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.     

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.     

Castleman's disease in two patients with spondyloarthritis treated by Tumor Necrosis Factor-alpha antagonists

Castleman's disease is a rare lymphoproliferative syndrome involving several anatomical and clinical entities. We report two cases of Castleman's disease in patients with spondyloarthritis (with and without psoriasis) treated with Tumor Necrosis Factor-alpha blockers. During follow-up, imaging examinations, carried out for disease assessment, fortuitously revealed a pelvic mass close to the psoas or an atypical cervical adenopathy. Both lesions were in moderate hypermetabolism on Positron emission tomography–computed tomography. After surgical excision and histological analysis, Castleman's disease was diagnosed without signs of malignancy. To the best of our knowledge, these are the first cases of Castleman's disease diagnosed in a context of spondyloarthritis treated with Tumor Necrosis Factor-alpha blockers. The relationship between these two pathologies and the link with Tumor Necrosis Factor-alpha blockers agents has not been explored in the literature until now.     

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.     

Increased serum vitamin B12 levels are associated with adult-onset Still's disease with reactive macrophage activation syndrome

BackgroundAdult-onset Still's disease (AOSD) can be complicated by reactive macrophage activation syndrome (rMAS). The objective of this study was to evaluate vitamin B₁₂ values in AOSD with and without rMAS.MethodsAll patients’ files with AOSD in one center were retrospectively reviewed. Hemophagocytosis was defined as phagocytosis of various hematopoietic cells by macrophages. Clinical data including fever, rash, sore throat, arthritis, lymphadenopathy were recorded. Laboratory tests included complete blood count, serum ferritin, transaminases, serum triglyceride and vitamin B₁₂ level. The control group was selected from our AOSD pool who had AOSD without rMAS.ResultsSeven patients (5 female) had AOSD with rMAS. Median age at the diagnosis of rMAS was 32 (range, 27–37) and median follow-up duration after rMAS diagnosis was 18 months (range, 2–60). All of the patients with rMAS had fever, sore throat, rash, arthritis, anemia and hyperferritenemia. Five of seven patients had hepatosplenomegaly and lymphadenopathy. Four of seven patients had normal or low leucocyte count, three of seven patients had increased triglyceride level. The patients with AOSD and rMAS mean ± standard deviation (S.D.) vitamin B₁₂ levels were significantly higher than without rMAS (1903 ± 960 vs 542 ± 328 pg/ml, p = 0.001). The specificity (75%) of increased vitamin B₁₂ level was high and sensitivity (100%) was excellent.ConclusionElevated vitamin B₁₂ levels seems to be a good marker for diagnostic marker in AOSD when complicated with rMAS.     

Still's disease in adulthood--case report of a female patient and review of the literature

A 29-year old women with adult onset Still's disease (AOSD) was presented. On addmision to hospital the patient was febrile, with sore throat, arthralgia/arthritis and myalgia. The patient had lymphadenopathy on the neck, and laboratory findings showed leukocytosis with neutrophilia, accelarated erythrocyte sedimation rate (ESR), increased reactants of acute inflammation and normocytic anemia. During the diagnostic process, infectious, hematologic and neoplastic diseases were ruled out and the diagnosis of AOSD was made. The therapy with metilprednisolone 1 mg/kg of body weight was started. One month latter, the clinical status of the patient improved as well as laboratory findings. The dose of steroids was tappered to 16 mg per day. After 6 months of therapy, steroids were stopped and patient was followed further one year and she was free of symptoms of disease.     

Cyclooxygenase-2 inhibitor-associated minimal-change disease

Selective cyclooxygenase-2 (COX-2) inhibitors are relatively newer anti-inflammatory drugs that produce comparable antiinflammatory and analgesic effects to the nonselective nonsteroidal antiinflammatory drugs (NSAIDs); but with fewer symptomatic gastric and duodenal ulcers. Limited data are available concerning the toxicity associated with COX-2 inhibitors outside the gastrointestinal tract. The NSAIDs have been known for their nephrotoxic potentials including minimal-change disease (MCD) with interstitial nephritis. Although the recent data suggests that COX-2 inhibitors may have the same adverse renal effect as NSAIDs, there is only one case report describing minimal change disease and acute interstitial nephritis (AIN) associated with a COX-2 inhibitor, celecoxib. We are reporting a case of MCD and acute tubular necrosis (ATN) but without interstitial nephritis in a patient treated with celecoxib. Although the proteinuria in our patient resolved completely after discontinuation of celecoxib, the renal function did not. We suggest that heightened suspicion of this side effect of COX-2 inhibitors should be maintained in all patients taking this class of drugs who present with nephrotic syndrome.     

