|
Intraocular
tumours.Magy
Onkol. 2005;49(1):9-13. Epub 2005 May 18.
Intraocular
tumours may be benign or malignant. The latter are more numerous, and
endanger not only vision but life as well. Two of them deserve special
attention: melanoma malignum oculi in adults and retinoblastoma in
children. Melanoma malignum may arise from all three areas of the uvea:
the iris, the ciliary body and the choroid. The more malignant growths
are those which are situated closer to the posterior pole.
Histologically the epitheloid cell-type of melanoma is more malignant
than those containing only spindle cells. Their treatment depends on
the size: in the case of large tumours enucleation is required, while
for the smaller ones, radiation therapy can be applied. Retinoblastoma
is most common in children of 1-2 years of age. It has familial and
sporadic forms. Sixty-seven percent of the inherited-type cases are
bilateral. An early symptom in small children is strabismus. A white
tissue mass growing into the vitreous is seen on the fundus. A
diagnostic feature that can be detected by ultrasound examination is
calcification. The tumour may also present intracranially, therefore
CT of the skull should be performed in each case. Histologically the
tumour contains malignant neuroepithelial cells, which may form a
rosette. In the case of large tumours the treatment is enucleation; in
bilateral processes the bulbus with the larger mass is removed and the
other eye is treated with radiation therapy. In both cases
chemotherapy is used according to a prescribed schedule. Metastases to
the eye occur most frequently from carcinomas of the breast, lungs or
gastrointestinal tract. These are treated with radiotherapy,
chemotherapy and hormone therapy. Primary intraocular lymphoma often
occurs bilaterally, and may be accompanied by primary lymphoma of the
central nervous system (CNS). Some benign tumours are found by chance
on routine eye examinations, others due to subjective and objective
symptoms.
TNM
classification of ophthalmic malignant tumors.Gan
To Kagaku Ryoho. 1998 Jul;25(8):1231-40.
The present TNM
classification of ophthalmic malignant tumors (lid cancer, malignant
melanoma of the lid, conjunctival cancer, conjunctival malignant
melanoma, uveal malignant melanoma, retinoblastoma, orbital sarcoma
and lacrimal gland cancer) has been explained according to the UICC in
a book edited by M. H. Harmer and J. A. Oosterhuis. Five studies
concerning the difference in clinical results with and without a
treatment were shown to confirm the meaning and importance of TNM
classification of ophthalmic malignancies.
Melanogenic neuroectodermal tumor of the retina (primary malignant
melanoma of the retina).Arch
Ophthalmol. 1997 Dec;115(12):1581-4
A 35-month-old
girl with leukocoria was clinically diagnosed with unilateral sporadic
retinoblastoma. Macroscopic examination of her enucleated eye
disclosed a white retinal tumor that appeared to be a retinoblastoma.
Histopathologic examination, however, revealed that the tumor was
composed of poorly differentiated neuroblastic cells, larger
spindle-shaped cells, and anaplastic epithelioid cells, which is
inconsistent with retinoblastoma. Immunohistochemical testing
disclosed that the tumor cells were immunoreactive for
melanoma-specific antigen HMB-45, while electron microscopy showed
premelanosomes in the tumor cells, both of which are consistent with
melanogenesis. To our knowledge, such an ocular tumor has not been
reported previously.
Familial risks
for eye melanoma and retinoblastoma: results from the Swedish
Family-Cancer Database.Melanoma
Res. 2006 Apr;16(2):191-5.
No systematic
population-based studies have been conducted on familial eye cancers.
Reliable data on familial risks are important for clinical counselling
and cancer genetics. The current analysis was based on the nation-wide
Swedish Family-Cancer Database on 10.5 million individuals, containing
families with parents and offspring. Cancer data were retrieved from
the Swedish Cancer Registry from the years 1958 to 2002, including
3636 patients with any type of eye cancer. Familial risk for offspring
was defined using the standardized incidence ratio (SIR), adjusted for
many variables. Ocular melanoma was detected in two parent-offspring
pairs, but the SIR of 3.90 was not significant. Parental upper
aerodigestive tract (2.05), left-sided colon (1.83) and male non-medullary
thyroid (6.98) cancers showed an association with ocular melanoma,
albeit some with a borderline significance. The SIR for leukaemia was
increased when parents were diagnosed with eye melanoma. There was no
evidence for the association of ocular melanoma with cutaneous
melanoma. The SIR for ocular melanoma was 1.76 when a sister was
diagnosed with breast cancer, but there was no increase when a mother
was diagnosed with breast cancer. When both a child and the parent
presented with retinoblastoma, the SIR was 900. The parents of
children with retinoblastoma had an excess of small intestinal and
rectal cancers and Hodgkin's disease. The present findings were based
on a limited number of cases, but they display a complex and
heterogeneous pattern of familial associations in ocular melanoma,
including an association with breast cancer through a putative
recessive mechanism. |
