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Abstract: An elderly man presented with fever and chills for six months, associated with loss of appetite and weight loss, bilateral edema, and non-tender inguinal lymphadenopathy. The left-sided testicle was bulky, firm, and non-tender. Investigations revealed anemia, leukopenia, elevated erythrocyte sedimentation rate, and hypergammaglobulinemia. Ultrasound of the abdomen revealed multiple enlarged necrotic lymph nodes in the periaortic, preaortic, precaval, and bilateral pelvic regions. Fine-needle aspiration cytology (FNAC) of the inguinal nodes showed no acid-fast bacilli (AFB). However, excision biopsy revealed lymphoid tissue with effacement of architecture by diffuse proliferation of mature plasma cells, suggestive of the plasma cell variant of multicentric Castleman disease. Due to suspicion of neoplastic testicular enlargement, the patient underwent bilateral orchidectomy, and histopathology showed hematocele with atrophy of the testis and secondary involvement of the testicular parenchyma by plasma cell variant of Castleman disease., (Copyright © 2023 Copyright: © 2023 Journal of Cancer Research and Therapeutics.)

BACKGROUND Castleman's disease (CD) is a reactive lymph node hyperplasia initially identified by Castleman in 1956. CD predominantly affects individuals 20-50 years of age, with low incidence in children. This case report describes 3 cases of CD treated in our hospital and reviews the relevant literature. The purpose of this case report was to enhance clinical understanding and treatment of CD in the head and neck in children. CASE REPORT To enhance clinical understanding and improve treatment of CD in the head and neck region in children, we present the cases of 3 patients who were admitted to the hospital, primarily presenting with a neck mass. Preoperatively, the patients collectively exhibited non-specific findings. Surgical interventions were performed with Cases 1 and 3 undergoing left functional (radical) neck lymph node dissection, in contrast to Case 2, in which bilateral functional (radical) neck lymph node dissection was executed. Pathological examination confirmed the diagnosis of CD in each of the 3 patients. Following surgery, a follow-up period ranging from 3 months to 1 year revealed that all patients had successfully recovered, with no recurrence. CONCLUSIONS Castleman disease is a rare disease in children and difficult clinical diagnosis. Some patients with unicentric Castleman disease (UCD) can be treated with surgery, and those with multicentric Castleman disease (MCD) need chemotherapy, but at present there is no widely accepted treatment plan.

Background: Castleman disease (CD) is an uncommon benign lymphoproliferative disease characterized by hypervascular lymphoid hyperplasia. We present a unique case of unicentric CD of the parotid gland treated by preoperative direct puncture embolization., Case Presentation: A 27-year-old female patient was admitted for a right neck mass. Ultrasound examination and MRI scan documented a hypervascular mass within the right parotid gland. Preoperative embolization was performed by direct puncture technique: a needle was inserted into the core of the mass under both ultrasound and fluoroscopic guidance and SQUID 12 was injected into the mass under fluoroscopic control, achieving a total devascularization., Conclusion: Preoperative direct puncture embolization was safe and effective and provides excellent hemostatic control during the surgical operation, limiting the amount of intraoperative bleeding.

Background: Castleman's disease is a rare lymphoproliferative disorder that is often misdiagnosed because of its untypical clinical or imaging features except for a painless mass. Besides, it is also difficult to cure Castleman's disease due to its unclear pathogenesis., Case Presentation: We present a Castleman's disease case with diagnostic significance regarding a 54-year-old Chinese male who has a painless mass in his left parotid gland for 18 months with a 30-years history of autoimmune disease psoriasis. Computed tomography scan showed a high-density nodule with clear boundaries in the left parotid and multiple enlarged lymph nodes in the left submandibular and neck region. General checkup, the extremely elevated serum interleukin-6 and lymph node biopsy in the left submandibular region gave us an initial suspicion of Castleman's disease. Then the patient underwent a left superficial parotidectomy. Based on histopathologic analysis, we made a certain diagnosis of Castleman's disease and gave corresponding treatments. In 18 months of follow-up, the patient showed no evidence of recurrence, with the level of serum interleukin-6 decreased., Conclusions: Clinicians should be aware of the possibility of Castleman's disease when faced with masses or enlarged lymph nodes in the parotid gland to avoid misdiagnosis, especially in patients with autoimmune diseases and elevated serum interleukin-6., (© 2024. The Author(s).)

