Primary Castleman’s Disease of the Liver

Journal of Gastrointestinal Surgery - Castleman's disease is a rare lymphoproliferative disorder that may affect the liver as part of mutlicentric disease or, rarely, as unicentric hepatic...     

Retroperitoneal Unicentric Castleman’s Disease—A Hyaline Vascular Challenge

Castleman’s disease is a rare lymphoproliferative disorder. Multiple variants of the disease exist, with differentiation based on tissue pathology and number of masses within the body. We present the medical diagnostic and surgical treatment pathway of a retroperitoneal unicentric hyaline vascular variant complicated by close proximity to major vascular structures.     

Mesenteric Castleman’s Disease in a 12-Year-Old Girl

Introduction  

Castleman’s disease is a rare disorder characterized by proliferation of the lymphoid tissue usually presenting as an asymptomatic mediastinal mass in children. The location of the disease in the mesentery is rare.     

Pancreatic Castleman’s Disease: Studies of Three Cases And A Cumulative Review of the Literature

Castleman’s disease (CD) is a relatively rare and benign disorder. Pancreatic localization of CD is even more rare and is usually indistinguishable from pancreatic neoplasms. We report three cases of CD in which pancreas was all involved. One located in the tail of the pancreas, who accepted distal pancreatectomy, and the others in the head accepted enucleation. In addition, we review current data on its pathogenesis, imaging findings, diagnosis, differential diagnosis, and treatment.     

Castleman’s disease of the spleen

Introduction: Castleman’s disease (CD), also known as giant or angiofolicular lymphoid hyperplasia or lymphoid hamartoma, is a group of atypical lymphoproliferative disorders that share common lymph node histological features and may be localized either to a single lymph node (unicentric) or occur systemically (multicentric).

Patient and Method: Herein, we present a rare case of a of 75-year-old female patient who was referred to our department and after a thorough work-up, underwent splenectomy with synchronous resection of an accessory spleen, splenic artery lymph nodes, and splenic hilar lymph nodes due to splenic involvement in a multicentric CD.

Results: The pathology of the specimens led to the conclusion that it was a case of polycentric HHV-8-positive CD, affecting the spleen, the accessory spleen, and the lymph nodes.

Conclusions: Incidence of this rare condition is believed to be approximately 0.001–0.05%. CD has been linked to the human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and is associated with malignancies. The pathogenesis mechanism is considered to be a dysregulation and hypersecretion of cytokines, either idiopathic or secondary to a viral infection, with the latter considered the most frequent. Solid organ involvement is very rare as is splenic involvement.     

Castleman’s disease of the pleura

Castleman’s disease is a rare benign disease of the lymph nodes. Its origin from the pleura is rare. Surgical excision, when feasible, appears to provide good results. We encountered a patient who had the disease arising from the parietal pleura. We present the clinical scenario, investigations, and our management of the patient.     

Pretibial Panniculitis Ossificans—A Rare but Significant Diagnosis

Pretibial panniculitis ossificans is a rare condition. In this report, we describe a 67-year-old male localized to his right pretibial tissue, approximately 20 years after contusion to the same area.     

Castleman’s Disease: A Unicentric Case of Abdominal Origin and Literature Review

Hellenic Journal of Surgery - Castleman’s disease (CD) is a rare lymphoproliferative disorder, which may be unicentric or multicentric. We present the case of a young man with the hyaline...     

Synchronous Squamous Cell Carcinoma of Tongue and Unicentric Cervical Castleman’s Disease Clinically Mimicking a Stage IV Disease: A Rare Association or Coincidence?

Malignancy in a setting of hyaline vascular type of Castleman’s disease (HVCD) is an exceptional occurrence. Herein, we report an extremely rare case of synchronous unicentric cervical HVCD and squamous cell carcinoma (SCC) of tongue mimicking stage IV disease. A 32-year-old gentleman presented with an ulcerated mass on the right tongue border and ipsilateral cervical nodal mass. As the clinical stage was IVB (T₁N₃M₀), an anterior two-third glossectomy with bilateral modified neck dissection was performed. On gross examination, an ulcerated mass on the right lateral border was identified. In addition, an 8 cm large nodal mass at right level III–V was seen. Microscopy from the ulcerated growth in the tongue revealed an invasive well differentiated squamous cell carcinoma. However, the right cervical nodal mass yielded surprise histology of Castleman’s disease, hyaline-vascular type. Final tumor pathological staging was revised to pT1N0M0_. This case reveals that HVCD can rarely be associated with an epithelial malignancy wherein it can clinically mimic nodal metastasis. Whether such a phenomenon occurs due to underlying immune aberrations or is a rare co-incidence remains unclear.     

