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Symptomatic Lower Limb Varices Secondary to Castleman’s Disease
Giuliano Trevisan, Valentina Gobessi, Cosimo Convertino, Mauro Melato1
Institute of Clinical Surgery, Cattinara Hospital, University of Trieste; and 1Institute of Pathologic Anatomy, University of Trieste, Trieste, Italy
Although lower limb varices are very frequent, their association with Castleman’s disease is extremely rare. We report a case of Castleman’s disease involving the right inguinal lymph nodes with subsequent development of the symptoms caused by the compression of the great saphenous vein’s gulf. The patient underwent surgical extirpation of the right inguinal mass, which successfully eliminated all the symptoms. The great saphenous vein was not stripped in order to leave an outlet for the deep venous blood flow in case of subsequent compression of the right femoral or iliac veins. This case confirms the effectiveness of surgical treatment in the localized form of Castleman’s disease.
Key words: angiofollicular lymphoid hyperplasia; hyaline substance; inguinal canal; lymphadenopathy; varices
Castleman’s lymphoma is a rare mediastinal lymph node hyperplasia, first described in 1956 (1), whose etiology and pathogenesis are still unknown. It is usually located in either superficial or deep lymphoid tissues such as mediastinum (2-5), neck (6-8), mesentery (9), pelvis (2,10,11), retroperitoneum (12), and/or axilla (13); it may exceptionally affect extranodal sites like striated muscle (14), thoracic wall (15), lung (16), skull, larynx, and/or vulva (10,11). Castleman’s disease is characterized by tumorous masses of lymphoid tissue showing plasma cell or hyaline vascular type changes in histological specimens (9,17). A more common localized benign form (18) and a rarer, less favorable systemic form (14,19,29) have been described.
Castleman’s disease is often difficult to diagnose (3,9). Many infectious and autoimmune diseases can mimic Castleman’s disease because of its nonspecific histological (2) and clinical (4) findings. For this reason, the diagnosis must be made by the exclusion of other diseases and in an appropriate clinical setting. We report a case of Castleman’s lymphoma located in the groin with secondary varices of the great saphenous vein.
Case Report
A 47 year-old female patient, was admitted to our hospital in February 1992 because of an enlarged mass in the right inguino-crural region. Physical examination revealed a well-defined solid mass with the typical features of lymph node enlargement. Laboratory tests and routine chest x-rays were negative. The patient underwent surgical excision. Surgical exploration revealed a mass compressing the femoral vein, and the great saphenous vein, enveloped by a tightly cohesive aggregate of several lymph nodes without coalescence.
The histological examination showed an atypical hyperplastic lymphadenopathy with angiofollicular features (Figure 1.) highly indicative of Castleman’s disease (7,10,11,13,17,20).
Lymphocyte characterization test and abdominal echography were negative. CT revealed multiple lymphonodal swellings of about 1 cm in diameter in the area around the aorta and inferior vena cava, and below the renal arteries down to the iliac bifurcation. Similar findings were found along the right iliac vessels and in the right inguinal area. The patient was consequently treated with 3 cycles of cortisone and chlorambucil.
Chest x-ray, abdominal ultrasonographic, and CT findings were unchanged at the follow-up. Inguinal swelling remained unchanged at the physical examination.
Two years later, the patient was readmitted to our hospital because of a varicose ectasias located along the great saphenous vein. The inguinal swelling seemed moderately increased but its symptomatic features were similar to those previously observed. Several varicose dilatations along the great saphenous vein were also found. A venous Doppler showed patency of the deep venous circulation with incontinence of the sapheno-femoral ostium. Laboratory tests, protein immunoelectrophoresis, and chest x-ray were within the normal range.
The patient underwent another right inguinal incision. A lump of about 10 cm extended superiorly as far as the inguinal ligament. It appeared encapsulated, compressing the great saphenous vein and its gulf (which explains secondary varices), the femoral vein, and the artery, but it was easily dissociable from these structures. After removal, the cut surface of the mass appeared highly vascularized and pink-gray in color. The great saphenous vein was not stripped in order to leave an outlet for the deep blood flow in case of subsequent compression of the right femoral vein or the right iliac vein.