Castleman disease (CD) is a rare, benign lymphoproliferative disorder with characteristic histopathological features, but variable aetiology, presentation, treatment and prognosis. It is broadly classified based on its location and histopathological features, with unicentric hyaline vascular disease presenting most commonly. We present a case of primary, unicentric hepatic CD that was incidentally found on imaging and managed with laparoscopic resection., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Castleman's disease is a rare and benign lymphoproliferative disorder which can be unicentric (UCD) or multicentric (MCD). UCD usually involves a single lymph node or less frequently a group of lymph nodes. The most common sites of nodal UCD presentation are the mediastinum, neck, abdomen and retroperitoneum. Rarely extranodal involvement has been reported. The intramuscular location is very unusual with only about 10 cases described in medical literature so far., Case Report: We present a case of atypical localization of Castleman's disease occurring in the right gluteal area in a 40-years-old female patient. The patient was asymptomatic and clinical examination was unremarkable except for a right gluteal palpable mass. The CT scanner-guided needle core biopsy was inconclusive. A surgical excision was then performed that revealed a hyaline-vascular type of Castleman's disease. The patient has an uneventful post-operative course., Conclusion: The present case is instructive in the work-up of primary soft tissue tumors, for which Castleman's disease is extremely rare and not considered in the differential diagnosis of clinicians. Pathologists must be aware of its existence so that it can be evoked in the presence of a lymphoid population on histological examination., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Background: Idiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disease often associated with pulmonary involvement. Recently, transbronchial lung cryobiopsy (TBLC) has been reported to be useful for the diagnosis of diffuse interstitial lung disease. However, there have been no reports of pathological assessment of TBLC for iMCD., Method: To clarify the efficacy of TBLC in the diagnosis of iMCD, we retrospectively reviewed four iMCD patients who had undergone both TBLC and surgical lung biopsy (SLB)., Results: The median age was 44 years; 2 males and 2 females. Two or three TBLC specimens were taken from each patient. All patients had no complications other than minimal bleeding. The size of the TBLC specimens was approximately 5-6 × 3-4 mm, and the alveolar region, and centrilobular and perilobular areas were adequately sampled. As with SLB, the extent of lung lesions and inflammatory cell infiltration could be sufficiently evaluated by TBLC. The presence of lymphoid follicles could also be assessed by TBLC; however, the germinal centers with lymphoid follicles were difficult to evaluate. The TBLC specimens could also be evaluated for immunostaining, especially IgG4 immunostaining, to rule out IgG4-related lung disease. Pulmonary pathological grading showed a high concordance rate between major pathological findings of TBLC and SLB. The pathologist's confidence level of TBLC for the diagnosis of iMCD was high in all cases., Conclusions: TBLC exhibits a high concordance rate with SLB in the pathological evaluation of iMCD, which may be useful for the diagnosis of iMCD., Competing Interests: Conflict of Interest The authors have no conflicts of interest., (Copyright © 2023. Published by Elsevier B.V.)

Castleman's disease with calcification of the chest wall is very rare, and there have been few reports of such cases to date. A 57-year-old woman was referred to our hospital for a tumor with calcification on her left lateral chest wall, which was detected on chest computed tomography. Findings of her chest magnetic resonance imaging suggested schwannoma or a solitary fibrous tumor, and therefore, we performed surgery for diagnostic and therapeutic purposes. Pathologically, the tumor with calcification was diagnosed as Castleman's disease of the hyaline-vascular type. After the surgery, the patient has had no obvious symptoms and continues to undergo regular follow-up examinations.

