Angiofollicular lymph node hyperplasia resembling a spinal nerve sheath tumor: a rare case of Castleman's disease

Background contextAngiofollicular lymph node hyperplasia (Castleman's disease) is a lymphoproliferative disorder of unknown etiology. Although uncommon, the localized form of this disease can manifest in the central nervous system, typically as a meningeal-based intracranial lesion. Castleman's disease involving the spine is exceedingly rare. This represents only the second reported case of a patient with Castleman's disease whose presentation mimicked that of a spinal nerve sheath tumor.PurposeWe report a rare case of angiofollicular lymph node hyperplasia that mimicked a spinal nerve sheath tumor and was treated with gross total resection.Study designCase report.Patient sampleA 31-year-old female with angiofollicular lymph node hyperplasia presenting with a paraspinal mass.Outcome measuresThe patient's outcome was based on clinical history, physical and radiographic examinations.MethodsA 31-year-old woman with a mediastinal mass previously diagnosed as sarcoid via biopsy presented with the new onset of radicular pain and radiographic enlargement of her mass. Magnetic resonance imaging demonstrated a 4.3×5.7×6.0 cm homogenously enhancing soft-tissue lesion that emanated from and widened the T3/T4 neural foramen on the left. The differential diagnosis based on the location of the lesion and imaging characteristics included schwannoma, neurofibroma, paraganglioma, sarcoid, and lymphoma. Gross total resection was performed via thoracotomy. Histological examination identified angiofollicular lymph node hyperplasia (Castleman's disease) of the hyaline-vascular subtype. The patient did not receive adjuvant chemotherapy or radiation therapy.ResultsThe patient had resolution of her symptoms without any clinical or radiographic evidence of recurrence at 1-year follow-up using magnetic resonance imaging with and without contrast.ConclusionsCastleman's disease is a rare pathologic entity that should be considered in the differential diagnosis of a patient with a paraspinal mass. Spine surgeons should be aware of this diagnosis as it has treatment and follow-up implications that differ from the neoplasms it can mimic. Complete surgical excision is an effective treatment for solitary lesions. Screening for concurrent and future malignancies in these patients is prudent.     

Multicentric Castleman's disease mimicking adult-onset Still's disease

Castleman's disease is a rare lymphoproliferative disorder having two types of presentation: the localized and the multicentric form. Multicentric Castleman's disease (MCD) typically presents with constitutional symptoms, generalized peripheral lymphadenopathy, hepatosplenomegaly, and laboratory markers of inflammation. Rash and arthritis may also be initial complaints of this disease. In these cases, MCD can resemble adult-onset Still's disease (AOSD), especially if the arthritis precedes other manifestations.We describe a patient with initial clinical suspicion of AOSD. Eighteen months later evidence of MCD was ascertained when the patient developed insidiously growing axillary lymphadenopathies. Despite its rarity, MCD should be borne in mind in the differential diagnosis of patients with suspicion of AOSD.     

Castleman's disease arising from the chest wall

An 18-year-old man was referred to our hospital because of an abnormality on chest radiography. Computed tomography showed a sharply defined, 5 × 2 × 3?cm, densely enhanced mass in the left side of the chest wall. We performed tumor resection under video-assisted thoracoscopy, with minimal bleeding. Histologically, the tumor was shown to be Castleman's disease. This is the eleventh case of Castleman's disease arising from the chest wall reported over the last 20 years.     

Castleman's disease in the pelvic retroperitoneum: A case report and review of the Japanese literature

INTRODUCTIONCastleman's disease is a fairly rare benign tumor of lymphoid origin. It can develop anywhere lymphoid tissue is found, but the expected origin is mediastinum and rarely pelvic retroperitoneum.PRESENTATION OF CASEA 22-year-old woman was admitted to our hospital for a mass in the pelvic retroperitoneum that was detected incidentally on an ultrasonography during a routine medical checkup with no signs of symptoms. After laboratory examination, ultrasonography, and magnetic resonance imaging (MRI), surgical resection was performed successfully through a lower midline incision. But the patient was needed transfusion because of massive bleeding. Postoperative histopathological diagnosis was hyaline-vascular type of Castleman's disease. The patient is leading an active social life without any signs of sequelae or recurrence.DISCUSSIONThrough the review of Japanese literature on Castleman's disease in the retroperitoneum, the characteristics of preoperative imaging findings are studied. Castleman's disease is easily misdiagnosed clinically because of its scarcity and no specific imaging findings. And the embolization via angiography should be considered in the hypervasular tumors such as in this case to prevent massive bleeding and transfusion.CONCLUSIONAlthough Castleman's disease is uncommon, it should always be included in the differential diagnosis of pelvic retroperitoneal tumors. A better knowledge of this disease would help surgeon to avoid unnecessarily extensive resection and massive bleeding for transfusion when dealing with retroperitoneal tumors.     

