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Disseminated glandular Kaposi sarcoma in a Ugandan child
452
The Journal of P E D I A T R I C S
Disseminated glandular Kaposi sarcoma in a Ugandan child Disseminated glandular Kaposi sarcoma was diagnosed in a 22-month-old Ugandan boy, who presented with large firm lymph nodes in bo)h cervical and inguinal regions and with radiologic evidence of hilar gland enlargement with areas of pulmonary collapse. Biopsy showed the typical histology. Treatment with a 4 day course o[ intravenous nitrogen mustard produced transient slight improvement [ollowed by relapse.
D. B. Jelliffe, M.D., "x"J. Cook, F.R.C.P., and J. N. P. Davies, M.D. *'z K A M P A L A , UGANDA
K A P O S I SARCOZaA is a condition of uncertain etiology, which usually presents with one or more bluish red skin nodules on the legs or, less commonly, the arms. African adult males living in the transSaharan part of the continent are more commonly affected than any other group in the world, as can be judged from the literature from Uganda, 1-a the Congo, 4 and South Africa. ~ At the same time, as noted in recent reviews by Dutz and Stout, G and by Davies and Lothe, a it is recognized that, although older children usually show the same syndrome as adults, younger patients, especially under 6 years of age, frequently have a more fulminating course and a different clinical picture, notably with disseminated lymphadFrom the Departments of Paediatrics and Child Health, Surgery and Pathology, Makerere Medical School, Kampala. ~Address P.O, Box 2072, Kampala, Uganda. *~'Department o] Morbid Anatomy, Postgraduate Medical School, Ducane, Road, London, W. 12, England.
enopathy and sometimes deposits in the eyelids7, s or enlarged salivary glands, occasionally producing the picture of the Mikulicz syndrome. CASE HISTORY
Joseph Okello, a 22-month-old male Itesot, who lived all his life in Kyagwe, Buganda, was admitted to Mulago Hospital or~ March 13, 1961. The mother complained that during the previous month the child had developed gradually increasing swellings on both sides of the neck, accompanied by cough, fever, and loss of weight. Examination. This showed an underweight child (8.5 kilograms) with poor muscle and subcutaneous fat and moderate hypochromotrichia. Mouth breathing and a mild degree of stridor were present. There was marked and visible bilateral enlargement of the lymph nodes in the anterior and posterior triangles of the neck and in the inguinal regions (Fig. 1, A and B). The affected nodes were discrete, mobile, and nontender.
Volume 61 Number 3
They were firm and rubbery in consistency with no fluctuation. A small rounded subcutaneous nodule, about 5 mm. in diameter, was present below the right nipple. This was firm, mobile, and nontender. There appeared to be a possible fullness of the upper eyelids bilaterally although no nodules or infiltration could be felt. Other systems showed no abnormality, save for scattered rhonchi in both sides of the chest. The liver and spleen were not palpable. There was no marked anemia. INVESTIGATIONS
The blood count showed hemoglobin, 10.5 Gin. per cent; white blood cell count, 13,750 per cubic millimeter; lymphocytes, 71 per cent; polymorphonuclear leukocytes, 22 per cent; eosinophils, 2 per cent; and monocytes, 2 per cent. Plasmodium faleiparum was present on the thick blood film. The tuberculin test (Heal) was negative. The x-ray of the chest showed bilateral hilar enlargement with slight deviation of the trachea to the right, pressure on the right main bronchus, and partial pulmonary collapse in both lower lobes (Fig. 2). The radiologic picture was
Disseminated glandular Kaposi sarcoma
453
thought to be related to severe hilar lymphadenopathy. Comment. The main feature of the child's clinical picture was the marked enlargement of lymph nodes in the neck, groins, and hila. A clinical and radiologic presumptive diagnosis of disseminated tuberculous lymphadenopathy was made, this being a not uncommon mode of presentation of tuberculous disease in African children. 9 The negative tuberculin test was considered to result from anergy related to the advanced stage of the disease and to associated poor nutrition. The second differential diagnosis was of malignant lymphoma, the commonest tumor of childhood in Uganda, which also can present with enlargement of lymph nodes. 10 Diagnosis. This was finally made by biopsy of nodes from both neck and groin which showed the histotogic picture of Kaposi sarcoma, that is, the characteristic spindle cells and vascular features, moderate numbers of mitotic figures, some pigment granules, and mononuclear inflammatory cells (Fig. 3). Future progress. The child's malaria was treated with a course of chloroquine, while
B
A Fig. 1. A and B. Kaposi sarcoma in African child in Uganda, showing marked bilateral enlargement of cervical lymph nodes.
