Osseous Hodgkin disease

Abstract

BACKGROUND Hodgkin disease rarely presents as an osseous lesion, and the majority of patients are found at staging to have concurrent disease in lymph nodes. Many cases of osseous Hodgkin disease have been misdiagnosed on initial biopsy. METHODS All cases of Hodgkin disease diagnosed by open bone biopsy at the Mayo Clinic were identified. These included patients with primary osseous tumors, those presenting with multiple sites of involvement (with osseous lesions), and those with recurrence in bone. Recut sections were subjected to immunohistochemical stains to confirm the diagnosis. Clinical data and follow‐up information were obtained from patients' charts. RESULTS Twenty‐five patients (15 males and 10 females with an average age of 37 years) with osseous Hodgkin disease were identified during the years 1927–1996. Three patients had solitary, osseous tumors and two had primary, multifocal, osseous Hodgkin disease without involvement of nonosseous sites. Twelve patients who presented with lesions in osseous sites also had nonosseous tumors detected at staging, and 8 patients had recurrent Hodgkin disease that presented in bone. The majority of patients with primary and recurrent tumors presented only with bone pain; >50% of patients with concurrent osseous and nonosseous disease also had B‐type symptoms. Nearly all lesions were in the axial and proximal appendicular skeleton. Radiographic features included osteosclerotic, osteolytic, and mixed lytic/sclerotic patterns. Cortical destruction, periosteal new bone formation, and soft tissue masses were present in 50% of cases. The histologic diagnosis of osseous Hodgkin disease occasionally was problematic; osteomyelitis was the most frequent misdiagnosis. Immunohistochemical stains revealed expression of CD15 and CD30 in neoplastic cells (which were negative for CD45 and B‐cell and T‐cell antigens) in all but two cases. Involved lymph nodes typically exhibited nodular sclerosis Hodgkin disease. Three patients with primary solitary osseous Hodgkin disease received radiation treatment only; at last follow‐up 2 patients were alive at 22 months and 10 years, respectively. Patients with concurrent osseous and nonosseous tumors exhibited a 60% overall survival rate, but at last follow‐up all 4 patients diagnosed after 1986 still were alive; those with Hodgkin disease that recurred as osseous lesions had a 60% survival rate at 8 years, but only 1 of the 5 patients diagnosed since 1984 had died of disease. CONCLUSIONS Osseous Hodgkin disease typically presents with bone pain, and the majority of patients have concurrent nonosseous lesions detected at staging. Radiographic features of osseous Hodgkin disease vary but indicate an aggressive malignant process. The histologic diagnosis may be problematic; immunohistochemical stains aid in establishing the diagnosis of Hodgkin disease in bone. Survival of patients with osseous Hodgkin disease has been found to be good for the last 10 years. Cancer 1999;85:1166–78. © 1999 American Cancer Society.

Utility of bone marrow biopsy at diagnosis in pediatric Hodgkin's lymphoma

“…We also found that, as in adults, 2,3,18 BM involvement did not significantly affect the relapse-free survival or overall survival of patients with stage IV HL, nor did it carry any prognostic disadvantage in stage IV HL. 25,26 This finding is extremely important because in some low-income countries, patients with BM involvement at diagnosis may be incorrectly deemed incurable, and curative frontline therapy or radiotherapy may be withheld. Furthermore, neither the site of extranodal disease (bone, lung, or liver) nor the number of extranodal sites (1, 2, 3, or 4) significantly affected the relapse-free or overall survival of patients with stage IV HL.…”

A 7-year-old girl with hip pain and leg weakness

“…Neuroblastoma and Ewing sarcoma are most often involved, but the dierential diagnosis also includes non-Hodgkin lymphomas, leukemia, metastatic lesions, Langerhans cell histiocytosis, and a variety of benign tumors [4,5].…”

“…Histologic diagnosis of osseous HL can also be very problematic, undoubtedly because of the rarity of HL in the bones. Immunohistochemical stains are very helpful in diagnosing this tumor [5].…”

Primary multifocal osseous Hodgkin disease: a case report and review of the literature

“…Most of the cases have been associated with synchronous lymph node involvement. Since 1927, only seven patients with primary solitary osseous HD [6,19,26,31,32,33] and five patients with primary multifocal osseous Hodgkin disease have been reported without lymphatic manifestations [13,16,29,31]. Two of these cases, which have been reported before 1944, presented limited clinical and radiographic evalu- ations.…”

“…In late stage HD, osseous localizations have been described in 10-20% of cases [3,10,17,20,24,30], with less then 2% of cases showing skeletal lesions at the time of initial presentation. To our knowledge multifocal primary osseous HD without lymphatic manifestations has been reported only in five cases [13,16,29,31]. This article describes one further case and reviews the literature with special emphasis on treatment and prognosis.…”