Benign Lymphoproliferative Disorder Research Articles

CT Findings of Thoracic Manifestations of Primary Sjögren Syndrome: Radiologic-Pathologic Correlation

Primary Sjögren syndrome is an immune-mediated exocrinopathy characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands. Various systemic extraglandular disorders are associated with primary Sjögren syndrome, and the thorax is commonly affected. The pulmonary manifestations of primary Sjögren syndrome may be categorized as airway abnormalities, interstitial pneumonias, and lymphoproliferative disorders; in each category, bronchiectasis or centrilobular nodules, nonspecific interstitial pneumonia, and lymphoid interstitial pneumonia are common. These manifestations do not usually occur in isolation; they are concomitantly seen with other types of lesions. Mucosa-associated lymphoid tissue (MALT) lymphoma and amyloidosis are key components of lymphoproliferative disorders, and MALT lymphoma should always be considered because its morphologic characteristics are similar to those of benign lymphoproliferative disorders. Amyloidosis is rare but important because it carries a risk for underlying MALT lymphoma or plasmacytoma, and it may lead to hemoptysis during biopsy. In addition, thin-walled air cysts are characteristic of primary Sjögren syndrome, irrespective of the main pulmonary manifestations. Lymphadenopathy and multilocular thymic cysts may be seen in the mediastinum. During the follow-up period, there is a risk for acute exacerbation of interstitial pneumonia and development of malignant lymphoma. Often, primary Sjögren syndrome is subclinical, but there are various underlying risks. Thus, imaging findings are important. In addition to the various types of interstitial pneumonia and airway abnormalities, air cysts and mediastinal manifestations may help diagnose primary Sjögren syndrome.

Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children

Although more common with advanced age, the entire spectrum of benign and malignant cutaneous lymphoproliferative disorders can present in the pediatric population. Nevertheless, these conditions are often mistaken for other more common, benign dermatoses, resulting in delayed diagnosis. Standardized management of these conditions is also lacking. The purpose of this review is to provide an update on the most common benign and malignant cutaneous lymphoproliferative disorders in children: pityriasis lichenoides and mycosis fungoides. The presentation, evaluation and management of each of these conditions are discussed.

Persistent Polyclonal B-cell Lymphocytosis With Splenomegaly

Persistent polyclonal B-cell lymphocytosis is a rare, benign lymphoproliferative disorder characterized by a stable, polyclonal CD19-positive CD5-negative lymphocytosis, the presence of binucleated lymphocytes in peripheral blood, and a polyclonal increase in serum immunoglobulin-M that may occasionally be accompanied by splenomegaly. Histopathologic diagnosis of these splenectomy specimens is difficult because of the massive spleen infiltration and the rarity of the descriptions of this condition. We describe the histopathologic findings from 2 splenectomy specimens. These included a partially preserved architecture with infiltration of the red pulp by small lymphocytes and partial replacement of the white pulp. Suggestions for identifying the disorder are made.

Cutaneous pseudolymphoma occurring after traumatic implantation of a foreign red pigment

Cutaneous pseudolymphoma is an uncommon, benign lymphoproliferative disorder of the skin. Although this condition is most commonly idiopathic, its occurrence has been associated with cosmetic tattoos. We report a unique case of cutaneous pseudolymphoma that occured after accidental, traumatic inoculation of a red pigment in a healthy 33-year-old woman.

Cervical unicentric Castleman's disease in children

Unicentric Castleman's disease (CD) is a rare benign lymphoproliferative disorder. A cervical location has rarely been reported in the pediatric literature. A 13-year-old boy presented with a mass in the right neck region of 3 months' evolution. Clinical examination revealed a smooth mobile 5 cm node deep in the sternocleidomastoid muscle. The diagnosis of hyaline vascular type CD was confirmed by complete surgical excision and histopathologic examination. Postoperative course was simple, without recurrence over 1 year's follow-up. Cervically located CD often shows few symptoms, and is rarely suggested by a cervical mass found in children. Definitive diagnosis is histopathological. The hyaline vascular type of unicentric CD has a good prognosis after complete resection of the lesion.

