Year : 1957 | Volume
: 5 | Issue : 3 | Page : 55--59
Ocular metastases in hutchison-peppers' syndrome
BK Das Gupta
Nilratan Sircar Hospital, Calcutta, India
B K Das Gupta
Nilratan Sircar Hospital, Calcutta
|How to cite this article:|
Das Gupta B K. Ocular metastases in hutchison-peppers' syndrome.Indian J Ophthalmol 1957;5:55-59
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Das Gupta B K. Ocular metastases in hutchison-peppers' syndrome. Indian J Ophthalmol [serial online] 1957 [cited 2020 Jul 12 ];5:55-59
Available from: http://www.ijo.in/text.asp?1957/5/3/55/40741
The purpose of this paper is to report on the involvement of the various structures of the eye in a case of Hutchison-Peppers' syndrome (Neuroblastoma of the adrenal gland with involvement of the liver and the orbit). The report seems justified in view of the fact that no reference could be found in the literature where metastatic deposits were reported in the ocular tissues.
The tumours of the adrenal gland consisting of immature pleuri-potential undifferentiated neuroblasts, which are now termed neuroblastoma or sympathicoblastoma, were first described as gliomata by Virchow (1864). Subsequently they were grouped as "round-celled sarcomas" of the adrenal gland or "Iymphosarcoma". A review of the previous records shows that many of such recorded tumours are of the nature of neuroblastoma.
Marchand (1891) noted the resemblance of this tumour to that of the developing central nervous system. Pepper (1901) described a tumour in a six-week old infant with involvement of the liver and right adrenal gland which he called "Iymphosarcoma". From his description originated the so-called "Pepper's syndrome" the syndrome of massive hepatic metastases from adrenal neuroblastoma. Hutchison (1907) reported ten cases of adrenal tumour with metastases to bones of the skull - a syndrome which, in later years, was named after him. These types (Pepper's and Hutchison's) are no longer accepted, since Farber (1940) and Karsner (1942) showed that the distribution of metastatic deposits bears no relation either to the anatomical location of the primary tumour or to its cytological character.
Marchand (1891) was the first to recognise the true nerve-cell character of the tumour. He noted the likeness of the tissue of the tumour to that of the developing sympathetic ganglia. Wiesel (1905) identified the rosettes of neuroblastoma with those of embryonic adrenal medulla or sympathetic ganglia. Wright (1910). by comparing the rosettes and parallel bundles of fibrils in the tumours, finally established the identity of the neuroblastoma as arising from embryonic adrenal medulla and sympathetic system.
Origin. It is now established that the neuroblastoma of the adrenal gland arises from the same cells as does the medulla of the adrenal gland as well as portions of the sympathetic nervous system. This tumour, arising from un-
differentiated migratory cells, may have its origin in widely varying parts of the body. The adrenal gland accounts for one-third of all cases, the abdominal and pelvic sympathetic chains and associated ganglia account for another one-third while the remaining one-third arises from cervical and thoracic parts of the sympathetic chain.
Histology. Microscopic examination of these tumours reveals a highly cellular structure consisting of closely aggregated small rounded or polyhedral cells with scanty ill-defined cytoplasm and each with a single spherical or slightly ovoid nucleus. A few larger rounded cells with vesicular nuclei and some pear-shaped cells resembling spongioblasts can be seen. Mitotic figures are plenty. All the cells give off delicate processes which unite to form a fibrillar syncytium. Two characteristic features of this tumour are considered to be diagnostic arrangement of groups of cells in rosette formations and development of neurofibrils.
Metastases. Neuroblastomas are highly malignant growths with the potentiality of wide and extensive dissemination. Metastases usually takes place from the primary site in one or all of three ways - by direct extension, through lymphatics or blood vessels. In a great majority of casts the primary tumour is so small and symptomless that attention is only drawn to the condition because of massive hepatic enlargement or metastatic deposits in bones. Some of these metastatic syndromes are so characteristic that they have been given eponyms e.g. Pepper's and Hutchison's syndrome.
Hutchison's syndrome is of great interest to ophthalmologists. In this syndrome main attention is drawn to the deposits in the skull and orbit. Agerty, Berthmaier and Fisher (1938) collected 275 cases with orbital involvement, Guiber (1942) later increased the figure to 291. In a recent survey Portray and Danis (1949) brought the total to 300 cases from the world literature.