4 54
September 1962
]eUiffe, Cook, and Davies
Nine days later, the nodes were possibly somewhat smaller in size and the stridor had subsided. The child had gained ly2 kilograms in weight. However, at the mother's insistence, the patient was discharged on placebo therapy. He reattended after 4 weeks when the lymph nodes had again increased in size and stridor had recurred. The mother refused further treatment and the child's ultimate fate is unknown, although it seems likely that he must have died in the subsequent weeks. DISCUSSION
Fig. 2. Radiograph (A. P.) of chest in child with Kaposi sarcoma, showing increased hilar shadows and scattered opacities due to pulmonary collapse.
Fig. 3. Histology of gland from child with Kaposi sarcoma, showing characteristic spindle cells, pigmerit granules, and mononuclear inflammatory cells. a high protein diet and iron were given also. Tumor chemotherapy was attempted with a 4 day course of nitrogen mustard given by intravenous drip (1 mg. on the first day, and 3 mg. on the last three). There were no obvious side-effects, in particular no pyrexia or leukopenia.
Kaposi sarcoma is unusual in children; in Dutz and Stout's 6 review of 1,256 cases in all age groups from all over the world, only 40 (3.2 per cent) were under 6 years old. Interestingly, 18 of these (45 per cent) had occurred in African children2 In a more recent review from Kampala, Davies and Lothe 3 state that of 281 patients with Kaposi sarcoma recorded in Uganda between 1934 and the middle of 1961 only 9 (4 per cent) were below 12 years of age2 If the present case, with widespread lymphadenopathy (cervical, axillae, groins, hila) and a small skin nodule below the right nipple, is added to this group, none of the 10 affected children showed the classical clinical picture found in the African adult male--that is, the development of multiple nodules on the limbs, especially the legs. The commonest way for the condition to develop, as with the present child, is with lymph node enlargement (70 per cent), always in the cervical region, and usually in the groins and axillae as well. Usually associated with this main feature, but sometimes alone or in various combinations, may be found scanty, often anomalously sited skin nodules, deposits in the eyelids, and enlargement of the salivary glands. A Mikulicz syndrome may result. Kaposi sarcoma in its typical form in adults has been treated in Kampala by Cook with the use of intra-arterial or, if the lesions were widely scattered, intravenous injections of nitrogen mustard. Regression of skin nodules has occurred with both methods
Volume 61
Number 3
of a d m i n i s t r a t i o n , t h o u g h intravenous nitrogen m u s t a r d in conventional doses usually only gives t e m p o r a r y or p a r t i a l palliation. 2 T r e a t m e n t with n i t r o g e n m u s t a r d h a d not been a t t e m p t e d previously in the dissemin a t e d g l a n d u l a r c h i l d h o o d form of the condition. I n the p r e s e n t patient, a 4 d a y intravenous course was followed b y transient, slight i m p r o v e m e n t , a n d then a relapse. 'As Davies a n d L o t h e r e m a r k , the childh o o d v a r i a n t of K a p o s i s a r c o m a is Considered to be f u l m i n a n t in course. I n U g a n d a , because of the e x t r e m e difficulty in followup after discharge f r o m the hospital, no definite i n f o r m a t i o n is k n o w n as to prognosis o r distribution of lesions t h r o u g h the b o d y as would be revealed by autopsy. H o w ever, as with the present child, it seems t h a t survival for m o r e t h a n a few months is unlikely.
Disseminated glandular Kaposi sarcoma
455
REFERENCES
1. Davies, J. N. P.: Cancer in Africa, in Collins, D. H., editor: Modern trends in pathology, London, 1959, Butterworth & Co., Ltd. 2. Cook, J.: Kaposi's sarcoma treated with nitrogen mustard, Lancet 1: 25, 1959. 3. Davies, J. N. P., and Lothe, F.: Kaposi sarcoma in African children, Acta Unio internat. contra cancrum 18: 394, 1962. 4. Thiys, A.: L'Angiosarcomatose de Kaposi au Congo Belge et au Ruanda-Urundi, Ann. Soe. belge m~d. Trop. 37: 295, 1957. 5. Murray, J. F.: Kaposi's haemangiosarcoma in the Bantu, Leech 22: 3, 1952. 6. Dutz, W., and Stout, A. D.: Kaposi's sarcoma in infants and children, Cancer 13: 684, 1960. 7. McLaren, D. S.: Kaposi's sarcoma of the eyelids of an African child, A. M. A. Arch. Ophth. 63: 859, 1960. 8. Sacks, I.: Kaposi's disease manifesting in the eye, Brit. J. Ophth. 40: 574, 1956. 9. Jelliffe, D. B.: Disseminated tuberculous lymphadenitis in Nigerian children, J. Trop. Med. 52: 251, 1949. 10. O'Conor, G. I., and Davies, J. N. P.: Malignant tumors in African children, J. PEDIAT. 56: 526, 1960.
