A 47 year old male with one year history of exertional dyspnea & palpitation was admitted with complaints of syncopal attacks of sudden onset. He denied any head ache, chest pain, palpitation, edema legs, persistent fever, weight loss or loss of appetite.
He had been seen at the trauma center 4 years ago following a bike accident. X-Ray at that time showed a lytic lesion in the distal end of the right femur with destruction of the lateral cortex and break in the anterior and posterior cortex of the lateral condyle with extension of the lesion to the distal femoral articular surface and a pathological fracture and periosteal reaction along the lateral aspect of distal femur (Figure ). CT scan of right lower femur showed expansile lytic lesion noted in the lateral condyle of femur with associated soft tissue mass noted extending out side the cortical margins with calcific densities and bony fragments within (Figure ).
X-Ray lower end of femur showing Giant Cell Tumor.
CT Scan of Knee Joint showing Tumor extent.
A biopsy was sent from lytic lesion of fractured lower end of femur. A curettage followed by cement application with cancellous leg screw was done. After 15 days bone cement removal, bone grafting and internal fixation with condylar blade plate was performed. Post operatively patient had minimal restriction of right knee flexion. Clinical and radiological follow up showed healing of the lesion. Cut section of the curettage material consisted of multiple hemorrhagic and tiny grey white focal areas. Microscopy showed bony trabeculae and a tumour composed of sheets of mononuclear stromal cells and many scattered multi nucleated osteoclast like giant cells (Figure ). Histologic diagnosis was Giant cell tumor of the bone.
Giant cell tumor. Sheets of mononuclear stromal cells with scattered multinucleated osteoclast-like tumor giant cells.
He had multiple spotty pigmentations (lentigines) on his trunk for several years (Figure ). He also had been noted to have swelling of submandibular region requiring three times surgical excisions for the recurrence of the mass (Figure ) which was pathologically confirmed to be a poorly circumscribed lesion composed of myxoid nodule containing thin walled capillaries along with spindle shaped or stellate fibroblasts. The features were suggestive of superficial angiomyxoma (Figure ).
Superficial Angiomyxoma . A small swelling in the submandibular region.
Superficial angiomyxoma. Spindle to stellate fibroblasts in myxoid stroma with thin walled capillaries.
His cardiac exam showed normal sinus rhythm at 84 beats per minutes and blood pressure of 130/80 mmHg. Mitral first heart sound was slightly accentuated, but the pulmonic sound was normal. Grade-I diastolic murmur was heard over the mitral area. Opening snap was absent. Lungs were clear and chest radiograph showed slight cardiomegaly. Trans-thoracic Echo cardiography revealed an intra-cardiac tumour attached to inter atrial septum, which was almost filling the left atrium & obstructing the mitral inflow. Moderate amount of mitral regurgitation was present (Figure ). Coronary angiogram showed normal epicardial coronary arteries.
An intra-cardiac tumour attached to inter atrial septum, which was almost filling the left atrium & obstructing the mitral inflow.
Patient underwent surgical excision of left atrial myxoma under cardiopulmonary bypass through right atrial approach. When a finger was introduced through the right atrial appendage a firm, smooth, egg sized tumour was encountered. The mitral valve was normal in structure and function. It was excised under vision. The patient had an uneventful recovery without neurologic or renal damage with significant relief of clinical symptoms. Follow up Echocardiography after 6 months showed no evidence of any intra cardiac recurrence.
Pathological examination of the tumour revealed a solitary mass weighing 50 gms & measuring 6.5 × 4.5 × 2.5 cms. Externally the tumour appeared congested, shining with myxoid areas (Figure ).
Gross picture of resected atrial myxoma showing glistening myxoid appearance with areas of congestion.
Microscopically it was a hypocellular myxoid tumour with small polygonal, spindle & ovoid tumour cells (Myxoid cells) with round to oval nucleus, scanty eosinophilic cytoplasm, arranged in strands, along with large " Lipidic" cells having abundant vacuolated, clear cytoplasm arranged around thin walled blood vessels in perivascular pattern. Focal nesting of tumour cells was seen. Stroma showed extensive myxoid change. Hemosiderin laden macrophage, focal dense lymphocytic infiltrate, plasma cells, mast cells were also seen (Figure ).
Atrial myxoma. Small polygonal to spindle shaped cells in myxoid stroma along with focal lymphoplasmacytic infiltrate.