Castleman disease is an uncommon lymphoproliferative disorder, located most commonly in the mediastinal lymph nodes. The intrapulmonary presentation is extremely rare, with 14 published cases in the English literature. The clinical presentation of the disorder is not specific and the diagnosis is often challenging. The main goal is to achieve an R0 surgical resection which gives the potential chance for a recurrence-free survival. We present the case of a symptomless, 15-year-old female patient with left-sided tumor mass. Transthoracal invasive tissue biopsy confirmed Castleman disease. The central mass involved the main structures in the left hilus and therefore left pulmonectomy was necessary to reach the complete, tumor-free resection margins. The patient had no local or distant relapse during the 7-year follow-up. Although Castleman disease treated by complete surgical resection provides excellent results, radical and extended lung resection is sometimes inevitable to reach tumor-free margins. Orv Hetil. 2023; 164(37): 1476-1483.

Castleman disease (CD) is a lymphoproliferative disorder that rarely occurs in the pancreas. We reported a 46-year-old man was admitted to our hospital due to recurrent minor epigastric pain for 7 days. The abdominal enhanced CT showed irregular foci in the body of the pancreas with abundant blood supply considered a neuroendocrine tumor. The patient was subsequently diagnosed with pancreatic neuroendocrine tumor by an ultrasound-guided fine needle aspiration biopsy. Therefore, he underwent an open pancreatic mass resection. The ultimate diagnosis was CD and belonged to hyaline vascular type based on the postoperative pathology. After a 13-month follow-up, no recurrence of abdominal pain or lesions was observed. This case suggests that fine needle aspiration biopsy is not conclusive and unsuitable for pancreatic CD and highlights the importance of routine pathology due to the absence of typical signs and symptoms.

Background: Castleman Disease (CD) is a group of diseases with characteristic lymph node histopathology, characterized by marked enlargement of deep or superficial lymph nodes. Adrenal CD is rarely reported, and an accurate preoperative diagnosis of adrenal CD is difficult., Method: We report four cases of CD in the adrenal gland confirmed by pathology and review the characteristics of this rare disease, highlighting the necessity of diagnostic evaluation and follow-up of the patients., Results: All of the patients sought medical advice because of adrenal incidentalomas. No significant abnormalities were presented in the biochemistry or endocrine systems. The imaging suggested a moderate-to-large mass with uneven moderate contrast enhancement of the adrenal region, similar to a pheochromocytoma. All cases were misdiagnosed as pheochromocytomas before operation and finally confirmed by histopathology. Three cases were pathologically diagnosed as hyaline vascular CD, and one case was diagnosed as plasma cell CD. All the patients are alive without recurrence after a median follow-up of 8 years., Conclusion: The adrenal CD should be considered after excluding pheochromocytoma and malignancy in the adrenal region. The long-term prognosis of patients with complete resection of the mass is excellent., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Yu, Wang, Li, Du, Guo, Gu, Lyu, Dou, Mu and Zang.)

Castleman disease is a rare lymphoproliferative disorder characterized by benign enlargement of lymph nodes. It is divided into unicentric disease, which involves a single enlarged lymph node, and multicentric disease, which affects multiple lymph node stations. In this report, we describe a rare case of a 28-year-old female patient with an unicentric Castleman disease. Computed tomography and magnetic resonance imaging revealed a well-circumscribed large mass in the left neck, characterized by intense homogenous enhancement and suspected for a malignant disease. The patient underwent an excisional biopsy for definitive diagnosis of unicentric Castleman disease and ruled out malignant conditions.

