Mesenteric Castleman’s Disease: A Rare Cause of Mesenteric Masses: A Case Report and Literature Review

Castleman’s disease, also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia, is a rare benign B-cell lymphoproliferative disorder of unknown etiology. We report a case of a 23-year old woman with mesenteric Castleman’s disease of plasma cell variant presented as a palpable mass in the left iliac fossa.

Case Report: Rare case of classical Hodgkin’s lymphoma, mixed cellularity in a male boy aged 12 years.

Castleman disease was initially explained in 1956 as a syndrome that is lymphoproliferative, also recognized as angiofollicular lymph node hyperplasia. It is a rare disease and may proceed to malignant lymphoma that is difficult to diagnose while the treatment is complex. Hodgkin lymphoma (HL) is a malignancy that is related to lymphoid and can be characterized as Reed-Sternberg cells in the lymph nodes. Prognosis as well as treatment is complex and depends upon the Ann Arbor staging as well as age of the affected patient. HL is divided into 2 distinct subtypes, Nodular Lymphocyte Predominant HL (LPHL), and classical HL (cHL). The present case was a 12year old male boy who presented with fever, cough and swelling on the body at multiple sites. Diagnosis of cervical lymph node biopsy was labeled as classical Hodgkin’s Lymphoma, mixed cellularity. Early diagnosis, better understanding of the pathophysiology as well as the biology of HL can assure much better prognosis.

Idiopathic multicentric Castleman’s disease, infrequent cause of Lupus-like. Case Report.

Castleman’s disease (CD) or angiofollicular lymph node hyperplasia includes a heterogeneous mix of reactive lymphoproliferative processes with well-defined histological features. However, they differ in their localization patterns, clinical expression and etiopathogenesis. There are 4 types, one of them is the multicentric CD that is not associated with any viruses and has recently been called idiopathic MCD (iMCD). iMCD is a lymphoproliferative disorder with specific histopathological characteristics, more than one region of affected lymph nodes and absence of infection associated to human herpesvirus 8 and human immunodeficiency virus (HIV). iMCD covers multiple differential diagnoses and might simulate autoimmune diseases such as systemic lupus erythematosus. The aim of this article is to report the case of a patient with Castleman’s disease and lupus-like presentation. We present the case of a 38-year-old man without morbid history, who presented lumbago, fever, diaphoresis and asthenia with two months of evolution, associated to bilateral cervical adenopathies. General examinations result negative, antinuclear antibodies at a dilution of 1/640 were positive, and extractable nuclear antigens were positive suggesting moderate Systemic Lupus Erythematosus (SLE) plus secondary Sjögren’s. Methylprednisolone and Hydroxycloroquine boli were thus initiated. The patient evolved with anasarca, severe anemia, acidosis, polyserositis and multiple mediastinal adenopathies. Immunoglobulin and cyclophosphamide were thus initiated. He later presented fever, thrombocytopenia and nephrotic syndrome. Biopsy of cervical lymph node reported lymphadenitis with polyclonal plasmacytosis and concentric lymphoid hyperplasia, in agreement with iMCD. Treatment with Rituximab was initiated, which led to the favorable evolution of the patient. iMCD is a systemic inflammatory disease, its presentation corresponds to a constitutional syndrome resulting in a wide differential diagnosis. Every time suspicious adenopathies appear, they must be biopsied since this might lead to a definitive diagnosis.

Variant of plasmablastic microlymphoma in Castleman disease: a case report and review of the literature

Castleman disease (CD) is a rare lymphoproliferative disorder also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia. CD can be unicentric CD (UCD) or multicentric CD (MCD). MCD affects more than one group of lymph nodes and/or lymphoid tissues and it is frequently associated with HIV and human herpes virus 8 (HHV-8) infections and, in contrast with UCD, it often results in systemic symptoms, such as fever, fatigue, anemia, inflammatory syndrome. HHV- 8-associated MCD recognizes HHV-8 as an etiopathogenetic agent and occurs generally in HIV-positive subjects. Our report describes an HHV-8 positive Castleman disease with plasmablastic microlymphoma occurring in a 51-year-old HIV seronegative woman, with a previous history of HBV infection and Kaposi’s sarcoma, who presented elevated procalcitonin levels during the acute phase of CD.

