[Unusual gluteal localization of unicentric Castleman's disease: A case report and review of the literature]. / Localisation intramusculaire d'une maladie de Castleman unicentrique : cas clinique et revue de la littérature.

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.

[Challenging Case of Primary Renal Castleman's Disease Accompanied with Bacterial Pyelonephritis].

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.

[Diagnosis and surgical treatment of a patient with Castleman disease]. / Diagnostika i khirurgicheskoe lechenie patsienta s bolezn'yu Kastlemana: klinicheskoe nablyudenie.

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.

Profile of vascular markers and CT enhancement of hyaline vascular type Castleman's disease.

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.

Bilateral lung transplantation for Castleman disease with end-stage bronchiolitis obliterans.

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/m2 . 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.

Unicentric castleman disease located in the left popliteal fossa: a case report.

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.

Oral lichenoid lesion simultaneously associated with Castleman's disease and papillary thyroid carcinoma: a rare case report.

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.

Concurrence of Marjolin's Ulcer in the Lower Limb in a Patient with Idiopathic Multicentric Castleman Disease: A Case Report.

Idiopathic multicentric Castleman disease (iMCD) is characterized by the benign proliferation of lymphoid cells in multiple regions. However, the co-occurrence of epithelial malignancy and idiopathic multicentric Castleman disease (iMCD) is rarely reported. Herein, we present a case of iMCD mimicking lymph nodal metastasis of Marjolin's ulcer in the lower extremity. A 53-year-old male presented with an unhealed chronic ulcer on the left lower leg and foot accompanied by an enlarged mass in the left inguinal region. Intralesional biopsy was performed, and pathological examination showed squamous cell carcinoma (SCC). Imaged studies revealed left calcaneus bone invasion, and lymph nodal metastasis was suspected by the cancer TNM staging of T4N2M0 pre-operatively. The patient received below-knee amputation and lymph node dissection; intraoperative histological examination showed no lymphatic nodal malignancy and diagnosed the patient as having iMCD with lymphadenopathy. The patient recovered uneventfully and was referred to a hematologist for further treatment.

[Surgical Treatment to Castleman's Disease after Embolization of Feeding Vessels].

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.

Combinatorial treatment for unresectable unicentric Castleman disease.

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.

A Rare Case of Castleman Disease Presenting as an Ovarian Tumor.

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.

Castleman Disease: An Unusual Cause of an Isolated Neck Mass.

ABSTRACT: A 15-year old female patient was complaining from swelling in the left side of the neck since 3 months. Physical examination showed palpable and mobile left supraclavicular neck mass in the carotid triangle, uvula deviation to the left side and normal vocal cords position and function. Neck imagings showed well-defined, well-encapsulated left neck mass in the left carotid sheath pushing the internal jugular vein anteriorly and the common carotid artery posteriorly. The mass was extending from the level of C4 superiorly and the supraclavicular region inferiorly. Patient was operated with left anterior approach and circumferential dissection and enucleation was performed. Post-operative period was uneventful and devoid of any complications or deficits. Histopathological examination revealed Castleman disease.

Intrapericardial pneumonectomy for unicentric hilar castleman disease.

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.

[A Case of Pelvic Unicentric Castleman Disease Treated by Preoperative Transcatheter Arterial Embolization and Tumor Complete Resection with Combined Lower Abdominal and Posterior Approach].

A 22-year-old woman was referred to our hospital for further examination of an incidentally discovered hypervascular pelvic tumor with a maximum diameter of 10 cm. Although Castleman disease was suspected based on the imaging findings and pathologic findings of the needle biopsy, a definitive diagnosis was not made. Preoperative transcatheter arterial embolization was performed to decrease intraoperative bleeding, and tumor resection was performed on the following day. As for posterior approach prior to anterior approach, the patient was placed in a prone position, and the dorsal aspect of tumor was approached through the dissection of gluteal muscles. Then, dilated branches of the internal iliac vein was found on the tumor capsule, which were safely ligated under direct vision with favorable visual field. Then, the patient was placed in a supine position, the tumor was completely resected by anterior approach without transfusion. Histopathological diagnosis was Castleman disease hyaline vascular type. The patient was discharged without complication and has been free from recurrence for 6 months after surgery.

