Paget

Disease

Humerus Cancellous Bone

Fig. 8.21 a, b The radiograph of the humerus (a) shows an enlargement of the whole bone with thickening of the cortex and an extreme coarseness of the cancellous bone pattern—so-called woven bone. This is a typical case of Paget. The view of a skull base specimen from above (b) illustrates the "textile" character of the bone in Paget disease and makes clear why the cranial nerves may eventually be compressed and why patients used to come to their physicians for advice because their hats would no longer fit. (Thanks to the Berlin Museum of Medical History for permission to use this photograph.)

Fig. 8.21 a, b The radiograph of the humerus (a) shows an enlargement of the whole bone with thickening of the cortex and an extreme coarseness of the cancellous bone pattern—so-called woven bone. This is a typical case of Paget. The view of a skull base specimen from above (b) illustrates the "textile" character of the bone in Paget disease and makes clear why the cranial nerves may eventually be compressed and why patients used to come to their physicians for advice because their hats would no longer fit. (Thanks to the Berlin Museum of Medical History for permission to use this photograph.)

inferior quality. Patients therefore are often afflicted with fractures and bending of the bone. In the early phase of the disease, osteolytic foci occur and the disease may have a rather aggressive plain radiographic appearance. The risk of a malignant transformation into an osteosarcoma is around 5%; this diagnosis should be suspected if patients develop pain in the absence of evidence of a fracture. Bone scans show a very high bone turnover.

Do You Know about Paget?

^^ Sir James Paget was a prominent surgeon and tL A pathologist in London during the late 19th O Jkt-_- century. He was the personal medical attendant to Her Majesty Queen Victoria. Several disease entities have been named after him, the most frequent being the osteitis deformans. One of his most important mottos is still true today and you as a doctor should probably also follow it at least when you converse with colleagues: "To be brief is to be wise."

Brown tumor (osteoclastoma): This phenomenon is associated with hyperthyroidism; it is an osteolytic lesion

(Fig. 8.22) that occasionally expands the bone. It owes its name to the hemosiderin content visible macroscopi-cally when dissected by the pathologist. Laboratory parameters and the other radiological signs of hyperpara-thyroidism (see p. 36) can help in the differentiation from malignant osteolytic lesions.

• Diagnosis: Giufeng is quite sure she knows what is going on. Mrs. Kristeeze suffers from osteoblastic metastases. The most probable primary tumor is a breast carcinoma. Now it is important to get a tissue sample of that primary tumor. First of all, the referring physician, who knows the patient best, is contacted and the further steps are agreed upon. Whether and how you inform the patient about a devastating condition depends on a lot of factors. If you decide to talk to your patient about such a diagnosis with far-reaching consequences, time, compassion, and a solid helping of empathy are needed. Giufeng has a look in the waiting area. Mrs. Kristeeze is completely absorbed by the latest book by Patricia Highsmith. Her personal doctor will tell her the diagnosis and guide her through the coming difficult times.

Brown Tumor

Brown Tumor

Girdle Wearers

Fig. 8.22 An extensive osteolytic focus is seen in the diaphysis of the radius of this patient with hyperparathyroidism.

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