Yancu Hertzanu

Congenital insensitivity to pain with anhidrosis (CIPA): the spectrum of radiological findings

Abstract.Background: Congenital insensitivity to pain with anhidrosis (CIPA) is an exceedingly rare, hereditary, sensory autonomic neuropathy (HSAN). Aim: To evaluate the various skeletal manifestations and cranial CT features in children affected by CIPA. Materials and methods: In the semidesert area of the Negev, the Bedouin tribes constitute a closed society where consanguineous marriages are the custom. This has resulted in a group of 20 children being affected by this rare autosomal recessive HSAN. The skeletal surveys and CT scans of these 20 Bedouin patients, 12 girls and 8 boys, ages ranging between 1 month and 8 years, were retrospectively analysed. Cranial CT scans were performed in ten children because of neonatal hypotonia and psychomotor retardation. The skeletal findings were classified as follows: fractures, joint deformities, joint dislocations, osteomyelitis, avascular necrosis and acro-osteolysis. Results: All 20 patients had fractures of the extremities and acro-osteolysis of the fingers. Six had joint deformities. Three children had recurrent hip joint dislocations and another three had avascular necrosis. Ten patients presented with osteomyelitis of the limbs, acetabulum and scapula. The cranial CT scans disclosed mild brain volume loss with some ventriculomegaly. Conclusions: CIPA is a severe autosomal recessive condition that leads to self-mutilation early in life and to fractures, osteomyelitis and limb amputation in older children. Mental retardation is common. Death from hyperpyrexia occurs in almost 20 % of patients in the first 3 years of life.

Sorafenib Combined with Transarterial Chemoembolization versus Transarterial Chemoembolization Alone for Advanced-Stage Hepatocellular Carcinoma: A Propensity Score Matching Study

Aims The purpose of the present study was to compare the efficacies of transarterial chemoembolization (TACE) combined with sorafenib versus TACE monotherapy for treating patients with advanced hepatocellular carcinoma (HCC). Methods We enrolled 321 patients and selected 280 with advanced HCC (Barcelona Clinic Liver Cancer stage C) who underwent TACE therapy between February 2009 and February 2013. TACE alone (monotherapy group) was administered to 198 patients (70.7%), and the remaining 82 (29.3%) underwent repeat combined TACE and sorafenib therapy (combined group). To minimize selection bias, these latter 82 patients were matched using propensity-score matching at a 1∶2 ratio with 164 patients who received TACE monotherapy. The primary endpoints were overall survival (OS) and related subgroup analysis. The secondary endpoints were time to progression (TTP) and treatment-related adverse events. Results Of the respective patients in the combined and monotherapy groups, 64.6% and 49.2% had vascular invasion, 87.8% and 91.1% had extrahepatic metastasis, and 54.3% and 47.1% had both. In the propensity-score–matched cohort, the OS survival of the combined group was significantly higher compared with the monotherapy group (7.0 months vs. 4.9 months, respectively, P = 0.003). The TTP was significantly longer in the combined group (2.6 months vs. 1.9 months, respectively, P = 0.001). Subgroup analysis showed that the outcomes of patients with advanced HCC without main portal vein invasion who were treated with combined therapy were significantly better compared with those who received monotherapy (P<0.05). Univariate and subsequent multivariate analyses revealed that the addition of sorafenib was an independent predictor of favorable OS and TTP (adjusted hazard ratios, 0.63 and 0.62, respectively; P<0.05 for both). Conclusion Sorafenib plus TACE was more effective than TACE monotherapy for treating patients with advanced HCC without main portal vein invasion. Future trials with larger samples are required to validate these preliminary findings.

Computed tomography of pulmonary endometriosis

A case of pulmonary endometriosis in a woman with catamenial hemoptysis is reported. This diagnosis was established by cessation of the hemoptysis following hormonal treatment. Computed tomography appears to be the method of choice for a correct intrapulmonary localization of this disease.