Histological examination confirmed the previous diagnosis of the hyaline-vascular variety of Castleman’s disease (2). The patient was discharged 5 days after the surgery. A subsequently performed thoracic CT showed a mild enlargement of the paratracheal lymph nodes in the aorto-pulmonary window.
After 5 years, the patient had no symptoms, with only minor varices, and has never needed any medical treatment.
Discussion
Many different terms for the Castelman’s disease (angiomatous lymphoid hamartoma, giant lymphoid hyperplasia, or angiofollicular hyperplastic lymphadenoma) indicate the uncertainty of the pathogenesis of this disease. Most theories describe it as an hamartoma or a chronic inflammatory process originating from a hyperplastic reaction to an unknown antigenic stimuli (12,14,20).
This disease affects both sexes, especially in the second to third decade of life, regardless of the racial or family factors. In children, the disease may delay growth (both statural growth and body weight). Intrathoracic lymph nodes are most frequently involved (more than 70% of cases), especially in the antero-superior mediastinum and the hilar region of the lung (2-5), whereas involvement of laterocervical (6-8), axillary (13), retroperitoneal (2,12), mesenteric (9), and pelvic (2) lymph nodes is less frequent.
Other localizations are extremely rare, particularly in the inguinal region, and symptoms due to venous compression are not reported in literature.
Castleman’s disease has two histological variants: the hyaline-vascular type (90% of cases) and the plasmacellular type (10% of the cases). The first type, almost exclusively in monofocal localization, becomes symptomatic when the surrounding organs are compressed by the deep lymph nodes, or when the involvement of the superficial lymph nodes causes cutaneous deformities and consequent esthetic alterations. The plasmacellular type is often associated with asthenia, night sweats, fever, and weight loss and, in terms of laboratory findings, anemia, leucocytosis, polyclonal hypergammaglobulinemia, ESR increase, and occasionally hypalbuminemia.
Another variant of the plasmacellular type which has recently been described (10,11,17) is a multicentric form which involves many or all the lymphonodal stations, both superficial and deep ones, and is frequently located in the spleen, liver, bone marrow, and lung. This type is usually found in older patients (6th-7th decade of life), although there have been reports of younger patients aged 54 (20). The general clinical manifestations of the monofocal plasmacellular type are accompanied by hepatosplenomegaly with trombocytopenia and sideropenic and/or hemolytic anemia. Autoimmune diseases such as lupus erythematosus, Sjögren’s syndrome or immune nephrotic syndrome are also frequently observed (20). Proliferation of lymphocytes and plasma cells may affect immunoregulation (10,11,14), favoring autoimmune or immunodeficiency syndromes, probably because of a regulation defect of T suppressor lymphocytes.
For these reasons, unlike the localized form, the outcome of the multicentric type is associated with the frequent development of serious infective complications (over 70%), and neoplastic evolution of the disease (39% of the reported cases), usually in the form of a non-Hodgkin lymphoma (15.2%) or Kaposi’s sarcoma (22%). Sixty-nine percent of the patients from 46 reported cases died (11,12,14,17,18), with an average survival from 30 to 37 months (11-20). Three cases of HIV-positive patients with a multicentric disease have been reported (19,21); all these patients developed Kaposi’s sarcoma only a few months after pathological manifestations of the disease. At present, surgical treatment is not recommended in the multiple localization form. The results of radiotherapy are questionable. However, temporary remission of the disease can be achieved by the corticosteroid therapy combined with mono or polychemotherapy (15,21).
It has been suggested that the two histological forms are different manifestations of the same disease due to different reactions to various antigenic stimuli (12,14,20) In this view, the hyaline-vascular type expresses limited reactivity, whereas the plasmacellular type indicates a more intense reaction. This hypothesis supports the theory of an infective-inflammatory origin of the disease. In both forms, the symptoms regress after a complete surgical removal of the affected lymph nodes.
References
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Received: February 24, 1997
Accepted: September 2, 1997
Correspondence to:
Dr Giuliano Trevisan
Cattedra di Chirurgia dell’apparato digerente presso
l’Istituto di Clinica Chirurgica Generale e Terapia
Chirurgica
Ospedale di Cattinara – Strada di Fiume
34100 Trieste, Italy
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