Rare Lymphoproliferative Disorder Castlemans Disease Biology Essay


Castleman's disease (CD) is a group of rare lymphoproliferative disorders sharing characteristic clinical and histological features, and usually accompanied by a marked systemic inflammatory response. Three histological types: the hyaline-vascular, the plasma-cell and the mixed types are described. The former is more common (80%-90%) and localized.The plasma cell type is more aggressive and usually multicentric. (1)

The localized form of CD arises predominantly in the mediastinum, where it was first described by B. Castleman. (2) Testa et al(3) have studied the location of localized CD and found 65% localized in the mediastinum, 16% in the neck, 12% in the abdomen, 3% in the axilla and 4% in diverse location.

A 37 years old female attended the gynaecology outpatient department with chief complain of pain abdomen. A suprapubic fixed firm mass was palpated on per abdominal examination. Per vaginal examination also revealed an irregular, not freely moving mass palpated through right fornix. The other fornix and uterus was unremarkable. Her routine hematological and biochemical examination were within normal limits apart from mild anaemia and raised fasting blood glucose levels.

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Patient underwent transvaginal sonography which showed a hypoechoic mass measuring 15.5 cm, attached to right broad ligament and separate from the right ovary and tube (Figure 1). The uterus and other side of the adnexae were with in normal limits. Keeping in mind, the clinical and radiological findings, a provisional diagnosis of broad ligament fibroid was made. After stabilizing her blood glucose levels, En bloc dissection of broad ligament fibroid along with hysterectomy was done and the specimen was sent to the department of pathology for histopathological examination.


On gross, the specimen consisted of body of uterus and cervix along with a large round well circumscribed mass measuring 17 cm in its greatest dimension. The cut surface of the mass was completely solid and gray white (Figure 2). Representative sections from mass was taken and processed for microscopic examination.

Microscopically, the tumor comprises large follicles scattered in a mass of lymphoid tissue. The follicles showed marked vascular proliferation and hyalinization of abnormal germinal centres. There was tight concentric layering of lymphocytes at the periphery of follicles resulting in onion skin appearance (Figure 3). Numerous post capillary venules and conspicuous absence of sinuses were also seen and a histopathological diagnosis of Castleman Disease, hyaline vascular type of broad ligament was made.

Patient was discharged on 12th post-operative day with an uneventful recovery. Post diagnosis, patient was screened for HIV 1&2 and HHV 8, which came out negative. The globulin levels were also within normal limits. Patient was also referred to endocrinology to control her blood glucose levels.


Castleman's Disease is a morphologic syndrome uniting a group of diseases with related and occasionally, overlapping pathogenesis. Recent authors favor classifying CD by histopathogenic type as opposed to the traditional unicentric versus multicentric model. (4,5) Danielle et al (6) has described four subtypes, clinical and etiopathogenic features of Castleman's disease based on histopathogenic type in Table 1.

The clinical presentation of retroperitoneal localized CD can be due to symptoms related to compression effect on surrounding organs as nausea, vomiting, weight loss, post-prandial discomfort, abdominal or lumbar pain. (7) The localized forms are asymptomatic in 51% of the cases and are often discovered at the time of routine physical examination, chest x-ray or routine abdominal ultrasonography. The present case had a huge broad ligament mass and clinical presentation was related to its location and size.

Ultrasonography, CT scan, and magnetic resonance imaging have proven to be useful in the diagnosis of masses located in the retroperitoneum. However, the images of CD resemble other masses including lymphoma, tuberculosis, sarcoidosis, and retroperitoneal sarcomas, the present case being misdiagnosed as broad ligament leiomyoma on TVS. Ultimately, biopsy of an enlarged lymph node or mass is mandatory to reach at a definitive diagnosis.(6) The present case was also misdiagnosed as leiomyoma on USG as well as on per-operative examination and definitive diagnosis could be reached only on histopathological examination. This could be attributed to the unusual location of the tumour which has a rare incidence.

Treatment of localized CD not associated with HHV-8 infection generally is surgical resection, with or without radiotherapy, which is almost always curable, resulting in rapid resolution of systemic symptoms and laboratory abnormalities. (6) Complete surgical resection of the broad ligament mass was done in this case also and the patient is keeping well till date. At present there is no consensus about the optimal management strategy for patients with the multicenteric form of CD. Successful treatment of such patients has been achieved by using combination chemotherapy with or without prednisone, administered at the time of initial diagnosis.(8)

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The patient's immunological status seems to play a major role in the development of this disease. While localized form is encountered mainly in immunocompetent patients, the systemic form is found in patient with AIDS or other immunodeppression related either to immunosuppression or pathological state. (9)The present case was seronegative for HIV and HSV 8 but was diabetic and was diagnosed as localized CD with no systemic manifestations.

It seems that CD is the result of a chronic low grade inflammatory process triggered by latent infection with HHV8. (10) Chronic infection by HHV8 stimulates secretion of IL-6 which in turn induces a hyperplasic reaction of the lymphoid system. (9,10) While this lymphoid hyperplasia could be contained in one lymph node as in the localized form, which is mainly of hyaline vascular type, it could also be generalized as in the systemic, or multifocal, form which is the predominant form for the plasma cell type. (9) Interestingly, the genome of HHV-8 encodes a viral analog to human IL-6 and is known to have both hematopoietic and angiogenic effects. Although the exact role of viral IL-6 in the pathogenesis of HHV-8- related diseases has yet to be elucidated. (4) Though the hypothesis has a scientific base, the present patient was negative for HHV-8 serology, so CD of hyaline vascular type may occur in persons who are serologically negative for HHV-8.

Lymph nodes involved by hyaline-vascular CD are known to have alterations in the follicular dendritic cell networks with reports of proliferations of dysplastic follicular dendritic cells and follicular dendritic cell tumors. Vascular endothelial growth factor may contribute to the prominent vascular proliferation seen in this subtype. (5)

In conclusion, pathologists as well as the clinician should be aware of the possibility of pelvic involvement of this disease in spite of its extreme rarity. Despite imperfect understanding of the complex pathogenesis of this disorder, pathologists must be aware of the various forms of CD and their relationship to follicular dendritic cell tumors, malignant lymphomas & Kaposi sarcoma and also, every patient should be serologically investigated for HIV & HHV 8 status to ensure better understanding about etiopathogenesis of this disease.