BACKGROUND Castleman disease was first described in 1956 as mediastinal masses composed of benign lymphoid hyperplasia with germinal center formation and capillary proliferation closely resembling thymomas. It has been linked with many multi-system disorders, including myasthenia gravis. Cases of Castleman disease with corresponding myasthenia gravis have higher rates of postoperative myasthenic crisis, which are reported as high as 37.5%. We encountered a case of Castleman disease with myasthenia gravis that was discovered early and managed successfully with complete surgical resection and no postoperative myasthenic crisis. CASE REPORT A 25-year-old woman with an uncomplicated history presented with shortness of breath, numbness in hands, tiring with chewing, and fatigue. Myasthenia gravis was diagnosed with serology test results, and a 7.5×7.0-cm mediastinal mass was discovered in addition to the incidental finding of a persistent left superior vena cava, closely abutting the mass. Biopsy showed lymphoid proliferation, regressed germinal centers surrounded by small lymphocytes, and vascular proliferation, consistent with unicentric Castleman disease, hyaline-vascular type. The patient was successfully treated for Castleman disease with myasthenia gravis, and no postoperative myasthenic crisis occurred. CONCLUSIONS Castleman disease associated with myasthenia gravis can dramatically increase the risk of postoperative myasthenic crisis. Our literature review of all 16 cases of Castleman disease with myasthenia gravis since 1973 revealed that 18.75% of cases were associated with a postoperative myasthenic crisis. This association elicits the importance of prompt diagnosis of myasthenia gravis when evaluating mediastinal masses and the value of having neurology and anesthesiology staff aware of the increased risk of crisis.

Castleman's disease is a rare lymphoproliferative disease, mostly found in the mediastinum. The number of Castleman's disease cases involving the kidneys is still limited. We report a case of primary renal Castleman's disease sporadically detected during a regular health check-up as pyelonephritis with ureteral stones. In addition, computed tomography showed renal pelvic and ureteral wall thickening with paraaortic lymphadenopathy. A lymph node biopsy was performed, but it did not confirm either malignancy or Castleman's disease. The patient underwent open nephroureterectomy for diagnostic and therapeutic purposes. The pathological diagnosis was renal and retroperitoneal lymph node Castleman's disease with pyelonephritis.

Castleman disease (CD) is a benign lymphoproliferative disease. Small prevalence and diverse clinical course of disease makes it difficult to standardize diagnostics and treatment. Currently, the number of CD patients has increased with improvement in the quality of examination. Therefore, differential diagnosis of this disease is important. We present a young patient with CD and retroperitoneal non-organ neoplasm. Despite a thorough preoperative examination, the final diagnosis was established only after histological examination of surgical specimen. We discuss the diagnosis and surgical treatment of a patient with unicentric type of CD.

Abstract: Castleman's disease is a rare angio-follicular lymph node hyperplasia[1,2] have varying presentation and can mimic malignancy. The common locations are mediastinum, neck, axilla, and pelvis but extra nodal sites can also be affected.[3] A 56-year-old patient incidentally found to have mass in the retroperitoneal region by Contract-enhanced computerized tomography (CT). Positron emission tomography scan showed well defined intensely enhancing fluorodeoxyglucose (FDG) avid lesion in the left iliac region with vascularity. Surgery was performed, and pathological diagnosis was hyaline vascular type-stroma-rich variant of Castleman disease. Here, we report a case of huge retroperitoneal Castleman's disease in a 56-year-old female patient.[4]., (Copyright © 2023 Copyright: © 2023 Journal of Cancer Research and Therapeutics.)

Background: Oral lichenoid lesion (OLL) is a term used to describe oral lesions that have clinical and/or histopathological features similar to oral lichen planus (OLP), but it is thought to be caused by specific triggers or systemic conditions and presents higher malignant transformation rate than OLP. To date, OLL simultaneously complicated with Castleman's disease (CD) and papillary thyroid carcinoma (PTC) has not been reported. Reporting from such disorders is crucial to avoid misdiagnosis and help in timely intervention., Case Presentation: We report a rare case of a 39-year-old female with extensive ulcerated lesions on the oral mucosa, diagnosed as OLL by histopathology. Routine oral treatment was scheduled to control the OLL, while the oral lesions remained unhealed. Computed tomography examination was performed after the oral treatment and revealed thyroid and mediastinal masses, which were then surgically removed and pathologically diagnosed as PTC and CD, respectively. Two months after complete excision of the neoplasms, the oral lesions showed obvious alleviation. With subsequent treatment for oral lesions, the patient's OLL healed., Conclusions: This is the first reported OLL case simultaneously associated with CD and PTC. This case reminds us to focus on the underlying etiologies of OLL and the multidisciplinary collaboration for oral lesions associated with systemic diseases., (© 2022. The Author(s).)

