Giant Cell Lesions Associated with Primary Hyperparathyroidism

Aims and Objective

To evaluate the prevalence, clinical features, diagnostic laboratory values and treatment outcome of giant cell lesions (brown tumors) associated with primary hyperparathyroidism (PHPT) in oral and maxillofacial region.

Study Design

A 5 year retrospective data was analyzed wherein all histopathologically proven cases of giant cell lesions involving oral and maxillofacial region were evaluated. Out of these cases, those associated with PHPT were tabulated. Correlation was established with other concomitant clinical features and also with the laboratory values of altered serum calcium, phosphate, alkaline phosphate and parathormone. Follow up of these cases after the correction of PHPT was also noted.

Result

Out of 85 cases of histopathologically proven giant cell lesions, five cases were associated with PHPT. There was involvement of maxilla and mandible in one case each. Only frontal bone was involved in two cases. Fifth case had multiple lytic lesions in maxilla and frontal bone. All patients consistently showed very high values of alkaline phosphate and parathormone. Hypercalcemia and hypophosphatemia was noted in four cases. All cases showed regression of the lytic lesion after parathyroidectomy obviating the need for surgical excision of the jaw lesions.

Conclusion

Giant cell lesions (brown tumors) associated with PHPT in oral and maxillofacial region are rare clinical entities. The prevalence of PHPT associated giant cell lesions is 5.9 %. They are clinically, radiologically and histopathologically similar to any other peripheral or central giant cell tumor. Relevant history may alert the clinician and altered biochemical values may help in correlating the oral and maxillofacial findings with the underlying systemic disease. At times, the brown tumor maybe the only presenting sign leading to the diagnosis of PHPT.     

Induction of phosphorylated c‐Jun in neonatal spinal motoneurons after axonal injury is coincident with both motoneuron death and regeneration

c‐Jun activation has been implicated not only in neuronal degeneration, but also in survival and regeneration. Here, we investigated c‐Jun activation in injured motoneurons by using a nerve crush model in neonatal rats. We identified two distinct subpopulations of motoneurons: about 60% underwent degeneration following injury whereas the remaining 40% survived and induced a regeneration response at 3 weeks post injury. However, all motoneurons examined expressed phosphorylated‐c‐Jun‐immunoreactivity (p‐c‐Jun‐IR) at the early stage of 3 days following injury. These results suggest that active c‐Jun was induced in all neonatal motoneurons following nerve crush injury, regardless of whether they were destined to degenerate or undergo successful regeneration at a later stage. Our findings therefore support the hypothesis that active c‐Jun is involved in both neuronal degeneration and regeneration.     

Castleman病相关性嗜血细胞综合征一例报道并文献复习

背景　Castleman病（CD）及嗜血细胞综合征（HPS）均是罕见淋巴增生性疾病,病死率高,迄今尚缺乏标准的治疗方案。目的　加强对CD相关性HPS的认识,提高临床医师对该病的诊疗水平。方法　报道2017-08-22贵州省黔西南州人民医院收治的1例CD相关性HPS的患儿,结合相关文献,总结其诊断及治疗特点。结果　本例患儿表现为反复高热、肝脾大、淋巴结肿大伴全身水肿,曾误诊为败血症、传染性单核细胞增多症、淋巴瘤,并予反复抗感染、大剂量激素、丙种球蛋白等冲击治疗,病情无明显好转;通过正电子发射断层显像/计算机断层成像（PET-CT）检查及颈部淋巴结活检符合淋巴结多中心CD,且骨髓细胞学检查提示,嗜血细胞增多,最终考虑CD相关性HPS。针对白介素6（IL-6）通路靶向治疗后临床症状得到持续缓解。结论　CD是一种较为罕见的疾病,进展迅速,预后较差,且明确诊断需行组织病理学检查,而针对IL-6通路靶向治疗可能成为CD患者有效治疗方案。     

A young-onset frontal dementia with dramatic calcifications due to a novel CSF1R mutation

ABSTRACT

Neuroimaging and genomic analysis greatly aid in the identification of young-onset dementia antemortem. We present the case of a 33-year-old female with a 2-year rapid decline to dementia and immobility marked by personality change, executive deficits including compulsions, attention deficit, apraxia, Parkinsonism, and pyramidal signs. She had unique and dramatic calcifications and confluent white matter changes on imaging and was found to have a novel mutation in the colony stimulating factor 1 receptor gene causing adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP). Here, we review ALSP and briefly discuss differential diagnoses.     

