PMC6424710_01
A: AP X-ray view of the left humerus of the patient obtained 38 years ago. Note the deformity of the humerus and the irregularity of lateral cortex by a lesion with calcific matrix which occupies proximal two thirds of the medulla. The patient was decided to be followed up with the diagnosis of Ollier's disease. B: AP X-ray view of the left humerus of the patient obtained 20 years ago. The lesions matrix was more calcified and deformity was relatively corrected by the time, when compared with the X-ray of the humerus obtained 18 years ago. No malignant degeneration was detected. C: The AP X-ray view of both hands of the patient which was obtained at annual follow-up visits 20 years ago. Multiple lesions can be detected in phalanges and metacarpals.
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The patient had the history of Ollier's disease which was diagnosed by detection of multiple enchondromas on both hands and mid-diaphysis of the left humerus at the age of 10 and followed-up annually since then (Fig. 1A-C).
A: The AP x-ray view of the left humerus of the patient at referral. Note the discontinuity in medial and lateral cortices between the proximal and mid-third of humerus. The matrix of the lesion demonstrates irregular punctate calcifications in the distal part. The proximal third seems more lytic without a transition zone. An oblique middiaphyseal fracture line is evident. B: The lateral X-ray view of the left humerus of the patient at referral. The anterior cortex is lytic. Posteriorly a sunburst-like calcification pattern which extends from the cortex is observed in the mid-third of humerus. The oblique fracture line can be detected at distal part of the lesion.
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A slightly oblique fracture line in the junction of proximal and mid-third of humerus with punctate cartilaginous calcifications was evident (Fig. 2A and B).
A, B: CT views of the left humerus indicate a 10 x 5 x 5 cm lytic lesion which expands and thins the cortices of proximal half of the humerus. The lesion includes frequent calcifications in matrix distally and causes an almost complete cortical deletion of medial and posteromedial cortices in proximal and mid-third collisions. An oblique fracture line can be detected.
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A skip lesion was also evident just proximal to the lesion (Fig. 3 A and B).
The MRI of the left humerus exhibits a parenchymal tissue-like lesion which is heterogeneous, and hypointense in T1-weighted, and heterogeneous-hyperintense in fat suppression sequences. Numerous millimetric hypointense foci and necrotic-like areas are unevenly distributed throughout the lesion. Note the mid-diaphyseal diffuse edema effecting the fibers of triceps brachialis muscle which is highly suggestive of pathologic fracture. A possible skip metastasis like lesion is evident in proximal medial humerus with the same characteristics as the lesion.
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The proximally located skip lesion was evident in all MRI sequences (Fig. 4A-C).
A: The macroscopic view of the resected specimen. The coronal plane section at the level of midshaft demonstrates a heterogenous, grayish lesion, including a white foci of cartilage distally. The surrounding the bone is interrupted in different focal points by the soft tissue extension. B: The lesion in the humerus. A cartilaginous tumor with mild to moderate atypia and mixoid quality of the matrix which diffusely infiltrates the surrounding tissue. The tissue is neighbored to a proliferation of malignant epithelial cells which is growing in glands and solid nests. The lesion also demonstrates squamoid features and focal mucus production within a desmoplastic reactive stroma. These features are highly consistent with a metastatic cancer developed in a grade 2 chondrosarcoma on Ollier's disease (Haematoxylin&Eosin, 10x). C: The lesion in the humerus with higher magnification (Haematoxylin&Eosin, 20x). D: Cytokeratin 7 positivity in the metastatic cancer (10x). E: The previous biopsy from lung cancer. The proliferation of malignant epithelial cells growing in glands and solid nests, with squamoid features and focal mucus production which is set in a desmoplastic reactive stroma (Haematoxylin&Eosin, 10x).
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Macroscopic histopathological evaluation demonstrated the presence of a 100 x 50 x 40 mm cortical bone which contained a focal area of a grayish lesion (Fig. 5A). During microscopic examination a distinct Grade I/II central chondrosarcoma and surprisingly, a metastatic, poorly differentiated carcinoma with squamoid aspects and desmoplastic reaction around metastatic region were detected in this patient with Ollier's disease (Fig. 5B-D). The morphological comparisons between the previous biopsy specimens obtained from breast and lung carcinomas of the patient revealed that the metastatic focus originated from pulmonary adenocarcinoma (Fig. 5E).
Postoperative 6th week follow up AP x-ray of left humerus demonstrated no sign of allograft fracture or subluxation.
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At six weeks follow-up, the patient was radiologically stable, no sign of peripheral neurologic deficit was detected and the mobility of the left hand was preserved (Fig. 6).