| Japanese Journal of Clinical Oncology | Pages |
Introduction
Case Report
Discussion
Acknowledgments
References
Coxalgia as the Initial Symptom in Hodgkin's Disease: A Case Report
INTRODUCTION
The most common presentation of Hodgkin's disease is progressive painless lymphadenopathy (1 ). Although bone involvement is frequent during the course of the disease (2 ,3 ), bone pain is rarely the initial presenting symptom (4 ). We report the case of a woman with Hodgkin's disease who had complained of increasing pain in the left hip joint 17 months before nodal disease was noted.
CASE REPORT
A 41-year-old woman was admitted to the National Cancer Center Hospital East in September 1993 because of progressive pain in the left hip joint, of two years' duration. A right cervical mass and nocturnal sweating developed 7 months prior to this admission. Body weight loss of 9 kg over two years, but no fever, was noted during her clinical course. Her past and family history was unremarkable and she did not drink alcohol or smoke.
On physical examination, the left inguinal portion was warm and swollen and a generalized firm enlargement of the superficial lymph nodes, with a maximum diameter of 2 cm, was noted. Her liver and spleen were not palpable.
Hematological study showed a white blood cell count of 16 900/[mu]l with 76% segmented neutrophils, 4% band forms, 1% eosinophils, 6% monocytes and 13% lymphocytes, a red blood cell count of 416 * 104/[mu]l, a hemoglobin level of 10.8 g/dl and a platelet count of 50.5 * 104/[mu]l. The erythrocyte sedimentation rate was 67 mm/h. Analysis of blood chemistry revealed her liver and renal functions were within normal limits, except for an elevated LDH level (556 IU/l). Her C-reactive protein and ß2-microglobulin levels were 4.2 and 2.03 mg/dl respectively, and serologic tests for human T-cell leukemia virus type 1 and human immunodificiency virus were negative. A chest X-ray was normal. Roentgenograms of the left hip joint revealed osteolytic and sclerotic lesions in the left acetabulum and the neck of the left femur (Fig. 1 ). The 99Tc bone scan showed elevated radioisotope uptake in these lesions and the 12th thoracic and first lumbar vertebrae. MRI scanning of the pelvis disclosed a mass lesion in the left femur, sacrum and left iliac bone, the latter of which was 6 * 4 cm in size and protruded into the pelvis. The masses were iso-intense on T1-weighted images and well enhanced on the early phase images after gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA) administration (Fig. 2 ). Ultrasound scanning of the abdomen showed no positive findings except for splenomegaly with multiple low echogenic nodules with a maximum diameter of 3 cm.
DISCUSSION
An onset with bone symptoms is extremely rare in Hodgkin's disease; only 21 cases have been reported in the English and Japanese literature (4 -14 ). Of these, three had primary Hodgkin's disease in the bone, the extension of which was definitely restricted to a single bone lesion. In the other cases, in which nodal diseases were noted during their clinical course, it was difficult to be certain that Hodgkin's disease originated from the bone. It was also difficult to prove this in our patient, but she probably had primary Hodgkin's disease of the bone because: 1, bone pain appeared 17 months prior to nodal disease becoming evident clinically; 2, on admission, the bone lesion was much larger than the lymph node lesions. The clinical features of these 22 patients are summarized in Table 1 .
The cell of origin in Hodgkin's disease has long been controversial, but is suspected to be B- or T-cell in most cases, according to recent studies on surface markers and rearrangements of immunoglobulin and T-cell-receptor gene (15 ). Hodgkin's disease can arise from almost all organs including the bone, although clinically it develops exclusively in the lymph nodes: the incidence of extranodal disease was estimated to be 0.25% (16 ). The histological subtypes of the primary Hodgkin's disease in the bone reported in the literature are nodular sclerosis in 9 of 17 (53%) cases, mixed cellularity in 5 of 17 (29%), lymphocyte depletion in 2 of 17 (12%) and lymphocyte predominant in 1 of 17 (6%) (4 ,8 -14 ). This distribution does not differ greatly from that of nodal or extranodal disease originating elsewhere (17 ).
Table 1
| Feature | No of cases |
| Sex male/female | 13/9 |
| Initial symptom | |
| Bony pain | 14 |
| Neuralgia | 5 |
| Sternal mass | 3 |
| Systemic symptom yes/no | 12/10 |
| Nodal disease yes/no | 19/3 |
| Bone involved | |
| Pelvis | 10 |
| Femur or tibia | 9 |
| Spine | 7 |
| Humerus or ulna | 4 |
| Sternum | 3 |
| Scapula | 3 |
| Ribs | 1 |
| Skull | 1 |
Clinical findings and small biopsy specimens are often insufficient to establish a diagnosis of Hodgkin's disease of the bone. Our review of the 21 reported cases disclosed that the bone lesions of most cases had been reported initially as metastatic bone tumors, Paget's disease, chondrosarcoma, primary sarcoma of the bone, eosinophilic granuloma or osteomyelitis, and that surgically resected specimens, biopsy specimens of the lymph nodes or autopsy samples were required to establish the true diagnosis (4 -14 ). In our patient, two years had passed before Hodgkin's disease was diagnosed from findings of the bone and lymph node biopsy specimens, when generalized lymphadenopathy was noted.
Patients with Hodgkin's disease localized to the bone (stage IE) treated with surgical resection and/or radiotherapy seemed to have a relatively good prognosis, whereas those with nodal disease (stage IV) required systemic therapy (4 ,7 ,11 ). Combination chemotherapy for advanced nodal Hodgkin's disease is well established: ABVD therapy for 6-8 months produced a complete response rate of 82% and 5-year survival of 73% (18 ). The efficacy of chemotherapy for disease of bone origin, however, has not been evaluated fully. Although complete remission was achieved in 8 of 11 (73%) patients treated with combination chemotherapy reported in the literature, their prognoses were unclear, because few of them were followed up for long (12 -14 ). The patient described in this report was never in complete remission. Her poor response to the cytotoxic treatment was probably due to the large size of the lesion and its site, as our review of the literature revealed that patients with pelvic or spinal involvement tended to show poorer responses and seemed to have poorer prognoses than those with peripheral bone lesions only, as appears to be the case with primary non-Hodgkin's lymphoma of the bone (19 ).
In conclusion, Hodgkin's disease rarely arises primarily from the bone. Most of such patients have stage IV disease by the time they receive initial treatment, because it is difficult to make the true diagnosis before lymphadenopathy is noted. Patients with Hodgkin's disease originating from the pelvis or spine are unlikely to survive for long, because their responses to cytotoxic therapies are relatively poor.
Acknowledgments
We thank Drs Koji Murakami and Shigeru Nawano for their contribution to evaluating the MRI images and Drs Hirofumi Fujii, Tomoko Ohtsu, Hisashi Wakita, Tadahiko Igarashi and Kuniaki Itoh for their valuable advice on the care and management of this patient and comments on the manuscript.
References
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Copyright© Japanese Journal of Clinical Oncology, 1997.
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