A 48-year-old woman with an abnormal shadow on chest X-ray was referred to our institution. Contrast-enhanced chest computed tomography( CT) showed a large mass, 4.4 cm in diameter, in the right upper mediastinum. Castleman's disease was suspected, and several vessels flowing into the tumor were identified. Since severe intraoperative bleeding was expected, preoperative embolization of the feeding vessels was performed, followed by thoracotomy and tumor extirpation. The amount of blood loss was 50 ml. The pathological diagnosis was Castleman's disease, hyaline vascular type.

We report a case of cardiac amyloid A (AA) amyloidosis due to unicentric Castleman disease (UCD) in a patient whose cardiac function was restored 15 years after surgical resection of the mesenteric lymph node lesion. A man in his 40s had recurrent palpitations and fainting spells. ECG revealed torsades de pointes Increased C-reactive protein, interleukin-6 and serum AA levels, and marked concentric thickening of the left ventricular (LV) wall with diastolic restrictive filling pattern were observed. Duodenal biopsy revealed AA amyloid deposits. He had a mesenteric tumour, comprising many plasma cells. He was diagnosed with plasma cell-type UCD associated with secondary AA amyloidosis. C-reactive protein, interleukin-6 and serum AA levels were normalised 2 months postresection. Episodes of lethal ventricular arrhythmias decreased. LV wall thickness was gradually reduced. Approximately 15 years postresection, the LV wall thickness nearly normalised and ventricular arrhythmias disappeared. Better outcomes are expected following surgical tumour resection., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

A female patient presented with fever of unknown origin, night sweats and weight loss. She had no pulmonary symptoms. Investigations revealed bilateral ground glass lung lesions which were subsequently followed-up with imaging. Two years later, a follow-up CT scan revealed an increase in the size of the lesions which exhibited a more solid appearance. A diagnostic biopsy was difficult to perform, and the patient underwent a left upper lobectomy for suspected primary lung malignancy. Histological examination showed lung involvement by Castleman's disease of plasma cell type which displayed a multifocal distribution. There was no evidence of nodal involvement. Following discussion at the multidisciplinary team meeting and correlation with radiology, a diagnosis of multicentric Castleman's disease of the lung was made. Here, we present an unusual case of multicentric Castleman's disease of the lung mimicking primary lung carcinoma. Our case highlights the importance of considering this entity in the differential diagnosis of multifocal lung lesions with a ground glass-like appearance to allow early diagnosis and management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Blood vessels in lymph nodes (LNs) are unique in comprising both capillaries and high endothelial venules (HEVs). Hyaline vascular type Castleman's disease accompanies robust angiogenesis, but it is unclear how the capillaries and HEVs respond. We retrospectively examined surgical specimens of hyaline vascular type unicentric Castleman's disease patients (n = 24) and control LNs (n = 9). We performed immunohistochemistry of CD 31 for capillaries and MECA-79 for HEVs and calculated their microvascular density. We measured CT enhancement as the ratio of Hounsfield Units (HUs) of the target lesion against muscle compared with microvascular density. The microvascular density of Castleman's disease specimen were (CD 31 + ) 169.7 ± 77.6, (MECA-79 + ) 203.5 ± 96.7, and the microvascular density of control LNs were (CD 31 + ) 80.7 ± 20.1, (MECA-79 + ) 67.4 ± 23.7, respectively. The microvascular density of both CD 31 + (P < 0.001) and MECA-79 + (P < 0.001) was higher in Castleman's disease. A positive correlation existed between CT HU ratio and microvascular density for both markers (CD 31: r = 0.517, P = 0.002; MECA-79: r = 0.521, P = 0.002). Intra-nodal angiogenesis of Castleman's disease involves robust proliferation of not only CD 31 + capillaries, but also MECA-79 + HVEs, which each correlated with degree of CT enhancement., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Castleman disease (CD) is a lymphoproliferative disease of unknown etiology, it can affect any lymph nodes of the body but rarely affects the popliteal fossa., Case Presentation: We present a 67-year-old woman with touching solitary painless mass in the left popliteal fossa for one week. Imaging showed multiple soft-tissue masses of different sizes in the left popliteal muscle space, the T1 weighted image showed hypointense to isointense, the fat-suppressed T2 weighted images showed subtle hypersignal intermingled with linear of hypointense,and displayed homogeneous contrast enhancement after administration of gadolinium. Complete surgical resection was performed. Pathologically demonstrated plasma cell type CD., Conclusion: We described a rare case plasma cell type of UCD located in the popliteal fossa which might help to enrich the clinical spectrum of this rare site and unique subtype of UCD. This case illustrates that CD should be considered in the differential diagnosis of every hypervascularity soft tissue tumor in any anatomic location, especially when they occur in the region of lymph node distribution., (© 2022. The Author(s).)