A unique presentation of Epstein-Barr virus-associated Castleman's disease

Castleman’s disease (CD) is a rare, benign lymphoproliferative disorder. The association of human herpes virus 8 (HHV8) and human immunodeficiency virus infections with CD is well established however the role of Epstein-Barr Virus in CD is less well understood. We present a unique case of Castleman’s disease in a patient with concomitant EBV infection, which mimicked the clinical presentation of Nasopharyngeal Carcinoma (NPC) versus lymphoma. After a delayed diagnosis, the patient underwent a left superficial parotidectomy and neck dissection and has had no recurrence of disease.

A Definitive Diagnosis of Mucosa-associated Lymphoid Tissue Lymphoma Made at a Second Biopsy

A case of mucosa-associated lymphoid tissue (MALT) lymphoma, the most frequent of the various conjunctival lymphoproliferative disorders, in which the initial biopsy was inconclusive but the second biopsy provided a definitive diagnosis, is reported. A 26-year-old woman with a 3-month history of bilateral conjunctival swelling was referred by a local physician for suspected MALT lymphoma. A salmon-pink elastic swelling was found to involve both eyes and to extend from the lower palpebral conjunctiva to the bulbar conjunctiva. Tonsillar swelling was also found, and ophthalmologic (left eye) and otolaryngologic biopsies were therefore performed simultaneously under general anesthesia. The otolaryngologic diagnosis was chronic tonsillitis. Light microscope examination of the conjunctival tissue showed proliferation of lymphocytes and small aggregates of small to medium-sized atypical lymphocytes. On immunohistochemical studies, atypical lymphocytes were positive for CD20 and CD79a, but differentiation to plasmacytes was not prominent, and neither Dutcher bodies nor evidence of immunoglobulin light chain restriction was found. The results were not incompatible with MALT lymphoma but were not definitive. A second biopsy of the right eye was therefore performed 3 months later. Staining with hematoxylin and eosin showed proliferation of small lymphocytes and monocytoid B cells and differentiation to plasmacytes. The hyperplastic cells were positive for CD19, CD79a, and CD20, and their cytoplasm were positive for Bcl-2 and slightly positive for Bcl-6. Cells positive for CD38 were noted where differentiation to plasmacytes and immunoglobulin light chain κ restriction was evident on immunohistochemical studies and in situ hybridization. The Ki-67-positivity rate was approximately 5%. The results of paraffin-embedded tissue section fluorescence in situ hybridization were negative for MALT-1 (18q21). A diagnosis of MALT lymphoma was made, and treatment with rituximab was started. Few findings lead directly to a definitive diagnosis of MALT lymphoma, and its differential diagnosis from benign lymphoproliferative disorders is difficult. In the present case definitive diagnosis was possible only after a second biopsy. This case suggests repeated biopsy may be necessary when a single biopsy is not definitive.

Successful Treatment of Mediastinal Unicentric Castleman’s Disease Using Video-Assisted Thoracoscopic Surgery with Preoperative Embolization

Unicentric Castleman's disease is a rare, benign lymphoproliferative disorder that is curable with surgical resection. However, significant bleeding often occurs during surgery because of tumor hypervascularity. We herein present a case of hyaline-vascular-type mediastinal unicentric Castleman's disease, successfully resected using video-assisted thoracoscopic surgery with preoperative embolization. In the present case, tumor hypervascularity and feeding vessels were revealed by computed tomography (CT), which led us to perform preoperative angiography and embolization to the tumor feeding arteries to reduce intraoperative bleeding. Castleman's disease should be considered in the differential diagnosis of hypervascular mediastinal tumors. Tumor vascularity should be assessed prior to surgery, and preoperative embolization should be considered.

Hepatitis C virus-related mixed cryoglobulinemia: Is genetics to blame?