Liver transplantation for acute liver failure caused by macrophage activation syndrome

Macrophage activation syndrome (MAS) is a rare, potentially fatal condition, which most frequently complicates rheumatological conditions and is often associated with liver dysfunction. In this case report of a patient with MAS, acute liver failure developed despite conventional immunosuppressive therapy. Liver transplantation resulted in rapid recovery and the patient has remained well for six years. A recent diagnosis of Adult Onset Still's Disease (AOSD) provides additional supporting evidence that the initial presentation was caused by MAS. While transplantation in the context of systemic disease remains controversial, this first reported case of successful adult liver transplantation for acute liver failure caused by MAS raises an interesting clinical dilemma.     

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.     

New Markers for Adult-Onset Still's Disease

Adult-onset Still's disease (AOSD) is a rare systemic auto-inflammatory disorder (SAID). Although the pathogenesis of the disease is complex and far from being fully understood, recent progresses in pathophysiological knowledge have paved the way to new diagnostic approaches. Indeed, AOSD diagnosis can be a real challenge, owing to its infrequency, and to the lack of specificity of the principal clinical features (high fever, arthralgia or arthritis, skin rash) and laboratory findings (elevated acute phase reactants, hyperleukocytosis ≥ 10,000 cells/mm³ with neutrophils ≥ 80%). None of these manifestations is disease-specific, so clinicians must first rule out neoplastic, infectious or inflammatory conditions. Besides these diagnostic difficulties, several other challenges remain. AOSD is very heterogeneous in terms of clinical presentation, evolution and severity. Thus, new biomarkers are required to assess: (i) disease activity; (ii) disease severity (through the identification of patients at risk of severe organ failure, and eventually of life-threatening complications, such as reactive haemophagocytic lymphohistiocytosis); (iii) disease evolution (which can be monophasic, relapsing, or progressive, with either systemic inflammation or chronic erosive arthritis); (iv) and treatment efficacy. The identification of new markers can only be done through a better understanding of the pathogenesis of the disease. After a short focus on the current AOSD pathophysiological knowledge, this article reviews the main biomarkers that have been proposed in the literature over the last few years.     

Intravenous anakinra to curb cytokine storm in adult-onset Still's disease and in macrophage activation syndrome: A case series

ObjectiveAdult-onset Still's disease (AOSD) is an auto-inflammatory polygenic disorder, for which the diagnosis is essentially clinical. The exclusion of mimickers [such as common bacterial and viral infections, hematologic malignancies, and, more recently, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)] is necessary to confirm the diagnosis. Anti-interleukin (IL)-1 therapy is considered a treatment milestone for AOSD. Herein, we present a short series of newly-diagnosed AOSD or upcoming macrophage activation syndrome (MAS) cases who received intravenous (IV) anakinra, an IL-1 receptor blocker.MethodsFour patients with newly-diagnosed AOSD or upcoming MAS were treated with IV anakinra at the Rheumatology Unit of Padova University Hospital, Italy. We obtained informed consent from the patients for use of their cases and medical images for publication purposes.ResultsAll patients presented with AOSD or MAS during the COVID-19 pandemic, making diagnosis challenging due to similar immunological and clinical characteristics across both pathologies. All patients presented with hyperpyrexia and elevated inflammatory markers; two patients had a skin rash typically seen in AOSD. IV anakinra slowed down AOSD progression in all patients, prevented severe outcomes and mitigated the risk of multiorgan failure. All cases improved within 24 hours of anakinra administration.ConclusionWe found that administration of anakinra in patients with newly-diagnosed AOSD and/or upcoming MAS reduced hyperinflammation and prevented life-threatening complications. The IV route appears to be preferable in the hospital setting, where comorbidities such as coagulopathies and thrombocytopenia can complicate the use of other routes of administration.     

