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 mesorectum: Report of a case

Castleman’s disease is a benign lymphoid proliferative disorder, which most commonly presents as a solitary mass in the mediastinum, although extrathoracic sites have been reported in the neck, axilla, pelvis, mesentery, pancreas, and retroperitoneum. We report a case of asymptomatic, isolated Castleman’s disease in the mesorectum, which is extremely rare. The patient was a 34-year-old woman who underwent investigations for vaginal spotting. A presacral mass was located on the left side of the rectum, 10 cm from the anal verge. Contrast-enhanced computed tomography showed a large, well-demarcated, strongly enhancing mass with internal radiating septa in the mesorectum. The mass was well circumscribed and isointense to muscle on T1-weighted magnetic resonance imaging, appearing as a slightly hyperintense mass on T2-weighted imaging. We performed laparoscopic mesorectal mass excision, and histological examination revealed features typical of the hyaline-vascular type of Castleman’s disease. Thus, when a mesorectal mass is being investigated, Castleman’s disease should be considered in the differential diagnosis.     

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

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

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...     

Dupuytren’s disease of the radial side of the hand

Dupuytren’s disease with involvement of the radial aspect of the hand is a clinical entity, which affects the thumb, the first web space, and the index finger. It can be primary or secondary. The palmar fascia anatomy and the clinical aspects of radial Dupuytren’s disease are described. The authors propose a new classification, which has the advantage in requiring only a clinical evaluation. One hundred and fifteen patients were examined for Dupuytren’s disease with radial involvement, primary or secondary, in the Hand Surgery Unit of the University of Modena between 1985 and 1994. Eighty-two (68.3%) of them were treated surgically as hand function was compromised by first web retraction. The most commonly used incisions of the first commissure are straight-line with Z-plasties or the Hirschowitz incision; either a zigzag incision or a Bruner incision is made over the thumb or index finger; Z-plasties described by Iselin are reserved for patients with severe contraction of the index finger. Results were assessed by improvement coefficient, calculated as the ratio of the difference between preoperative and postoperative scores to postoperative scores. Generally, Dupuytren’s disease with radial prevalence is not aggressive and progresses slowly and may be exclusively radial or associated with ulnar disease. In other patients, as the disease progresses, loss of function becomes apparent owing to retraction in adduction of the first commissure and flexion contracture of the thumb or index finger.     

Laparoscopy-assisted resection for Mesenteric Castleman’s disease: Report of a case

A 65-year-old female patient was diagnosed with breast cancer in 1995. An abdominal mass was detected by computed tomography and the patient was preoperatively diagnosed with a gastrointestinal stromal tumor. Laparoscopy-assisted surgical resection revealed a mesenteric Castleman’s tumor of the hyaline vascular type. Mesenteric Castleman’s disease is rare, and there have been only 42 cases reported in the English literature. We herein discuss the clinical findings of these cases.     

Retroperitoneal unicentric Castleman’s disease: A case report

IntroductionCastleman’s disease (CD) is an angio-follicular lymph node hyperplasia presenting as a localized or a systemic disease masquerading malignancy. The most common sites of CD are mediastinum, neck, axilla and pelvis. Unicentric CD in the peripancreatic region is very rare.Presentation of caseWe report a case of the 34-year-old lady presenting with epigastric pain for 3 months. Abdominal imaging revealed a retroperitoneal mass arising from the pancreas suspected to be neuroendocrine tumor. Tumor markers were not elevated. Complete surgical excision was performed and patient had uneventful recovery. Pathologic findings demonstrated localized hyaline-vascular type of Castleman’s disease.DiscussionCD is a very rare cause for development of retroperitoneal mass. It is more frequent in young adults without predilection of sex. It can occur anywhere along the lymphoid chain. Abdominal and retroperitoneal locations usually present with symptoms due to the mass effect on adjacent organs. CD appears as a homogeneously hypoechoic mass on ultrasound and non-specific enhancing homogeneous mass with micro calcifications on computed tomography. Histologically, the hyaline vascular type demonstrates a follicular and inter-follicular capillary proliferation with peri-vascular hyalinization, with expansion of the mantle zones by a mixed inflammatory infiltrate of numerous small lymphocytes and plasma cells. The standard therapy of localized form is en bloc surgical excision as performed in our case.ConclusionUnicentric CD in the peripancreatic region is difficult to differentiate from pancreatic neoplasm preoperatively. However, preoperative biopsy in cases of high clinical suspicion can help in avoiding extensive surgery for this benign disease.     

