Martin Charron, M.D.
J.A. Parker, M.D., Ph.D
December 1, 1987
A 47 year-old white male presented with coughing for three months, accompanied
by painful swollen knees, legs and ankles.
A chest X-ray (arrow) and a CT scan (arrow) done 1 month after
presentation showed a 4 cm right lower lobe mass. The lung washing was
positive for bronchial adenocarcinoma of the lung. The bone scintigram revealed increased uptake in the lower
extremities consistent with hypertrophic pulmonary osteoarthropathy (HPO). He
was started on PFM (Platinium, 5-Fluorouracil, Methotrexate).
Secondary hypertrophic osteoarthropathy (HO), also known as the Marie-Bamberg
Syndrome, consists of clubbing of the finger and toes, periostitis of the long
bones, sometimes a polyarthritis resembling rheumatoid arthritis,
hyperhydrosis, flushing, and blanching. Ossifying periostitis is seen on x-ray
at the distal end of the shaft of the long bones as an opaque line of new bone
formation, separated from the underlying cortex by a narrow radiolucent band.
As it accumulates, the periosteal new bone becomes irregular, rough and
undulating and eventually fusing with the cortex. The radiological
differential diagnosis includes thyroid acropachy, chronic venous stasis,
hypervitminosis A and infantile cortical hyperostosis.
Four to twelve percent of the patients with bronchial carcinoma develop HO. But
HO may precede the discovery of the neoplasm by several months. There are over
60 extra and intra-thoracic diseases which have been associated with HO. When
there is no know associated disease this condition is referred as
pachydermoperiostitis, a rare familial disorder.
Pathophysiology: Histologically there is an inflammatory reaction
with round cell infiltrates and latter the periosteal new bone formation
develops. The pathogenesis of HO remains elusive. Estrogens, circulating
factors, neurogenic factors and growth hormone have been postulated to play a
role (5). Of special interest is the regression of the new periosteal bone which
frequently occurs after removal of a lung carcinoma. The pre-operative pain
may also regress completely in 4-5 days (3).
Thoracotomy, hilar neurectomy, vagotomy, ipsilateral occlusion of the pulmonary
artery, radiotherapy, chemotherapy, intercostal nerve section, laparatomy (1),
and chemical vagatomy sometimes lead to remission of the symptom (2), but the
mechanism by which these diverse procedures lead to remission is uncertain.
Radionuclide bone imaging is a very sensitive method for detecting
abnormalities of HO. A diffuse symmetric increased uptake in the diaphysis and
metaphysis of tubular bones along their cortical margin create a distinctive
"parallel track". Associated synovitis can lead to increased radionuclide
uptake in the periarticular region. Ali (1) reported the following
distribution of bony involvement in 48 patients: tibia and fibula 95%, femur
88%, hands and carpels 88%, radius and ulna 85%, feet 81%, scapula 67%,
mandible 42%, clavicle 33%, ribs 2% and pelvis 2%. Of note, none of these 48
patients had increased spinal uptake. A patchy uptake in the long bones is not
These scintigraphic findings frequently appear before roentgenographic
findings, correspond well with clinical manifestation and decreased after
1) Ali, et al. Distribution of HPO. AJR 1980; 134:771-780.
2) Resnick, et al. Diagnosis of bone... Saunders 1980; 3:2988.
3) Rosenthal, et al. Skeletal imaging. ACC 1984; 297.
4) McFee. SNM 1987; 17(4):346.
5) Galasko. Radionuclide scintigraphy in orthopedics. C.L. 1984; 253.
6) Kelley, et al. Textbook of Rheumatology. Saunders 1985; 2:1594.
J. Anthony Parker, MD PhD, email@example.com