At a Glance: Bronchiolitis obliterans in paraneoplastic pemphigus associated with Castleman disease possesses the progressive nature even when it is treated with intensive medical therapy. Antibodies were at least in low titers before the Lung transplant and remain negative after the procedure. Explanted lungs showed coexistence of cellular destructive bronchiolitis and constrictive bronchiolitis., Background: Bronchiolitis obliterans (BO) in paraneoplastic pemphigus (PNP) associated with Castleman disease (CD) possesses the progressive nature of pulmonary disease even when it is treated with intensive medical therapy. Lung transplantation (LT) offers an acceptable form of treatment., Methods: We conducted a retrospective study of two cases of BO in PNP associated with CD who underwent LT between March 2017 and March 2020 at the China-Japan Friendship Hospital. We also included one case from the literature., Results: In this patient series, PNP was the primary clinical presentation in all patients, and it was accompanied by respiratory symptoms before/after CD excision. In spite of being treated with various combinations of immunosuppressive and anti-inflammatory agents, the patients had great or total improvement in mucosal erosions, whereas their pulmonary function test (PFT) deteriorated gradually or sharply. The duration times from disease onset to timing of LT were 1, 2 and 5 years. All antibodies were negative or were present at low titers before the LT procedure and remain negative after the procedure. The histopathological features of explanted lungs showed cellular and coexistent destructive bronchiolitis and constrictive bronchiolitis in two cases. Granulation with numerous foamy macrophages, scattered giant cells and cholesterol clefts were especially prominent in case one., Conclusion: BO in PNP associated with CD had poor clinical outcomes. LT was preferable choice in end-stage BO when PNP and CD were controlled., (© 2022 The Authors. The Clinical Respiratory Journal published by John Wiley & Sons Ltd.)

Bronchiolitis obliterans (BO) is a severe complication of Castleman disease (CD), a rare lymphoproliferative disease with unclear pathogenesis. Currently, there are no reports on the safety or outcomes of bilateral lung transplantation in patients with BO due to CD. This study aimed to characterize the clinical manifestations and features of BO and CD. We retrospectively analyzed the medical records of six consecutive patients with BO and CD who underwent bilateral lung transplantation between December 2012 and December 2020. The average age of patients at lung transplantation was 33 ± 15 years, and the age range of patients at diagnosis of CD was about 9-56 years. The body mass index was 15.2 ± 1.9 kg/m 2 . The average time from diagnosis to lung transplantation was 4.1 ± 2.7 years. All the patients had unicentric CD (UCD); five had concomitant paraneoplastic pemphigus, and four received extracorporeal membrane oxygenation during surgery. The average hospital stay was 51 ± 53 days. Infection was the most common postoperative complication. CD did not recur in any of the patients. Thus, bilateral lung transplantation is a viable and safe treatment for selected patients with CD and BO, which can improve the quality of life and prolong survival., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The patient was a 29-year-old female with a chief complaint of transient right-sided abdominal pain. A CT scan revealed homogenously contrasted tumor of 4 cm in diameter with smooth margins and clear borders in the lower part of the pancreatic head. The tumor was contrast-enhanced on MRI and stained on abdominal angiography using the proximal branch of the right colonic artery as a feeding vessel. PET scan showed moderate accumulation. Malignancy could not be ruled out, and tumor resection including the ascending colon was performed. The pathological diagnosis was hyaline vascular-type Castleman's disease in the mesentery of the colon.