Mixed cryoglobulinemia (MC) is the extrahepatic manifestation most strictly correlated with hepatitis C virus (HCV) infection; it is a benign autoimmune and lymphoproliferative disorder that evolves to lymphoma in 5%-10% of cases. MC is reputed to be a multistep and multifactorial process whose pathogenicity is still poorly understood. It is still unknown why only some chronically infected HCV patients develop MC and only some of these exhibit systemic symptoms (MC syndrome). Several studies have investigated the pathogenetic basis of MC and the most recent ones suggest that the virus is able to trigger such a disorder only in the presence of genetic factors that are still unknown. Here, we try to clarify the complex relationship between HCV-related MC and the host's genetic background. The data that we report are heterogeneous and sometimes even conflicting. Therefore, large, multicenter studies are clearly needed. The identification of a characteristic genetic signature of cryoglobulinemic patients would be an important step toward a personalized approach in their clinical care. The new wide-ranging genomics technologies will hopefully help to resolve these complex issues.

Multicentric Castleman’s Disease, Associated with Idiopathic Thrombocytopenic Purpura

The most common cause of a neck mass in young adults is hyperplastic lymphadenopathy consequent to infection and inflammation. Castleman's disease (CD), an unusual benign lymphoproliferative disorder, infrequently causes neck masses. It occurs in unicentric (UCD) and multicentric (MCD) forms and is associated with human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and Kaposi's sarcoma. We present the third known association between MCD and previous immune thrombocytopenia in the absence of HIV and HHV-8 infection and review its association with other autoimmune disorders and attendant implications for pathogenesis. Finally, we summarize the current approach to therapy.

Castleman Disease: An Unexpected Cause of a Solitary Pleural Mass

Castleman disease (CD) is a rare benign lymphoproliferative disorder, the etiology of which is unclear. Clinically it may manifest as localized disease (unicentric) or disseminated disease (multicentric). CD occurs in the thorax in 70% of cases, abdomen and pelvis in 15%, and in the neck in 10–15% of cases. We present a case of a pleural mass located posteriorly in a paraspinal location, which was discovered incidentally in a 50-year-old man and was subsequently resected followed by an unexpected diagnosis of Castleman disease on histological examination. In this report, we review the clinical and histological findings in a rare presentation of Castleman disease and discuss the findings in this case as part of an overall review of the typical radiological findings seen in Castleman disease.

Castleman’s Disease in the Axilla: A Case Report

INTRODUCTION: Castleman s disease is a rare benign lymphoproliferative disorder of uncertain origin which most commonly involves the mediastinum but rarely affects the axilla. REPORTING: We report a case of a 40-year-old woman, without comorbidities and no family history of cancer, that sought medical assistance for having presented a right axillary tumor during two years. Physical examination showed a mass in the right axilla approximately 4. 0 x 3. 0 cm, mobile and painless palpation. The ultrasonography of the breast demonstrated a well-defined, heterogeneous, hypoechoic, ovoid mass at the right axilla whose measurement was 5, 2 x 3, 6 x 2, 5 cm, and peripheral hypervascularity was observed at a color Doppler sonography. We conducted a percutaneous needle biopsy which showed reactive follicular hyperplasia. The patient underwent resection of the lesion and the microscopic sections of the enlarged lymph node showed numerous small follicle like structures with radially penetrating capillaries, some with hyalinization in the central portion. The final pathologic diagnosis was angiofollicular lymphoid hyperplasia, hyaline vascular type (Castleman disease). The patient is asymptomatic seven months after surgery with no change in the new ultrasound exams. DISCUSSION: Castleman s disease is an uncommon lymphoproliferative disorder. There are three histological types, hyaline vascular (the most common), plasma cell variant and mixed form. The hyaline vascular variant is typically characterized by a benign clinical course without constitutional symptoms (localized disease). It is usually managed with surgery and/or radiotherapy. They usually consider the patient free from disease after surgical resection of localized Castleman s disease, but there is a risk of developing non-Hodgkin lymphoma in the long-term. The purpose of this report is to present this rare case and to stress that Castleman s disease should be included in the differential diagnosis of axillaries’ tumors, mainly the axillary lymph node metastasis and the primary occult breast carcinoma.