A Rare Internal Herniation in Adult: Meckel’s Diverticulum

Intestinal obstruction due to Meckel’s diverticulum is the most common presentation in adults. There are various mechanisms by which it can cause intestinal obstruction such as volvulus of small intestine around a fibrous band extending from Meckel’s diverticulum to umbilicus, intussusception, and Littre’s hernia. We report the case of a young adult operated on emergency for acute intestinal obstruction. The CT scan suggested a nonspecific internal herniation. Surgical exploration confirmed a rare type of obstruction due to Meckel’s diverticulum.     

Therapeutics in Huntington’s Disease

There is no specific treatment for Huntington’s disease (HD). Its many symptoms of motor, psychiatric, and cognitive deterioration are managed with symptomatic relief, rehabilitation, and support. The only drug approved by the US Food and Drug Administration (FDA) for the treatment of HD is an antichoreic agent, tetrabenazine, but this drug is used sparingly because of uneasiness regarding its propensity to cause depression and suicidality in this population, which is already at risk for these complications. Neuroleptics are still first-line treatments for chorea accompanied by comorbid depression and/or behavioral or psychotic symptoms, as is often the case. Psychiatric features, which have a significant impact on a patient’s professional and personal life, often become the major focus of management. In addition to neuroleptics, commonly used medications include antidepressants, mood stabilizers, anxiolytics, and psychostimulants. In contrast, few treatment options are available for cognitive impairment in HD; this remains an important and largely unmet therapeutic need. HD patients typically lack insight into their disease manifestations, failing to recognize their need for treatment, and possibly even arguing against it. Multipurpose medications are employed advantageously to simplify the medication regimen, so as to facilitate compliance and not overwhelm the patient. For example, haloperidol can be prescribed for a patient with chorea, agitation, and anorexia, rather than targeting each symptom with a different drug. This approach also limits the potential for adverse effects, which can be difficult to distinguish from the features of the disease itself. With HD’s complexity, it is best managed with a multidisciplinary approach that includes a movement disorders specialist, a genetic counselor, a mental health professional, a physical therapist, and a social worker for support and coordination of services. As the disease progresses, there may be need for other specialists, such as a speech and occupational therapist, a nutritionist for weight loss, and ultimately, a palliative care specialist.     

Dementia in Parkinson’s Disease

Dementia in Parkinson’s disease encompasses a spectrum relating to motor, psychiatric, and cognitive symptoms that are classified as either Dementia with Lewy Bodies (DLB) (initial cognitive symptoms) or Parkinson’s Disease Dementia (PDD) (initial motor signs preceding cognitive symptoms by at least a year). Anticholinergic and antipsychotic drugs have a high risk of adverse cognitive and/or motor effects, so their use should be minimized or avoided. Neuroleptic sensitivity is a severe psychomotor adverse reaction that is particularly associated with potent dopamine-blocking agents such as haloperidol. It occurs in up to 50% of individuals with PDD or DLB. Mild psychotic symptoms should first be addressed by reducing anticholinergic and/or dopaminergic agents, if possible. Patients with psychotic symptoms that threaten the safety of the patient or caregiver may benefit from treatment with quetiapine or, in refractory cases, clozapine. Cholinesterase inhibitors as a drug class have been shown to have beneficial effects on cognition in DLB and PDD, and may help to alleviate some psychiatric symptoms, such as apathy, anxiety, hallucinations, and delusions. Memantine may help to moderate cognitive symptoms in DLB and PDD, although current data suggest a more variable response, particularly in PDD. Parkinsonian motor signs that are accompanied by clinically significant cognitive impairment should be treated with carbidopa/levodopa only, as dopamine agonists and other antiparkinsonian medications generally carry a higher risk of provoking or exacerbating psychotic symptoms. Excessive daytime sleepiness and REM sleep behavior disorder are common associated features of PDD and DLB. Minimizing sedating medications during the day and promoting nocturnal sleep may help the daytime sleepiness; melatonin, clonazepam, gabapentin, and possibly memantine may be useful in treating REM sleep behavior disorder. Orthostatic hypotension can be managed with various nonpharmacologic interventions, and if needed, fludrocortisone and pyridostigmine. Midodrine should be used cautiously, if at all.     

Intestinal Cancer Risk in Crohn’s Disease: A Meta-Analysis

Aim of the study  

To clarify the intestinal cancer risk in Crohn’s disease (CD).     

Diagnosis in Childhood Abdominal Burkitt’s Lymphoma

Background  

The role of surgery has changed substantially over the years in abdominal Burkitt’s lymphoma. Laparotomy without total excision of the tumor does not have a positive effect on survival, might cause complications, and delays initiation of chemotherapy. Here we present our diagnostic management of patients with abdominal Burkitt’s lymphoma.     

