|Year : 1976 | Volume
| Issue : 3 | Page : 27-29
Shashi Kapoor, GC Sood, AL Aurora, M Sood
Jawahar Lal Institute of Postgraduate Medical Education and Research, Pondicherry, India
Jawahar Lal Institute of Postgraduate Medical Education and Research, Pondicherry
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kapoor S, Sood G C, Aurora A L, Sood M. Alveolar Rhabdomyosarcoma. Indian J Ophthalmol 1976;24:27-9
The high incidence of rhabdomyosarcoma has been appreciated only in the recent years. It is considered to be the third commonest tumour in children. The origin of the tumour is believed to be from the immature orbital tissues which have remained there from the embryonal stage of development.
Rhabdomyosarcomas have been classified into the pleomorphic; the embryonal and the alveolar types. A large number of cases belonging to the first two groups have been described,,,,,,,, but only a few reports are available about the alveolar rhabdomyosarcoma involving the orbit,,,.
The purpose of this communication is to present a case of alveolar type of rhabdomyosarcoma in a young female child of three years, presenting as a lid mass without proptosis in initial stages.
| Case Report|| |
A 3-year-old girl, reported with a rapidly progressive painless swelling of the right lower lid. The swelling appeared for the first time about 3 months back.
On clinical examination, the growth was found to be nodular with a cystic feel at a few places. The skin was non-adherent and there was no sign of active inflammation. The posterior limit of the swelling could not be felt. The eye was deviated up with limitation of its movement in the down gaze. There was no proptosis [Figure - 1]. The fundus was normal. Intra-ocular pressure was 17.3 mm. Systemic examination was negative. A clinical diagnosis of neurofibromatosis was made.
All the investigations including total and differential leucocytic counts, stool examination, X-Ray of orbit and optic foramina and chest and sternal biopsy were found to be normal.
The tumour was removed through a horizontal incision about 5 mm. below and parallel to the lid margin extending almost from the medial to the lateral canthus. The mass was dissected all around. Posteriorly it was extending into the orbit as a cone below the eye ball for a distance of about 2 cm.
No growth was apparent for about a month, when the mass again started appearing. This time it was all around the globe. The eye ball was still shifted up but now it was protruding forward also. Simultaneous with the reappearance of the orbital growth, the right sided submandibular lymph nodes started enlarging. The patient was advised to undergo exenteration and radio therapy, but the patient refused.
The gross examination of the growth showed it to be pear shaped, lobulated mass brownish in colour and was measuring 3.3 x 3 x 1.3 cm. Cut surface showed lobulated greyish appearance with areas of haemorrhages
Microscopic examination revealed alveolar pattern with setae lined with cells which had their nuclei close to the septum. The alveoli had almost a clear lumen with a few cells, some of which were multinucleated.
Tadpole-shaped cells were seen in plenty. Bizarrepattern of cross striations could also be made out. [Figure - 2][Figure - 3][Figure - 4]
| Discussion|| |
Rhabdomyosarcoma in the recent years has become the commonest orbital tumour in children. Out of its three varieties, the embryonal type is the commonest variety. But since the description of alveolar type of rhabdomyosarcoma, a few cases of this variety have been reported from the orbital region also.,,,
The alveolar rhabdomyosarcoma differs from the embryonal type in many respects. The embryonal type has a predilection for the superior nasal quadrant, while the alveolar type is seen more often in the lower part of the orbit. The growth in our patient also appeared for the first time in the inferior part of the orbit.
The histological picture of the alveolar type of rhabdomyosarcoma is also very characteristically different from other varieties. The alveolar pattern can be easily made out. The alveoli are more or less empty except for a few free cells, some of which are multinucleated where sometimes both longitudnal as well as cross striations can be made out. The presence of tadpole-shaped cells is another diagnostic feature.
The alveolar type of rhabdomyosarcoma is believed to be a highly malignant tumour. Secondaries due to this tumour have been described in brain, paranasal sinuses and lungs. Its extension in to the cervical lymph nodes has been described only once. In our patient, secondaries in the submandibular lymph nodes appeared 4 months after the growth made its first appearance. The early involvement of the submandibular lymph nodes suggest its rapid lymphatic spread.
| Summary|| |
A case of alveolar type of rhabdomyosarcoma is reported in a female child of 3 years. A very rapid rate of growth with early metastasis in cervical lymph nodes was noticed. Upward deviation of the globe and a lid mass were the first signs to appear, proptosis appeared quite late.
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]