CASTLEMAN’S DISEASE – CASE REPORT

We report a case of a 16-year-old girl with hyaline-vascular type of abdominal Castleman’s disease. The patient presented with a history of palpable mass below left ribcage for 6 months. On admission the girl was in good general condition. Physical examination showed a palpable mass in the left hemiabdomen but was otherwise unremarkable. Laboratory findings were within normal limits except hypergammaglobulinemia with elevated immunoglobulin A level. Computed tomography (CT) revealed well-demarcated homogeneous mass, extending from left diaphragm to the lower pole of left kidney, as well as from peritoneum to the pancreas. CT-guided biopsy was performed. A biopsy material was considered insufficient for definitive diagnosis. A complete surgical resection was done, and a diagnosis of hyaline-vascular type of Castleman’s disease was established. Eight years after surgery the patient is in complete remission. Conclusion – Castleman’s disease is a rare benign lymphoproliferative disorder, whose early recognition can be difficult due to nonspecific symptoms and radiologic findings. The right pathohistological diagnosis and appropriate staging of the disease are crucial in the treatment planning.

Castleman disease mimicked pancreatic carcinoma: report of two cases

Castleman disease (CD) is an uncommon benign lymphoproliferative disorder, which usually presents as solitary or multiple masses in the mediastinum. Peripancreatic CD was rarely reported. Herein, we report two cases of unicentric peripancreatic CD from our center. A 43-year-old man and a 58-year-old woman were detected to have a pancreatic mass in the routine medical examinations. Both of them were asymptomatic. The computed tomography and ultrasonographic examination revealed a mild enhancing solitary mass at the pancreatic head/neck. No definite preoperative diagnosis was established and Whipple operations were originally planned. The intraoperative frozen section diagnosis of both patients revealed lymphoproliferation. Then the local excisions of mass were performed. Histological examination revealed features of CD of hyaline-vascular type. No recurrence was found during the follow-up period. CD should be included in the differential diagnosis of pancreatic tumors. Local excision is a suitable surgical choice.

Preoperative Embolization for the Treatment of Cervical Castleman Disease

Castleman disease (CD) is a rare benign lymphoproliferative disorder commonly described as a hypervascular mass that causes progressive lymph node enlargement. Head and neck involvement occurs only in 15% to 20% of cases. The recommended treatment in solitary CD is radical resection. Few reports have described the use of angiographic study and preoperative embolization to minimize the intraoperative risk of hemorrhage. We report a clinical case of a solitary large painless, slow-growing mass located in the neck of a 34-year-old woman. Contrast-enhanced computed tomographic and magnetic resonance imaging scan demonstrated a well-defined mass with internal calcifications and peripheral vessels located in the posterior cervical space, extending inferiorly to the supraclavicular space, which moderately enhanced after contrast administration. In the preoperative arteriography, a hypervascularized mass was identified, which mainly received an arterial supply from thyrocervical trunk. Successful embolization with polyvinyl alcohol microparticles was performed, resulting in a significant reduction of intraoperative bleeding, allowing a subsequently safe removal of the tumor. Histopathologic examination corresponded to hyaline vascular-type CD.

Efficacy of tocilizumab, a humanized neutralizing antibody against interleukin-6 receptor, in progressive renal injury associated with Castleman's disease

Castleman's disease is a benign lymphoproliferative disorder in which interleukin-6 (IL-6), a pleiotropic proinflammatory cytokine, is thought to play a pathogenetic role. Presented is the case of a 72-year-old man with Castleman's disease who exhibited progressive renal dysfunction with proteinuria. Renal biopsy revealed mesangial hypercellularity and matrix expansion in most glomeruli and peritubular inflammatory cell infiltration. Immunofluorescence studies showed intense deposition of IgG in a granular pattern along the glomerular basement membrane. Histological features were compatible with membranoproliferative glomerulonephritis accompanied by interstitial inflammatory cell infiltration. Immunohistological analysis showed that IL-6 was abundantly expressed by tubular cells and interstitial macrophages, suggesting involvement of IL-6 in the renal injury. As a result of administration of tocilizumab, a humanized anti-IL-6 receptor antibody, the patient experienced clinical and biochemical improvement of Castleman's disease, including marked reduction of proteinuria and stabilization of renal function. These findings suggest the efficacy of tocilizumab against Castleman's disease and its renal complications.