Metastases in the orbit . Clark (1939) estimated that approximately 25% of neuroblastoma showed metastatic deposits in the orbit and skull and the earliest manifestations were ecchymosis of the lids or exophthalmos or both. The condition was usually unilateral and occurred in children from 1 to 15 years of age. X-ray of the skull revealed a fine granular type of osteoporosis with resorption. The growths were situated on the inner and outer tables of the skull.
Metastases in other tissues such as lymph glands, lungs, kidney, spleen, pancreas and ovary are quite frequently found.
Spontaneous retrogression. While it is true that neoplasms, benign or malignant, grow without interruption and indefinitely, spontaneous cessation of growth and retrogressive changes are sometimes seen, more frequently in benign than in malignant growths. Very rare cases of spontaneous disappearance of proven malignant growt4 such as mammary carcinoma, ovarian growths and malignant melanoma have been reported. Duke-Elder cites II cases of spontaneous retrogression in retinoblastoma, a histologically allied tumour to neuroblastorna of the adrenal gland. Spontaneous retrogression of neuroblastoma of the adrenal have been reported in 3 cases without any specific treatment by Gross, after X-ray therapy by Lehman and by transformation into ganglioneuroma by Cushing and Woolbach.
Neuroblastoma of the adrenal is primarily a condition of infancy and childhood, though it has been known to occur in adults as well. It occurs equally in two sexes.
Scott et al (1933) showed that in 50% of the reported cases the patients had been less than 2 years old and in at least 75% less than 4 years. Gross (1953) showed from his collection that 30% occur in the first year of life, 80% within the first 5 years and only 20% occur beyond that age.
As the primary tumour may arise from many different sites the presenting complaint is necessarily variable, and unless one is on the look-out : the diagnosis may be entirely misplaced. The initial complaint may be abdominal swelling, proptosis, ecchymosis of the lids, anuria, nervous disorder or bony swellings. General non-specific findings of increasing pallor, anemia, progressive loss of weight and listlessness are usually present. The primary tumour may not be evident and the symptoms and signs may result from metastases alone. Headache. nausea, vomiting, papilledema, separation of the suture lines and hydrocephalus may result from increased intracranial pressure due to metastases in the skull; and proptosis, ecchymosis of the upper eye-lid and optic atrophy may be seen with retrobulbar involvement.
Straight X-ray of the abdomen may show an area of increased density in the region of the tumour. Radiological evidence of calcification above kidney region is an extremely important clinical aid in diagnosis. X-rays of bones may show radiating spicules or bony resorption an evidence of skeletal metastases.
Clinically Hutchison's tumour appears in the orbit of infants and young children, appearing first on the side corresponding with the affected adrenal. The orbital involvement, however, may be bilateral. It usually occurs in children between 2 to 3 years and is seldom seen above 10. The first localising sign of orbital involvement are ecchymosis of the lids and proptosis. The proptosis may be very rapid and extreme and the whole of the orbital cavity may be filled up with the tumour mass. It is very important to keep in mind that orbital involvement may be the sole evidence of the parent tumour, which may only be discovered at autopsy and sometimes not even then.
Prognosis. As a general rule prognosis is very poor once a diagnosis of neuroblastoma is made. Average duration of life has been differently estimated by various authors---8 to 9 weeks by Blacklock; 6 months by Lewis and others.
There are increasing evidences from reports of Roussy. Wyatt and Farber ; Wittenborg and others to moderate a little about the hopeless prognosis of the previous writers. Gross comparing his collection of cases, the first group before 1940 and the second group from 1940 through 1947, found an encouraging result in the three-year from 19% to 29% an increase which he attributes to improved operative technique and more thorough use of x-rays irradiation.
A boy of 12 years was brought to the ophthalmic out-patients' department of Nilratan Sircar Hospital, Calcutta, on May 2nd, 1956, for pain, proptosis and chemosis of the right eye and a big swelling on the left side of the face. History revealed that about a month back the swelling on the jaw appeared, which had been on the increase since then. The ocular condition developed about 10 days after the swelling on the jaw had appeared.