Single-Axonal Organelle Analysis Method Reveals New Protein–Motor Associations

相似文献   

Bilateral Lower Limb Edema Caused by Compression of the Retrohepatic Inferior Vena Cava by a Giant Hepatic Hemangioma

Hemangiomas are the most common benign primary tumors of the liver and their prevalence ranges from 0.4% to 20%. Approximately 85% of hemangiomas are clinically asymptomatic and are incidentally detected in imaging studies performed for other causes. In a very small minority of patients, nausea, vomiting, abdominal pain, distension, palpable mass, obstructive jaundice, bleeding, and signs and symptoms of Budd-Chiari syndrome may develop due to compression of bile duct, hepatic vein, portal vein, and adjacent organs. Occasionally, external compression of inferior vena cava may lead to edema and/or indirect symptoms such as deep vein thrombosis of the lower limbs. In this report, we present a case of giant hepatic hemangioma that completely filled the right lobe of the liver. The patient presented with bilateral lower limb edema and pain. A computed tomography scan detected a 9 × 11 × 12 cm mass indicative of a hemangioma in the right lobe of the liver that compressed the inferior vena cava. The patient refused treatment initially but returned 6 months later presenting with the same symptoms. At that time, the mass had increased in size and a hepatectomy was performed, preserving the middle hepatic vein. By postoperative month 13, the swelling in the lower extremities had decreased significantly and the inferior vena cava appeared normal.     

Robotic Repair of Giant Paraesophageal Hernias

Background and Objectives:

Giant paraesophageal hernia accounts for 5% of all hiatal hernias, and it is commonly seen in elderly patients with comorbidities. Some series report complication rates up to 28%, recurrence rates between 10% and 25%, and a mortality rate close to 2%. Recently, the da Vinci Surgical System (Intuitive Surgical, Sunnyvale, CA, USA) has shown equivocal benefits when used for elective surgeries, whereas for complex procedures, the benefits appear to be clearer. The purpose of this study is to present our preliminary experience in robotic giant paraesophageal hernia repair.

Methods:

We retrospectively collected data from patients who had a diagnosis of giant paraesophageal hernia and underwent a paraesophageal hernia repair with the da Vinci Surgical System.

Results:

Nineteen patients (12 women [63.1%]) underwent surgery for giant paraesophageal hernia at our center. The mean age was 70.4 ± 13.9 years (range, 40–97 years). The mean American Society of Anesthesiologists score was 2.15. The mean surgical time and hospital length of stay were 184.5 ± 96.2 minutes (range, 96–395 minutes) and 4.3 days (range, 2–22 days), respectively. Nissen fundoplications were performed in 3 cases (15.7%), and 16 patients (84.2%) had mesh placed. Six patients (31.5%) presented with gastric volvulus, and 2 patients had other herniated viscera (colon and duodenum). There were 2 surgery-related complications (10.5%) (1 dysphagia that required dilatation and 1 pleural injury) and 1 conversion to open repair (partial gastric resection). No recurrences or deaths were observed in this series.

Conclusion:

In our experience robotic giant paraesophageal hernia repair is not different from the laparoscopic approach in terms of complications and mortality rate, but it may be associated with lower recurrence rates. However, larger series with longer follow-up are necessary to further substantiate our results.     

NATURAL SKIN REDUCTION AND BREAST RECOVERY USING A TISSUE EXPANDER AFTER ENUCLEATION OF A GIANT BREAST TUMOUR

We report a new use of the tissue expander for reshaping a breast after resection of a giant tumour. After resection of giant fibroadenomas, two patients had expanders inserted into the tissue defect and gradually reduced in size over five months. This facilitated healing and natural skin shrinkage and resulted in a natural shape and size.