The Journal of P E D I A T R I C S
Disseminated glandular Kaposi sarcoma in a Ugandan child Disseminated glandular Kaposi sarcoma was diagnosed in a 22-month-old Ugandan boy, who presented with large firm lymph nodes in bo)h cervical and inguinal regions and with radiologic evidence of hilar gland enlargement with areas of pulmonary collapse. Biopsy showed the typical histology. Treatment with a 4 day course o[ intravenous nitrogen mustard produced transient slight improvement [ollowed by relapse.
D. B. Jelliffe, M.D., "x"J. Cook, F.R.C.P., and J. N. P. Davies, M.D. *'z K A M P A L A , UGANDA
K A P O S I SARCOZaA is a condition of uncertain etiology, which usually presents with one or more bluish red skin nodules on the legs or, less commonly, the arms. African adult males living in the transSaharan part of the continent are more commonly affected than any other group in the world, as can be judged from the literature from Uganda, 1-a the Congo, 4 and South Africa. ~ At the same time, as noted in recent reviews by Dutz and Stout, G and by Davies and Lothe, a it is recognized that, although older children usually show the same syndrome as adults, younger patients, especially under 6 years of age, frequently have a more fulminating course and a different clinical picture, notably with disseminated lymphadFrom the Departments of Paediatrics and Child Health, Surgery and Pathology, Makerere Medical School, Kampala. ~Address P.O, Box 2072, Kampala, Uganda. *~'Department o] Morbid Anatomy, Postgraduate Medical School, Ducane, Road, London, W. 12, England.
enopathy and sometimes deposits in the eyelids7, s or enlarged salivary glands, occasionally producing the picture of the Mikulicz syndrome. CASE HISTORY
Joseph Okello, a 22-month-old male Itesot, who lived all his life in Kyagwe, Buganda, was admitted to Mulago Hospital or~ March 13, 1961. The mother complained that during the previous month the child had developed gradually increasing swellings on both sides of the neck, accompanied by cough, fever, and loss of weight. Examination. This showed an underweight child (8.5 kilograms) with poor muscle and subcutaneous fat and moderate hypochromotrichia. Mouth breathing and a mild degree of stridor were present. There was marked and visible bilateral enlargement of the lymph nodes in the anterior and posterior triangles of the neck and in the inguinal regions (Fig. 1, A and B). The affected nodes were discrete, mobile, and nontender.
Volume 61 Number 3
They were firm and rubbery in consistency with no fluctuation. A small rounded subcutaneous nodule, about 5 mm. in diameter, was present below the right nipple. This was firm, mobile, and nontender. There appeared to be a possible fullness of the upper eyelids bilaterally although no nodules or infiltration could be felt. Other systems showed no abnormality, save for scattered rhonchi in both sides of the chest. The liver and spleen were not palpable. There was no marked anemia. INVESTIGATIONS
The blood count showed hemoglobin, 10.5 Gin. per cent; white blood cell count, 13,750 per cubic millimeter; lymphocytes, 71 per cent; polymorphonuclear leukocytes, 22 per cent; eosinophils, 2 per cent; and monocytes, 2 per cent. Plasmodium faleiparum was present on the thick blood film. The tuberculin test (Heal) was negative. The x-ray of the chest showed bilateral hilar enlargement with slight deviation of the trachea to the right, pressure on the right main bronchus, and partial pulmonary collapse in both lower lobes (Fig. 2). The radiologic picture was
Disseminated glandular Kaposi sarcoma
453
thought to be related to severe hilar lymphadenopathy. Comment. The main feature of the child's clinical picture was the marked enlargement of lymph nodes in the neck, groins, and hila. A clinical and radiologic presumptive diagnosis of disseminated tuberculous lymphadenopathy was made, this being a not uncommon mode of presentation of tuberculous disease in African children. 9 The negative tuberculin test was considered to result from anergy related to the advanced stage of the disease and to associated poor nutrition. The second differential diagnosis was of malignant lymphoma, the commonest tumor of childhood in Uganda, which also can present with enlargement of lymph nodes. 10 Diagnosis. This was finally made by biopsy of nodes from both neck and groin which showed the histotogic picture of Kaposi sarcoma, that is, the characteristic spindle cells and vascular features, moderate numbers of mitotic figures, some pigment granules, and mononuclear inflammatory cells (Fig. 3). Future progress. The child's malaria was treated with a course of chloroquine, while
B
A Fig. 1. A and B. Kaposi sarcoma in African child in Uganda, showing marked bilateral enlargement of cervical lymph nodes.