Immunophenotyping of Chronic B-Cell Neoplasms: Flow Cytometry versus Immunohistochemistry

Morphological differentiation between benign and malignant lymphoproliferative disorders (LPDs) can be challenging. Immunophenotyping (IPT) by either technique, flow cytometry or immunohistochemistry (IHC), is an important step in solving such difficulty. Thirty-five newly diagnosed patients with chronic B-cell neoplasms (11 chronic lymphocytic leukemia, 22 non Hodgkin lymphoma and 2 hairy cell leukemia) were included in this study with age range from 20 to 70 years. Monoclonal antibodies surface expression using lymphoproliferative disorders panel (CD45, CD19, CD5, CD10, CD11c, CD20, CD22, CD23, CD38, CD79b, FMC7, CD103, CD25, kappa and lambda light chains) by flow cytometry was done on bone marrow samples. CD20, CD5, CD23, Bcl-2, Bcl-6, kappa and lambda light chain immunostaining were performed on fixed bone marrow trephine biopsy specimen. The sensitivity of IHC was 81.8% in chronic lymphocytic leukemia (CLL) and 100% in non Hodgkin lymphoma (NHL) as regards CD20, 100% in both groups as regards CD5, 46% in CLL and 66.7% in NHL as regards CD23, 33.3% in CLL and 50% in NHL as regards kappa chain, 20% in CLL and 33.3% in NHL as regards lambda chain. We found that IHC and flow cytometry are equally effective in diagnosing CLL; however, IHC might be slightly more sensitive than flow cytometry in detecting bone marrow infiltration in NHL and hairy cell leukemia (HCL).

Rare Presentation of Unicentric Castleman's Disease in the Parotid Gland

Castleman's disease is a rare benign lymphoproliferative disorder of unknown etiology; however, recent data have suggested a strong association with human herpesvirus type 8 and hypersecretion of the cytokine interleukin 6. Castleman's disease can present anywhere throughout the body; however, it is most commonly detected in the chest, neck, or abdomen. Two main forms of the disease have been identified according to location: unicentric and multicentric. Unicentric Castleman's disease differs from the multicentric form in that patients are often asymptomatic, and surgical resection of the lesion is considered curative. This unicentric or multicentric nature often serves as a distinguishing feature between benign or potentially malignant presentations. The diagnosis of Castleman's disease is challenging because of its nonspecific manifestation. The most reliable diagnosis is achieved though surgical resection and histopathologic confirmation. We report a rare case of unicentric Castleman's disease in the parotid gland and emphasize its importance in the differential diagnosis of oral-facial masses.

Osseous Metaplasia in Castleman's Disease: A Case Report

Background. Castleman's disease is a benign lymphoproliferative disorder. The disease may be localized or multicentric. Three histologic variants are recognized: hyaline vascular, plasma cell, and mixed types. Case. A 20-year-old man presented with large left axillary mass. The histologic examination of the resected mass showed follicular pattern with large nodules of mantle cells arranged concentrically around atrophic and vascularized germinal centers. There was also some benign-appearing bone trabeculae interspersed with lymphoid tissue. The diagnosis of Castleman's disease, hyaline-vascular type with osseous metaplasia, was made.

Castleman's Disease of the Renal Sinus Presenting as a Urothelial Malignancy: A Brief Case Report

Castleman's disease is a rare benign lymphoproliferative disorder that frequently affects lymph nodes of the mediastinal thorax and the neck. It very rarely affects the renal sinus. We report a case of Castleman's disease arising in the renal sinus in a 64-year-old man. The patient visited the hospital with the chief complaint of hematuria. Abdominal computed tomography revealed a homogeneous mass in the sinus of the left kidney, radiologically interpreted as a malignant urothelial tumor. Subsequently, nephroureterectomy was performed, after which microscopic examination of the specimen revealed a diffuse lymphoproliferative lesion with reactive lymphoid follicles of various sizes and prominent plasma cell infiltration of interfollicular spaces, highlighted by immunohistochemical staining for CD138. The lesion was diagnosed as Castleman's disease of the plasma cell type. Although preoperative diagnosis of Castleman's disease is difficult and the incidence is exceedingly rare, it should be considered in the differential diagnosis of renal sinus tumors.