Case report 606

This 66-year -o ld o u t p a t i e n t presented with a b d o m i n a l pa in for which an a b d o m i n a l CT e x a m i n a t i o n was pe r fo rmed . The th ickness slice was 10 mm. A round , wel l -de l inea ted r ad io lucen t lesion, 18 m m in d iameter in the r ight i l ium and ad jacen t to the sacroi l iac jo in t , was d e m o n s t ra tcd (Fig. 1). The pelvic f i lm d e m o n s t r a t e d aga in the lucent skeletal lesion, t ha t w i thou t CT w o u l d p r o b a b l y be confused wi th air in the bowel (Fig. 2).

Intrathoracic rib demonstrated by helical CT with three-dimensional reconstruction

Abstract. Intrathoracic rib is a rare congenital anomaly. An unusual location with atypical pleural tenting is reported. Helical CT with three-dimensional reconstruction seems the best modality for demonstrating the origin and location of these abnormal ribs. The importance of the diagnosis of intrathoracic rib is to rule out pulmonary lesions and prevent unnecessary investigations.

Case report: Mesenteric lipoblastoma

A child with mesenteric lipoblastoma is presented. The computed tomographic (CT) features of this unusual tumour are described and correlated with the sonographic findings. The pre-operative diagnosis may be suggested by these features.

Congenital unilateral hydrocephalus — CT findings

Congenital unilateral hydrocephalus is extremely uncommon with 18 cases previously reported in the English literature. Two additional newborns with unilateral hydrocephalus are presented. The second baby also presented a mega cisterna magna. This unusual association between Dandy-Walker variant and unilateral hydrocephalus has not been previously reported. Following ventriculo-peritoneal shunt, the babies had a normal cognitive neurodevelopment. The role of cranial computed tomography (CT) in diagnosis and follow-up is emphasized.

Testicular carcinoma with inferior vena cava thrombosis extending into the right atrium treated with chemotherapy and anticoagulation

A 34-year-old male with pulmonary emboli and thrombosis of the inferior vena cava extending into the right atrium was found at presentation to have a mixed seminoma and embryonal cell testicular carcinoma with high-volume retroperitoneal disease and visceral metastases. The patient was free of disease 19 months after treatment with combination chemotherapy and anticoagulation followed by resection of the residual mass. We could not find any previous report of a patient with bulky retroperitoneal disease and vena cava thrombosis successfully treated with chemotherapy without vena cava resection.

Primary lymphoma of brain in childhood

Primary brain lymphoma is exceedingly rare during the first decade of life. We report an unusual case, not only being one of the youngest presented, but also because of the unusual CT features exhibited.

Comparison of Treatment Safety and Patient Survival in Elderly versus Nonelderly Patients with Advanced Hepatocellular Carcinoma Receiving Sorafenib Combined with Transarterial Chemoembolization: A Propensity Score Matching Study

Aims This retrospective study was carried out to compare the outcomes between elderly (≥70 years of age) and nonelderly patients (<70 years of age) with advanced hepatocellular carcinoma (HCC) who received sorafenib combined with transarterial chemoembolization (TACE). Methods 88 patients with a confirmed diagnosis of advanced HCC were enrolled in this study. Of these, 24 elderly patients were matched with 48 nonelderly patients at a 1:2 ratio using propensity score matching to minimize selection bias. The related adverse events and survival benefits were compared between the two groups. Results Sorafenib combined with TACE was equally well tolerated in both age groups, and grade 3 or 4 adverse events were similarly observed in 54.2% of elderly and 50.0% of nonelderly patients (P = 0.739). There were no significant differences in survival time between the elderly and nonelderly patients (P = 0.876). Significant prognostic factors for overall survival as identified by multivariate analysis were the Child–Pugh score and portal vein invasion. Conclusions Sorafenib combined with TACE may be well tolerated and effective in elderly patients with advanced HCC. Age alone is not a parameter for the treatment of advanced HCC patients.