[A Case of Hyaline Vascular-Type Castleman's Disease].

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.

18F-FDG PET/CT in lung B lymphoid hyperplasia-type Castleman disease.

We present a 62-year-old woman who, after a catarrhal episode, underwent a chest xray and a chest CT, identifying a pleural-based mass in the left lower lobe. This lesion presented a mixed pattern with solid and peripheral ground-glass attenuation, and an air-bronchogram sign. Despite de suspicion of bronchopneumonia, a lung neoplasm like a predominantly lepidic adenocarcinoma could not be ruled. A 18 F-FDG PET/CT showed a focal radiotracer uptake with SUVmax of 5.34 in the pulmonary lesion, supporting the neoplastic etiology. Consequently, a lower lobectomy was performed and histological examination concluded that the final diagnosis was a B lymphoid hyperplasia-type Castleman disease. Castleman's disease is an uncommon disorder that can be easily misdiagnosed as lymphoma, neoplasm or infection. Unicentric Castleman's disease (UCD) usually presents a hyaline-vascular histological subtype and is usually asymptomatic. Extranodal involvement is very rare. Only a few cases of solid organ involvement such us spleen or parotid gland have been described. UCD originating in the lung is extremely rare and should be considered in the differential diagnosis of a primary pulmonary malignant tumor.

Retroperitoneal Castleman Disease Invading Iliac Vein and Inferior Vena Cava Treated by Tumorectomy with Vascular Repair: A Case Report.

Unicentric Castleman disease, a rare lymphoproliferative disorder, is always known as a solitary, well-defined lymph node enlargement. We reported an extraordinary case of retroperitoneal Castleman disease, which invades wall of right iliac vein and inferior vena cava, treated successfully by tumorectomy with vascular repair.

Video-assisted thoracoscopic surgery is a safe and effective method to treat intrathoracic unicentric Castleman's disease.

BACKGROUND: Castleman's disease (CD) is a rare non-clonal lymphadenopathy. Application of video-assisted thoracoscopic surgery (VATs) in intrathoracic unicentric Castleman's disease (UCD) is rarely reported. This study is aimed to clarify the role of VATs for diagnosis and treatment in intrathoracic UCD. METHODS: The authors reviewed and identified patients who had received a histologic diagnosis of CD through VATs at our hospital from January2010 to June 2018. Clinical and radiologic variables, histopathology, type of approach, complications, and long-term effect were analyzed to evaluate the safety and efficacy of VATs. RESULTS: A total of 10 patients were included in this study, with 8 hyaline vascular type and 2 plasma cell type. The mean maximum diameter of the lesions was 4.66 cm. Nine cases underwent complete surgical excision by VATs, and 1 case was converted to thoracotomy. All patients had no postoperative complications. With a median follow-up of 5 years (range: 1-9 years), no tumor recurrence was found in 9 patients receiving complete tumor resection, and 1 patient with incomplete tumor resection remained symptom free without clinical or radiographic progression. CONCLUSIONS: VATs is an alternative, minimally invasive technique for the diagnosis and treatment in patients with intrathoracic UCD.

Castleman disease of the neck: A case report.

Castleman disease is a rare lymphoproliferative disorder with two distinctive presentations, namely Unicentric disease and Multicentric disease. Unicentric disease occurs as a solitary enlarged mass in the mediastinum and is rarely found in the head and the neck, whereas, Multicentric disease appears as a systemic disease with peripheral lymphadenopathy. Here we discuss an unusual case with characteristic clinical, radiologic and histologic findings of a 22-year-old male with Unicentric disease demonstrating a slow growing neck mass often times misdiagnosed as a lymphoma.

A Case of Castleman Disease With Recurrence in the Neck: Case Report and Literature Review.

Castleman disease (CD) is a rare lymphoproliferative disorder with an uncertain etiology. It is necessary to review known cases because the possibility of CD is easy to overlook in the clinical diagnosis.