Malignant lymphoma presenting as Tietze's syndrome


Tietze's syndrome is usually considered a benign process that generally strikes young adults. It is characterized by a painful, nonsuppurative swelling of the sternoclavicular joint, or by 1 or more chondrosternal joints of the thorax (1). In most cases (80%), only 1 costal cartilage is involved, but other joints can also be affected simultaneously. The etiopathogenesis of this disease, which many authors consider to be an enthesis disease, is unknown; the microtraumatic theory currently seems to be the most probable hypothesis (2, 3). Histologic examination of the swelling generally shows the nonspecific aspects of hypervascularization and degenerative phenomenon of the cartilage (4).

The symptoms and signs of Tietze's syndrome may occur in all arthropathies involving the anterior thoracic wall, particularly in seronegative spondylarthropathy (5, 6). Arthritis of the sternoclavicular joint is a frequent finding in rheumatoid arthritis, whereas diseases such as gout and septic arthritis caused by pyogens, Brucella, mycetes, or Mycobacterium tubercolosis are rarely localized at the level of the condrosternal joints. Besides these pathologies, which are interesting from a strictly rheumatologic point of view, there are many other diseases to consider in the differential diagnosis, including multiple myeloma, primitive neoplasms of the bone and soft tissues, chondrosarcoma of the chondrocostal joints, extension to the costal cartilage of tumors of the breast and the lung, as well as metastases of breast, kidney, and prostate neoplasms (7).

We have observed 4 young patients presenting with a painful swelling at the level of the first sternocostal joints. These cases serve as a reminder that Tietze's syndrome may be a manifestation of a malignant disease. In all cases, physical examination results were otherwise within normal limits. Erythrocyte sedimentation rate and serum C-reactive protein were normal. Liver and renal function were normal. Standard radiography of the chest showed normal results. Telethermography showed an area of hyperthermia at the site of the lesion. In these cases, the first diagnoses were of Tietze's syndrome, and these patients were treated with nonsteroidal antiinflammatory drugs and local infiltrations of steroids. Given the persistence of the clinical picture, a computerized axial tomography (CT) of the chest was performed. In 2 cases, a biopsy of the mass identified by CT revealed high-grade non-Hodgkin's lymphoma. In the other 2 patients, laterocervical lymphadenopathy appeared and a lymph node biopsy was carried out. Hodgkin's disease was diagnosed in both of these cases. The staging of the disease was carried out by CT, lymphography, bone marrow biopsy, and an otorhinolaryngologic examination.

A long-lasting remission of the disease was achieved with appropriate chemotherapy and radiotherapy. The skeletal system is frequently involved in malignant lymphomas, but arthritis with infiltration of the synovia by lymphomatous cells is rare. In 1980, the case of a woman with non-Hodgkin's lymphoma who presented with symptoms of arthritis of the right sternoclavicular joints was described (8).

The involvement of the sternum is frequent in the initial phases of Hodgkin's lymphoma, perhaps because of its proximity to the thoracal lymphatic ducts, or possibly because of the mediastinal localization of lymphoma, which can compress and invade the structures of the thoracic cage. Costal or clavicular localizations are, however, rare (9, 10).

The cases that we report demonstrate that the suspicion of Tietze's syndrome always requires an accurate differential diagnosis, and it underlines the importance of a CT examination for this purpose.