Multicentric Castleman's Disease in a 21 Years Old Female : A Rare But Important Condition

Castleman’s disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which plays a major role in determining therapy. The rare multicentric type is a lymphoproliferative disorder of unknown etiology and is characterized by various clinical manifestations and multiple organ involvement. This disease runs a more aggressive course and a poor prognosis. Optimal therapies have not been well established till now. We here report a case of rare Multicentric Castleman's Disease (MCD) in a 21yrs old female. She presented with slowly enlarging lymph nodes in cervical and inguinal regions which lead to a histological diagnosis of this rare condition. Its clinical features, types, relevant investigations and current treatment modalities are discussed. Though rare, early suspicion of this condition may relieve the suffering, avoid unnecessary investigations, give opportunity to choose treatment options and can save lives.Chatt Maa Shi Hosp Med Coll J; Vol.16 (1); Jan 2017; Page 57-59

A Case Report of Unicentric Castleman Disease

Castleman disease (CD) or angiofollicular lymph node hyperplasia is an uncommon benign lymphoproliferative disorder. Sometimes it is related to the chronic human herpes virus 8 (HHV-8) infection and the human immunodeficiency virus (HIV). Two clinical entities have been described: a unicentric presentation with the disease confined to a single anatomic lymph node and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course. Also, three histopathological subtypes have been described: hyalinevascular, plasma cell, and a mixed variant.We report the case of a 22-year-old young man who was diagnosed as unicentric Castleman Disease. We found on routine physical examination a mass localized in the left abdomen without clinically important constitutional symptoms for 3 years. Abdominal ultrasound and computed tomography scans revealed minimally enhancing soft tissue density lesion & it was seen in the left para aortic region measuring about 71mm x 42 mm in size. The patient subse-quently underwent an exploratory laparotomy. After opening of the abdomen, a lump measuring about 8 cm x 5 cm was found in the mesentery. The whole lump was dissected out and the gap in the mesentery was closed. No other lymphadenopathy was found. The specimen was sent for histopathology which revealed Castleman disease, plasma cell type. The patient completely recovered after surgery. Castleman disease is commonly misdiagnosed as malignant lymphoma, lymphadenitis or ectopic thymoma. So far, its diagnosis is mainly achieved via histopathological examination of surgically obtained tissue. Local or unicentric CD has a good prognosis if surgically excised properly.J Dhaka Medical College, Vol. 23, No.2, October, 2014, Page 256-258

Castleman’s Disease Presenting as a Parotid Mass in the Pediatric Population: A Report of 2 Cases

Introduction. Angiofollicular lymph node hyperplasia (Castleman’s disease) is a nonmalignant lymphoproliferative disorder that generally involves the lymph nodes of young adults, most commonly in the mediastinum. Rarely, Castleman’s disease may present in the parotid gland. The disease can be further classified into unicentric or multicentric forms, with considerable differences in presentation, treatment, and prognosis.Case(s). We present cases of two pediatric patients, aged 7 and 11, who both presented with a slow-growing, painless parotid mass. In each case, the mass was excised via a superficial parotidectomy and the diagnosis made postoperatively upon further pathologic examination. At 6 months of follow-up, both had fully intact facial nerve function and no evidence of recurrence.Discussion. Castleman’s disease presents a diagnostic challenge in the head and neck region, as radiographic characteristics and fine needle aspiration results are often inconclusive. Definitive diagnosis requires surgical excision for pathologic examination. The unicentric form generally presents as a painless mass and can be successfully treated with complete excision. The multicentric form is associated with constitutional symptoms and its treatment remains controversial.Conclusion. Although rare, clinicians should be aware of both forms of Castleman’s disease when creating a differential diagnosis for parotid masses.

Castleman's Disease: A Case Report of the Unicentric Type

Castleman's disease, or angiofollicular lymph node hyperplasia, is a relatively rare disorder characterized by the benign proliferation of lymphoid tissue related to the chronic human herpes virus 8 (HHV-8) infection and the human immunodeficiency virus (HIV). Two clinical entities have been described: a unicentric presentation with the disease confined to a single anatomic lymph node and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course. Also, three histopathological subtypes have been described: hyaline-vascular, plasma cell, and a mixed variant. Preoperative diagnosis of hyaline-vascular Castleman's disease is difficult, and the definitive result is based on postoperative pathological findings. The gold standard therapy is the complete surgical excision.