Introduction: The intrapulmonary involvement of Castleman disease is extremely rare with very few cases reported in the literature., Case Report: We present a case of a 16-year-old male who underwent pulmonary resection for Castleman disease. The tumor was located at the right hilum and measured 10 cm in diameter. The patient underwent an intrapericardial pneumonectomy with a mediastinal lymph node dissection because the tumor showed infiltration into the lung parenchyma, the major pulmonary vessels and the main bronchus., Conclusion: We emphasize that some cases with intrapulmonary Castleman disease may require a pneumonectomy if the tumor is centrally located.

Unresectable, symptomatic unicentric Castleman disease (UCD) can represent a formidable therapeutic challenge. UCD masses are often highly vascularized offering the opportunity for therapeutic embolization. Herein, we report on 6 patients in which therapeutic embolization was combined with other medical interventions including surgery (n = 3), rituximab (n = 6), cryoablation (n = 2), and chemotherapy (n = 3). Five patients had significant tumor volume reductions (median: 83.2%; range: 76.7-100). All five responding patients had resolution of symptomatology. There were no serious complications in the patients who received embolization and proceeded to surgery. In conclusion, effective disease and symptom control can be obtained in patients with symptomatic, unresectable UCD by combining different therapeutic interventions., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The paper reports the case of a 13-year-old female adolescent presenting with persistent fever. She had no other significant symptoms or signs. Laboratory examinations showed mild anemia and elevated C-reactive protein (CRP) and erythrosedimentation rate (ERS). The abdominal ultrasonography revealed para-aortic lymphadenopathy that was confirmed by magnetic resonance imaging (MRI) and positron emission tomography-computed tomography (PET/CT) that showed no other locations. The patient underwent laparoscopic excision but complete removal was not possible due to the position of the mass. The histological exam documented unicentric Castleman's disease. After surgery a clinical improvement was assisted but with persistence of very high CPR, ERS and serum amyloid. According to guidelines, she was treated with tocilizumab achieving complete remission of indices of inflammation. In the case with symptomatic unresectable unicentric Castleman's disease treatment with anti-IL-6 agents should be considered.

Castleman disease (CD) is a rare lymphoproliferative disorder of unknown etiology. Its most common location is the mediastinum, but many other sites have been reported. We report a case of primary CD of the ovary, a rare localization with only 2 cases including the present case described in the world literature to date. A 58-yr-old woman who initially presented with abdominal pain underwent computed tomography scan which showed bilateral well-circumscribed solid adnexal masses. Because an ovarian bilateral tumor was suspected the patient was treated with a hysterectomy and bilateral salpingo-oophorectomy and the histopathologic examination confirmed the diagnosis of CD hyaline-vascular type of the right ovary associated with a contralateral fibroma. Three years after surgery the patient is alive and well and shows no signs of recurrent disease. The occurrence of this rare presentation of CD is the subject of this report. The problems of differential diagnosis with the most frequent lesions of the female pelvis are also discussed., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 by the International Society of Gynecological Pathologists.)

Castleman's disease (CD) is a rare lymphoproliferative disorder. This case report, to the best of our knowledge, is the first report of CD simulating a pancreatic neuroendocrine tumour . The patient was a 58-year-old woman who initially presented with bilateral iritis and underwent investigation for possible systemic rheumatological disease. CT of the chest demonstrated an incidental finding of a well-demarcated retropancreatic mass. As the mass was found to enhance on DOTATATE (tetraazacyclododecanetetraacetic acid-DPhe1-Tyr3-octreotate) positron emission tomography, a diagnosis of pancreatic neuroendocrine tumour was made. The patient underwent an open distal pancreatectomy and splenectomy. Histopathological examination revealed the unexpected diagnosis of hyaline vascular CD of a lymph node posterior to the pancreas. After 2 years of follow-up, there is no evidence of disease recurrence., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)