.1007/s00405-021-06683-y. Online ahead of print.

ABSTRACT

PURPOSE: Prognostic assessment of patients with post-infectious olfactory dysfunction (PIOD) poses a challenge for clinicians. While there have been some studies on the prognostic factors of PIOD focusing on demographic factors, the aim of this study was to investigate whether event-related potentials (ERPs) could be used as a new predictor of olfactory recovery in PIOD.

METHODS: This was a retrospective study involving patients who underwent olfactory examinations using Sniffin' Sticks test before treatment and after 1 year of follow-up. The responder group was defined by an increase of threshold-discrimination-identification (TDI) score of ≥ 6 points. All patients underwent ERP examination and the amplitude and latency of each wave of ERPs were recorded before treatment.

RESULTS: A total of 61 patients (age 47.50 ± 1 1.04 years, 27 males) were analyzed. The presence of olfactory ERPs (oERPs) was greater in the responder group than in the non-responder group (P = 0.007), while that of trigeminal ERPs (tERPs) did not differ between the two groups (P = 0.346). Logistic-regression analyses showed that factors associated with improvement of subjective olfactory function were duration (OR, 1.604; 95% CI, 1.062-2.423; P = 0.025), initial threshold (odds ratio [OR], 0.043; 95% confidence interval [CI], 0.004-0.439; P = 0.008), and latency of N1 in oERPs (OR, 1.007; 95% CI, 1.001-1.013; P = 0.021).

CONCLUSION: Our study shows that duration of OD, initial threshold, and latency of N1 in oERPs were associated with olfactory improvement in PIOD patients, which may provide guidance for clinicians.

PMID:33644842 | DOI:10.1007/s00405-021-06683-y

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