Castleman’s disease along the left tracheobronchial tree with a difficult preoperative diagnosis

A 39-year-old man was referred to our hospital because of an asymptomatic middle mediastinal tumor. A preliminary histological diagnosis of the tumor by bronchoscopy was difficult to obtain because the tumor was located along the left tracheobronchial tree, which is difficult to approach. The tumor was resected through a right anteroaxillary thoracotomy without any major complications, and histopathological examination revealed that the lesion was Castleman’s disease, hyaline-vascular type. Radiological findings of the lesion were typical; however, the rarity of the tumor made the imaging diagnosis difficult. If a lesion is located along the tracheobronchial tree, Castleman’s disease should be considered in the differential diagnosis.     

Renal complications of Castleman’s disease: report of two cases and analysis of 75 cases

Biopsy-proven renal complications of Castleman’s disease (CD) are rare and current knowledge is largely based on sporadic case reports. We reported two more cases, both of which were multicentric CD with hyaline-vascular pathological pattern and presented with chronic renal failure. Case 1 was multicentric CD with renal mesangial proliferative glomerulonephritis complications, and case 2 was multicentric CD with membranoproliferative glomerulonephritis-like complications. Although both were eventually administered corticosteroids combined with cytotoxic drugs, both behaved in an aggressive and relapsing manner. We then made an analysis of 75 cases of biopsy-proven renal complications of CD (including our two cases) which were reported in 51 English literatures from January 1954 to March 2011. We found that the clinical and histological findings of renal complications of CD were heterogeneous. Death was observed in 17% patients after a median follow-up time of 22 months (0–204 months) since histological diagnosis of renal complications. The estimated 5-year cumulative survival rate was 75%. Better understanding and therapeutic interventions are required in further investigations.     

Dupuytren’s disease – the story of an eponym

December 5, 1831, baron Guillaume Dupuytren presents at the surgical clinics of the Hotel-Dieu of Paris a report upon "an entirely new subject, with a new theory and a new method of treatment for a disease generally regarded as incurable". The patient presented had a permanent contraction of the ring finger and adjacent fingers of both hands, which appeared spontaneously without any injury or previous illness. Dupuytren explains he had the opportunity to dissect the hand of a man who died after having had a contraction of the fingers for many years. He defines the cause of the contraction to be the palmar fascia. He describes the operative treatment: aponeurotomy by a transverse incision. "Probably many surgeons (Cline 1808, Astley Cooper 1822) were aware of the palmar aponeurosis producing this condition before Dupuytren's lecture". "Thoroughness of his demonstration, the brilliance of the presentation and the supremacy of his surgical authority combined to link permanently the name of Dupuytren with this hand deformity" (J. Hueston).     

Characterisation of the inflammatory response in Dupuytren’s disease

Background: Dupuytren’s disease is characterised by fibrotic nodule and cord formation in the palmar aponeurosis. The pathophysiology of the disease is still unknown, although cell stress and subsequent activation of immune mechanisms seems to be crucial. Materials and methods: Surgically obtained tissue and blood samples of 100 Dupuytren patients were processed by immunohistochemistry, flow cytometry, as well as immunoscope analysis. Macroscopically normal aponeurotic tissue served as control. Results: Locally, microvascular alterations and massive infiltration by mononuclear cells (CD3+, CD4?>?CD8, CD45RO?>?CD45RA, S100 protein, CD56, CD68, scarce CD19 and mast cells) forming perivascular clusters were found in DD tissue. Cytokine profiling of fibromatosis tissue-derived T-cells showed a Th1/TH17-weighted immune response. Immunoscope analysis revealed a restricted T-cell receptor α/β repertoire pointing to an (auto)antigen-driven process. Conclusion: The striking accumulation of immune cells, expression of leukocyte adhesion molecules, as well as pro-inflammatory and pro-fibrotic cytokines near markedly narrowed vessels supports the theory that the abnormal proliferation of fibroblasts and production of extracellular matrix proteins in DD seems to be related to immune-mediated microvascular damage. The restricted T-cell receptor repertoire of intra-lesional T-cells points to an antigen-driven process. T-cells seem to play an important role in the development of Dupuytren’s disease.     

Crohn’s disease of the esophagus: Report of a case

(Received for publication on Jan. 18, 1999; accepted on Sept. 17, 1999)     

Surgical management of Crohn’s disease

Introduction

Crohn’s disease is an inflammatory bowel disease that can affect the entire gastrointestinal tract. It is chronic and incurable, and the mainstay of therapy is medical management with surgical intervention as complications arise. Surgery is required in approximately 70% of patients with Crohn’s disease. Because repeat interventions are often needed, these patients may benefit from bowel-sparing techniques and minimally invasive approaches. Various bowel-sparing techniques, including strictureplasty, can be applied to reduce the risk of short-bowel syndrome.