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.     

腹膜后局灶性Castleman病的临床分析并文献复习

目的分析总结腹膜后局灶性Castleman病(localized Castleman’s disease,LCD)的临床特点及诊治方法,以期对该病的诊断和治疗过程提供合理指导意见。方法回顾性分析2014年12月武汉大学人民医院收治的1例腹膜后LCD病人临床资料,并针对性复习相关文献。结果病人术前检查未明确诊断,手术行腹膜后肿瘤切除,术后病理切片检查确诊,未行辅助性治疗。1年后随访复查,病人恢复可,手术效果较好。结论腹膜后LCD术前诊断较困难,手术切除预后良好,术前充分评估可以为治疗方式的选择以及手术方式提供依据,提高手术成功率,减少副损伤及并发症。     

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.     

Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis

Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease.     

Psoas abscess complicating Crohn's disease

Psoas abscess complicating Crohn's disease is an established but relatively rare condition that can present a difficult management problem. A high index of suspicion is paramount in early diagnosis and advances in therapeutic imaging techniques may allow a non-operative approach in the initial treatment plan. We also advocate consideration of definitive surgical management if suitable. We present one such case and a review of the literature.     

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...     

A Case of Adult Onset Still's Disease Misdiagnosed as Septic Arthritis

We present a case of adult onset Still's disease (AOSD) that was misdiagnosed as septic arthritis of the shoulder and knee. A forty-nine-year-old woman was admitted for pain in the left knee. The patient's medical history showed that she had undergone arthroscopic irrigation twice and an open debridement under the diagnosis of septic shoulder at another hospital. The laboratory and joint fluid analysis findings led us to suspect septic knee. Arthroscopic irrigation and antibiotics treatment were performed. At five weeks after discharge, she presented with pain in the same joint, fever, and rash. The symptoms were consistent with Yamaguchi's criteria for AOSD. We started corticosteroid therapy, and clinical remission was achieved. In conclusion, we suggest that AOSD should be considered as a diagnosis of exclusion to avoid misdiagnosis with septic arthritis.     

Autosomal dominant medullary cystic disease: a disorder with variable clinical pictures and exclusion of linkage with the NPH1 locus

Background. The nephronophthisis-medullary cystic disease (NPH/MCD) complex represents a heterogeneous group of hereditary tubulointerstitial nephritis. The most common variant is juvenile recessive NPH, for which a gene locus (NPH1) has been mapped on chromosome 2q13. MCD is a less common dominant condition usually recognized later in life, which resembles NPH in many aspects, still presenting remarkable clinical differences. Nothing is known about the chromosome locus of MCD. Methods. Five MCD families were studied. Diagnosis was made by inference from family history, type of inheritance, clinical signs and histology. Multipoint linkage analysis was performed by markers D2S293, D2S340 and D2S160 spanning the entire NPH1 locus. Results. Diagnosis of MCD was made in 28 affected members (16 males; 12 females), belonging to five families. Histological diagnosis was available in 10 patients; clinical diagnosis in 11; seven deceased relatives had diagnosis of chronic nephritis. The age at diagnosis ranged from 8 to 65 years. Renal medullary cysts were found in a minority of patients. In family 1, the disease was associated with hyperuricaemia and gouty arthritis. Progression of renal disease presented intra- and extra-family variability with members of the same family showing mild elevation of creatinine or terminal renal failure. The NPH1 locus associated to recessive NPH was excluded from linkage to the dominant MCD. Conclusions. MCD might be more common than previously assumed. Variability in clinical presentation and absence of histopathological hallmarks contribute to make the diagnosis uncommon. The most remarkable clinical difference with NPH is the age of onset in some kindreds and a delayed progression towards renal failure. The exclusion of linkage to the NPH1 locus suggests the existence of an MCD responsible locus, still to be mapped.