Paraneoplastic pemphigus associated with a pericardial ectopic thymoma

Paraneoplastic Pemphigus (PNP), a rare autoimmune blistering disease, can be accompanied by both benign and malignant neoplasms. The most frequently reported associated malignancies include lymphomatoid and hematologic malignancies, Castleman's disease, carcinoma, thymoma. In a patient suspected of PNP, with no known history of malignancy, an extensive workup is suggested to look for underlying malignancy, which has to be treated to induce PNP remission. In this clinical case report, cross sectional imaging of a young female diagnosed with PNP, unveiled a pericardial mass lesion extending into transverse pericardial sinus. Excisional biopsy was performed. Histopathology revealed pericardial ectopic thymoma.     

Diagnose und Therapie der axialen Spondyloarthritis einschlie?lich des Morbus Bechterew

The term axial spondyloarthritis covers patients with established structural changes visible on x-ray in sacroiliac joints and/or in the spine (classical ankylosing spondylitis) but also patients with non-radiographic axial spondyloarthritis in whom early inflammatory signs of the disease can only be visualized with magnetic resonance imaging (MRI). The MRI technique plays an important role in the diagnosis of this disease and an early diagnosis is normally based on a combination of clinical, laboratory and imaging parameters. Only non-steroidal anti-inflammatory drugs and TNF-α blockers are effective in the treatment of axial spondyloarthritis. Patients with short disease duration and elevated acute phase reactant levels demonstrate best therapy response and, therefore, should be closely followed-up and consistently treated.     

Amylose AA

AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthritis and autoinflammatory diseases. Castleman's disease is a specific cause of AA amyloidosis that can be cured by surgery. A chronic inflammatory response is required to develop amyloidosis. Other genetic and environmental factors are also involved. The first clinical manifestation is a chronic glomerular nephropathy, which can be detected by urine examination and serum creatinine measure. Immunohistochemistry is mandatory to confirm the clinical diagnosis of AA amyloidosis and to avoid misdiagnosis. Long-term prognosis remains poor on chronic dialysis in case of clinical gut involvement. Current treatment is based on the control of the inflammatory response. Specific treatment aimed at inhibiting amyloid formation targeting serum amyloid P component and heparan sulphate are currently evaluated.     

Localized Castleman's disease in the breast

INTRODUCTIONCastleman's disease (CD) is a rare disease with unknown etiology and is clinically associated with lymph nodes enlargement. Primary axillary localization of CD represents 2% of the cases. CD rarely occurs in the breasts.PRESENTATION OF CASEWe herein describe a rare case of CD that initially presented in the breast intramammary lymph node and demonstrated axillary adenopathy. Pathologic analysis showed the hyaline vascular form. The patient underwent axillary lymphadenectomy. The natural history was irregular because the localized CD progressed to a systemic form of CD. At 4.6 years of follow-up a Hodgkin's lymphoma appeared.DISCUSSIONThis is the fourth published case of localized breast CD published. It is important to evaluate other clinical lymphadenopathies at the time of diagnosis, and computed tomography is important for disease evaluation and follow-up.CONCLUSIONPatients must undergo axillary lymphadenectomy when showing clinical symptoms. Irregular progression prompts new lymph node biopsy because of the potential presence of associated diseases.     