Sleep Abnormalities in Wilson’s Disease

Purpose of review

The aim of this article was to review the different sleep disorders associated with Wilson’s disease (WD), their mechanisms and their treatments.

Recent findings

Some of these disorders such as REM sleep behavior disorder or sleepiness can appear as a prodromal phase phenomenon in WD allowing an early treatment of the disease and sometimes a resolution of the sleep disorder.

Summary

Sleep disorders in WD are frequent combining insomnia, daytime sleepiness, restless legs syndrome (RLS), cataplexy-like episodes, and REM sleep behavior disorder (RBD). Sleep recordings confirm these disorders. Mechanisms involved in these disorders are complex associating (a) lesions of the pathways regulating sleep and wake or mood but also controlling movement, (b) iatrogenic effects of the treatments, and (3) consequences of the motor or dysautonomic or metabolic disorders.

     

Gastrointestinal Dysfunction in Parkinson’s Disease

Purpose of review

During the past 25 years, there has been an explosion of information regarding the occurrence of gastrointestinal dysfunction in Parkinson’s disease. In this review, the clinical features of gastrointestinal dysfunction in Parkinson’s disease will be described and information regarding the potential role of the enteric nervous system and the gut microbiome in the genesis of Parkinson’s disease will be addressed.

Recent findings

Recognition is growing regarding the role that gastroparesis and small intestinal dysfunction may play in Parkinson’s disease, especially with regard to erratic responses to anti-Parkinson medication. The presence of enteric nervous system involvement in Parkinson’s disease is now well established, but whether the enteric nervous system is the starting point for Parkinson’s disease pathology remains a source of debate. The potential role of the gut microbiome also is beginning to emerge.

Summary

Gastrointestinal dysfunction is a prominent nonmotor feature of Parkinson’s disease and dysfunction can be found along the entire length of the gastrointestinal tract. The enteric nervous system is clearly involved in Parkinson’s disease. Whether it is the initial source of pathology is still a source of controversy. There also is growing recognition of the role that the gut microbiome may play in Parkinson’s disease, but much more research is needed to fully assess this aspect of the disorder.

     

Castleman’s disease in the posterior mediastinum: Report of a case

(Received for publication on June 4, 1999; accepted on Nov. 11, 1999)     

Revised Tubiana’s Staging System for Assessment of Disease Severity in Dupuytren’s Disease—Preliminary Clinical Findings

There are few objective staging systems to assess severity of Dupuytren's disease (DD). Previous methods to assess severity of DD were based primarily on the degree of contracture of an affected digit measured using a goniometer. Nonetheless, this method of assessment alone may be incomplete, and other factors should be considered. White (n = 92) patients diagnosed with DD from northwest of England were assessed for DD. Objective criteria for evaluating severity incorporated quantified variables. The revised severity stage was correlated to a known staging system of DD (Tubiana's staging system) which measures total flexion deformity for a single affected digit. Total revised severity staging scores ranged between 4 and 53 (mean = 18.7) and revealed significant positive correlation to Tubiana's original staging system (r (2) = 0.8, p < 0.001). There was significant difference between severity staging scores in those with a positive family history compared to those without (p < 0.01). In current practice, often, the degree of contracture in an affected digit is used solely as a measure of disease severity. Additional objective clinical information may provide useful prognostic indices for disease progression as well as postoperative outcome.     

Role of the HLA System in the Pathogenesis of Dupuytren’s Disease

Dupuytren's disease (DD) is a familial, fibroproliferative, irreversible, and progressive disease of the palmar fascia, yet with unknown etiology. However, there is compelling evidence which has consistently suggested a genetic ethiopathogenesis given the high occurrence among the Northern European extraction, familial nature, and demonstration of concordance in twins. DD is an incurable, recurrent, and potentially debilitating disease with limited and ineffective treatments. Although a number of possible candidate genes have been investigated including matrix metalloproteinases (MMPs) and transforming growth factor-beta (TGF-β) genes, as yet, no consistent genetic biomarker has been identified for DD. The highly polymorphic human leukocyte antigen (HLA) region is an ideal biomarker target. There have been some coherent data within the literature to suggest a genotype to phenotype association between certain HLA loci and a number of fibrotic disorders such as keloid and scleroderma, markedly with class II molecules and disease pervasiveness and clinical progression. The aim of this review, therefore, was to investigate the evidence indicative of both positive and negative associations between particular HLA alleles and DD. There is a clear association with specific HLA alleles and predilection or protection to DD, though there is a pressing need for further supportive data. The most promising of links to the HLA region in terms of a definitive genetic biomarker is with the class II HLA-DR loci. This paper presents a detailed account of the immunogenetic component of DD and explores the possible mechanisms of association between specific HLA molecules and susceptibility to DD.