Methods

A review of the available literature using the PubMed search engine was undertaken to compile data on the surgical treatment of Crohn’s disease.

Results and conclusion

Data support the use of laparoscopy in treating Crohn’s disease, although the potential technical challenges in these settings mandate appropriate prerequisite surgical expertise.     

Initial treatment of parkinson’s disease

Opinion statement Initial treatment of early idiopathic Parkinson’s disease (PD) begins with diagnosis based on clinical evaluation supplemented by laboratory studies and brain imaging to exclude causes of secondary parkinsonism. In most cases, testing is normal and the diagnosis of PD rests on clinical criteria. In patients with mild symptoms and signs, the diagnosis of PD may not initially be apparent, and follow-up evaluation is needed to arrive at a diagnosis. Once the diagnosis is made, pharmacologic treatment may not be the first step. First, patient education is essential, especially because PD is a highprofile disease for which information and misinformation are readily available to patients and families. Counseling concerning prognosis, future symptoms, future disability, and treatment must be provided. Questions from patients concerning diet, lifestyle, and exercise are especially common at this point. The decision of when to initiate treatment is the next major consideration. Much controversy but relatively little light has been brought to bear on this issue. L-dopa was the first major antiparkinson medication to be introduced and remains the “gold standard” of treatment. Next in efficacy are the dopamine agonists (DAs). A debate has raged concerning whether initial dopaminergic treatment should be with L-dopa or DAs. Physicians have been concerned about forestalling the appearance of dyskinesias and motor fluctuations, whereas patients have incorrectly understood that L-dopa and possibly other antiparkinson drugs have a finite duration of usefulness, making it important to defer treatment for as long as possible. This has created “L-dopa phobia,” which may stand in the way of useful treatment. In spite of this controversy, there is uniform agreement that the appropriate time to treat is when the patient is beginning to be disabled. This varies from patient to patient and depends on age, employment status, nature of job, level of physical activity, concern about appearance, and other factors. The choice of a specific drug is sometimes dictated by the patient’s symptoms. For example, L-dopa is preferable for severe akinesia, an anticholinergic may be useful when tremor is the most prominent symptom (especially in those aged younger than 70 years), and DAs may be indicated for younger patients, more prone to dyskinesias and fluctuations, with relatively mild symptoms. It is also important to manage non-motor symptoms in patients with early PD. Anxiety and depression are particularly common at this stage and may be presenting symptoms of PD. Where appropriate, counseling and/or treatment with anxiolytics and antidepressants should be considered.     

New discoveries in the basic science understanding of Peyronie’s disease

Peyronie’s disease is an acquired benign condition representing localized fibrosis of the penis. The disease is caused by microvascular trauma to the penis, with subsequent fibrin deposition resulting in aberrant wound healing and possibly other etiologies. Histopathologic studies of the Peyronie’s plaque have demonstrated the role of transforming growth factor-a1 in the pathologic development of penile plaque. Animal models have been developed to test pathophysiology and therapeutics. The role of inducible nitric oxide synthase and nitric oxide has been established to limit and counteract fibrosis. Differential gene expression studies have identified candidate up-regulated or down-regulated genes that are involved in the pathophysiology of Peyronie’s disease     

Ultrasonographic evaluation of the first extensor compartment of the wrist in de Quervain’s disease

Background

This prospective cohort study was undertaken to explore the hypothesis that the retinaculum is thicker in de Quervain’s disease and to identify another landmark for the presence of the intracompartmental septum.

Methods

A case group (60 wrists) comprised of Korean female patients with unilateral de Quervain’s disease and a healthy control group (60 wrists) were evaluated by ultrasonography. The case group underwent surgery for extensor retinacular release, and surgical findings were used as the reference standard. The mean extensor retinacular thickness in the case and control groups was compared using a Wilcoxon rank-sum test. A receiver operation characteristic curve was constructed for the parameter.

Results

The mean thickness of the extensor retinaculum was 0.94 mm (SD 0.37) in the case group and 0.35 mm (SD 0.07) in the control group, and this difference was significant. The cutoff value of the extensor retinaculum for diagnosing de Quervain’s disease was 0.45 mm (sensitivity 96.3 %, specificity 93.3 %). Bony crests were found in all cases of presence of the intracompartmental septum and could be classified into three types according to shape.

Conclusions

We concluded that the extensor retinaculum is thicker in de Quervain’s disease. And the bony crest on the radial styloid can be considered a new landmark for determining the presence of an intracompartmental septum.

Type of study and level of evidence

Diagnostic, level II.