Examination of the right eye revealed a hard fixed swelling on the lateral aspect of the superior orbital margin encroaching on to the upper eye lid; conjunctiva was slightly chemosed at the upper part; cornea, pupil, lens and intraocular pressure were normal; proptosis was directed forwards about 4 mm.; ocular movements were not limited except for a little restriction upwards; vision was 2/60: ophthalmoscopic examination revealed a slight edema and hyperemia of the disc and congestion of the retinal veins.
Left Eye:- nothing abnormal was detected. Vison was 6/6. Nothing abnormal was detected in the fundus.
Swelling on the jaw on the left side . A hard swelling about the size of an orange with well-defined margins overlying the parotid region was seen. The skin was free. but the swelling was fixed to the bone. There was no difficulty in opening and closing the mouth; articulation and speech were impaired.
General examination . A fulness was noted in the right hypochondrium and epigastric regions. Liver was enlarged four fingers and was firm in consistency. A firm lump was palpated in the epigastric region and a firm rounded swelling in the right renal region. The case was diagnosed as Hutchison-Peppers' syndrome and was admitted into the hospital on the same day.
Progress of the case . From the day of his admission onwards the condition of the patient was rapidly getting worse. On May 7th, 1956, signs of portal hypertension appeared. The liver mass, swelling on the jaw and the lump in the right renal region together with the proptosis of the right eye were gradually increasing. Lagophthalmos, due to proptosis, caused ulceration of the cornea, which eventually sloughed out completely with extrusion of the lens. The patient became very anemic and edema of the feet developed.
On 14th June, 1956, a palpable swelling 4 cm. x 3.5 cm. on the left side of the vertebral column over the ribs in the mid-dorsal region developed. The deep cervical glands on the left side of the neck were now all enlarged and the patient could hardly open his mouth. He was placed entirely on liquids.
Regression. On June 26th. 1956, a sudden change in the whole picture was noticed: the lump on the back disappeared. the lumps on the jaw and liver regions were felt to become soft and regress to a certain extent; the enlarged hard cervical glands had become soft: the proptosis was getting reduced and the patient now started talking and taking his meals. From now onwards the patient was showing signs of general improvement; he was gaining strength and was able to move out of his bed; edema of the feet disappeared; swellings on the jaw. liver and kidney regions regressed totally except a small nodule in the cheek.
1. Straight X-ray of the left mandible showed bony resorption. No change was found in the bony wall of the right orbit.
2. Perirenal pneumography showed a tumour mass in the right kidney region. No calcification was seen.
3. Presacral pneumography with intravenous pyelography showed kidney free of the tumour mass and calyces normal.
4. Biopsy of the mass on the jaw showed closely aggregated small rounded cells with deep staining nuclei and very scanty ill-defined cytoplasm. Miotic figures were plenty. Groups of cells in rosette-formations were seen.
5. The right eye was enucleated on 13th July, 1956. The iris, ciliary body, ciliary processes, choroid. sclera and optic nerve were found to be infiltrated with the same type of cells as was seen in the histological section of the soft tissues of the jaw. In addition larger rounded cells with vesicular nuclei and pear-shaped cells with tail-processes were seen. Mitotic figures were plenty and rosette-formations were seen in places.
6. Blood examination showed moderate degree of anemia.
7. Total quantity of urine in 24 hours - 1560 ml.
17-Ketosteroid 1.6 mgm. in 24 hours
Ketogenic steroid 2.24 mgm. in 24 hours.
Subsequent development. The patient was progressing fairly well. On September 22nd. 1956, a small nodule was detected on the left side of the cheek. Two other nodules appeared some days later, and on October 29th. 1956. a biopsy was done which showed the same malignant characteristics. Thereafter, the patient developed multiple swellings all over the body. A lump was detected on the medial side of the vertebral border of the right scapula and a fairly big swelling at the posteromedial aspect of the left thigh in the muscle. Towards the end of November 1956 an enormous swelling developed on the left side of the face extending over the nape of the neck and the patient's condition deteriorated gradually. He had now no control over the sphincter and there was constant dribbling of urine. Edema now extended to the whole of the face and the scalp. Lately he developed basal congestion on both sides; he became cyanosed and dyspneic and he expired on January 12th. 1957.
A report is given of a case of Hutchison-Peppers' syndrome on the right side, in which spontaneous recovery took place for a period of 3 months after which the pathology shifted more to the left side, the condition deteriorated and the patient expired six months after admission to the hospital.
Relevant literature on the condition is presented.