In vitro antiproliferative efficacy of Interferon-alpha,-gamma and Tumor Necrosis Factor on two human renal tumor xenografts

Summary The in vitro antitumor activity of recombinant alpha-and gamma-Interferon as well as recombinant Tumor Necrosis Factor alpha was tested on renal cell carcinoma xenografts using the double layer soft agar method. Using this assay, the effect of the drugs on the clonogenic potential of tumor cells in soft agar was determined and used as an indication for the antiproliferative capacity of these drugs. There appeared to be a differential response of the tested xenografts towards these drugs in a dose dependent way. However, when used in combination in most cases an additive or synergistic effect was observed. Even though again the response was differential, the combination of alpha-Interferon (10 ng/dish) and Tumor Necrosis Factor (100 ng/dish) resulted in a complete inhibition of colony formation in soft agar for both tumor lines. We conclude that there is a direct effect of alpha-Interferon, gamma-Interferon and Tumor Necrosis Factor on renal cell carcinoma xenografts. In combination drug tests the effect of alpha-Interferon and Tumor Necrosis Factor was strongly synergistic. The implication of these studies for in vivo use of these drugs remain to be established.     

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...     

Clinical characteristics of anterior chest wall pain in spondyloarthritis: An analysis of 275 patients

ObjectivesAnterior chest wall pain is a common but little studied feature of spondyloarthritis. The objectives of our study were to assess the prevalence of anterior chest wall pain and to describe its clinical characteristics in a cohort of spondyloarthritis patients in a tertiary care center.MethodsStudy design: retrolective single center observational study in 2010 (COSPA). Consecutive patients with definite spondyloarthritis according to Amor's criteria were included. Data collection: each patient underwent direct interview by a physician. Prevalence of anterior chest wall pain, according to spondyloarthritis subtype and its date of appearance, localization and nature were collected.ResultsIn all, 275 consecutive spondyloarthritis patients were assessed. Among them, 102 patients (37.1%) suffered from spondyloarthritis-associated anterior chest wall pain. It was the first symptom of spondyloarthritis in 3.6% of cases. The prevalence after 5 and 10 years following the diagnosis of spondyloarthritis was 26.0% and 35.5%, respectively. Pain was usually in the upper chest and acute, increased by respiratory movements and movements of the arm; pain during the night was less frequent (41.0%). A flare lasted on average 5 weeks; recurrences were frequent (75%). Non-steroidal anti-inflammatory drugs and anti-tumor necrosis factor agents were reported as effective in 49.3% and 80.0% of cases, respectively.ConclusionAnterior chest wall pain was a frequent manifestation in spondyloarthritis. It occurred early in the disease course, but the risk persisted after disease onset. Better knowledge of the clinical characteristics of this symptom may help physicians for diagnosis and follow-up.     

Retroperitoneal unicentric Castleman's disease—A case report and review of literature

Introduction and importanceCastleman's disease is not so commonly diagnosed worldwide due to non-specific symptoms Clinical findings are variable.No definite blood investigation or any biomarkers are established to diagnose this disease. Radiological investigations do not play much role in diagnosing. It can be unicentric or multicentric. Etiological factors are not well understood except predilection of this disease towards immune-compromised persons. Surgery is considered as a prime modality to treat, if resectable.Case presentationPatient had recurrent abdominal pain in left lumbar region which was intermittent for last 6 months, dull aching with no aggravating and relieving factors. There was no history of abnormal bowel habits, urinary complaints, fever, night sweats, weight loss, rashes in body, joint pains and loss of appetite. On examination, there was a single, firm, non-tender, intra-abdominal, retroperitoneal mass of approximately size 10 1 7 cm in left lumbar region encroaching inferiorly towards left inguinal region. Digital rectal examination (DRE) and external genitalia were normal.Clinical discussionAll routine blood investigations along with relevant tumour markers were normal. Magnetic Resonance Imaging (MRI) abdomen showed a well-defined mass suggestive of stromal tumour or retroperitoneal sarcoma. Patient underwent surgery in which complete excision of the mass was done. Postoperative event was uneventful and currently doing well.ConclusionTake away lesson in this case report is that we should not presume all retroperitoneal mass as cancer or sarcoma, we have to think about other rare causes like Castleman's disease, if clinical picture, blood and radiological investigation are discordant.     

Osteomyelitis complicating Sever‘s disease: A report of two cases

Sever's disease is a common cause of heel pain in young children and it has a self-limiting course without long term complications. Osteomyelitis of calcaneus is a sinister condition which can lead to serious complication if treatment is delayed. Indolent nature of calcaneus osteomyelitis leads to overlap in its clinical picture with Sever's disease. Two cases of Sever's disease which were later diagnosed and managed for osteomyelitis are reported. Salient differentiating features of Sever's disease and calcaneus osteomyelitis along with management strategy are discussed.     

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.     

Association between hidradenitis suppurativa and spondyloarthritis

Objectives

We aimed to investigate the existence of an association between hidrosadenitis suppurativa and spondyloarthritis.

Crohn's disease of the duodenum. A report and analysis of eleven new cases

Eleven new cases of Crohn's disease involving the duodenum are reported. It is rare for the duodenum to be involved without other segments of the bowel also being involved. Only one of our patients had isolated duodenal disease. The clinical presentation suggests peptic ulceration. Therapy is conservative, with surgery restricted to those patients with obstruction. The procedure of choice is bypass gastrojejunostomy. Six patients are now asymptomatic, four have had symptomatic relief, and one has died. It is likely that the incidence of Crohn's disease of the duodenum is more frequent than the number of reported cases would suggest, but that these cases are not diagnosed because of unfamiliarity with the condition.     

Frontal bone hyperostotic mass associated with fibrous dysplasia in a male patient with systemic lupus erythematosus (SLE)

We present a rare clinical report of a 45-year-old man affected by Systemic Lupus Erythematosus (SLE) with a unilateral mass of the left frontal bone, diagnosed as a possible recurrence of fibrous dysplasia. This patient was evaluated with computed tomography (CT scan) and was treated with resection of the mass and reconstruction with splitting a calvarian bone graft. The pathological evidence was suggestive for a bone cavernous haemangioma.No previously described cases of bone dysplasia associated with this systemic syndrome were reported so far. Patient's disease was under stringent control at the time of hospitalization, and the outcome has been successful, even though a mild increase of inflammatory indexes was reported after surgery. This laboratory evidence was transient and not related to further clinical complications.     

Local sacroiliac injections in the treatment of spondyloarthritis. What is the evidence?

Local sacroiliac injections represent an available treatment option in spondyloarthritis (SpA). There is no synthetic data about efficacy of this procedure in SpA. The aim of this review is to analyze the available data of the literature of local sacroiliac injections in SpA.MethodsA Pubmed search of papers published after 1990 was performed.ResultsPubmed search retained 15 publications in spondyloarthritis, 12 with corticosteroids (total of 468 injections in 268 patients), including 2 small-sized controlled studies, and 3 with TNF blockers (24 patients in open studies). With steroids, the two controlled studies (with a total number of 30 patients only) demonstrated significant results over placebo. In open studies, good response was reported in more than 80% of the cases, with a mean duration of improvement over 8 months. Reduction of sacroiliac inflammation on MRI was also found in several studies after injection. There is no consensus about the technique of injection or the type of steroid injected. No particular safety concern was reported. Many limitations have to be considered in the interpretation of these results.ConclusionThis kind of procedure should be kept in the therapeutic armament in the current setting of costly targeted systemic treatments of spondyloarthritis. However, definition of a clear position in the treatment strategy needs further well-conducted studies.     

Two cases of Takayasu's arteritis occurring under anti-TNF therapy

Takayasu's arteritis is a granulomatous, large vessel vasculitis that affects the aorta, its major branches and the pulmonary arteries. Compelling evidence exists to support the notion that Takayasu's arteritis is a T-cell mediated process and that tumor necrosis factor alpha (TNFa) is an important factor in the pathogenesis of this disease. Moreover, encouraging results from recent studies support the use of anti-TNFa therapy for relapsing or resistant cases of Takayasu's arteritis. Here, however, we describe the case of two patients: one with seropositive rheumatoid arthritis, the other with HLA-B27 negative spondylarthropathy, who developed Takayasu's arteritis during treatment with TNFa inhibitors (adalimumab and golimumab respectively). This is the first report of Takayasu's arteritis in rheumatic patients under TNFa blocking agents which suggests the presence of different pathogenetic mechanism in a subgroup of patients with Takayasu's arteritis, as well as a potential role of TNFa blockers as triggers of this disease in some cases.