4 54
September 1962
]eUiffe, Cook, and Davies
Nine days later, the nodes were possibly somewhat smaller in size and the stridor had subsided. The child had gained ly2 kilograms in weight. However, at the mother's insistence, the patient was discharged on placebo therapy. He reattended after 4 weeks when the lymph nodes had again increased in size and stridor had recurred. The mother refused further treatment and the child's ultimate fate is unknown, although it seems likely that he must have died in the subsequent weeks. DISCUSSION
Fig. 2. Radiograph (A. P.) of chest in child with Kaposi sarcoma, showing increased hilar shadows and scattered opacities due to pulmonary collapse.
Fig. 3. Histology of gland from child with Kaposi sarcoma, showing characteristic spindle cells, pigmerit granules, and mononuclear inflammatory cells. a high protein diet and iron were given also. Tumor chemotherapy was attempted with a 4 day course of nitrogen mustard given by intravenous drip (1 mg. on the first day, and 3 mg. on the last three). There were no obvious side-effects, in particular no pyrexia or leukopenia.
Kaposi sarcoma is unusual in children; in Dutz and Stout's 6 review of 1,256 cases in all age groups from all over the world, only 40 (3.2 per cent) were under 6 years old. Interestingly, 18 of these (45 per cent) had occurred in African children2 In a more recent review from Kampala, Davies and Lothe 3 state that of 281 patients with Kaposi sarcoma recorded in Uganda between 1934 and the middle of 1961 only 9 (4 per cent) were below 12 years of age2 If the present case, with widespread lymphadenopathy (cervical, axillae, groins, hila) and a small skin nodule below the right nipple, is added to this group, none of the 10 affected children showed the classical clinical picture found in the African adult male--that is, the development of multiple nodules on the limbs, especially the legs. The commonest way for the condition to develop, as with the present child, is with lymph node enlargement (70 per cent), always in the cervical region, and usually in the groins and axillae as well. Usually associated with this main feature, but sometimes alone or in various combinations, may be found scanty, often anomalously sited skin nodules, deposits in the eyelids, and enlargement of the salivary glands. A Mikulicz syndrome may result. Kaposi sarcoma in its typical form in adults has been treated in Kampala by Cook with the use of intra-arterial or, if the lesions were widely scattered, intravenous injections of nitrogen mustard. Regression of skin nodules has occurred with both methods
Volume 61
Number 3
of a d m i n i s t r a t i o n , t h o u g h intravenous nitrogen m u s t a r d in conventional doses usually only gives t e m p o r a r y or p a r t i a l palliation. 2 T r e a t m e n t with n i t r o g e n m u s t a r d h a d not been a t t e m p t e d previously in the dissemin a t e d g l a n d u l a r c h i l d h o o d form of the condition. I n the p r e s e n t patient, a 4 d a y intravenous course was followed b y transient, slight i m p r o v e m e n t , a n d then a relapse. 'As Davies a n d L o t h e r e m a r k , the childh o o d v a r i a n t of K a p o s i s a r c o m a is Considered to be f u l m i n a n t in course. I n U g a n d a , because of the e x t r e m e difficulty in followup after discharge f r o m the hospital, no definite i n f o r m a t i o n is k n o w n as to prognosis o r distribution of lesions t h r o u g h the b o d y as would be revealed by autopsy. H o w ever, as with the present child, it seems t h a t survival for m o r e t h a n a few months is unlikely.
Disseminated glandular Kaposi sarcoma
455
REFERENCES
1. Davies, J. N. P.: Cancer in Africa, in Collins, D. H., editor: Modern trends in pathology, London, 1959, Butterworth & Co., Ltd. 2. Cook, J.: Kaposi's sarcoma treated with nitrogen mustard, Lancet 1: 25, 1959. 3. Davies, J. N. P., and Lothe, F.: Kaposi sarcoma in African children, Acta Unio internat. contra cancrum 18: 394, 1962. 4. Thiys, A.: L'Angiosarcomatose de Kaposi au Congo Belge et au Ruanda-Urundi, Ann. Soe. belge m~d. Trop. 37: 295, 1957. 5. Murray, J. F.: Kaposi's haemangiosarcoma in the Bantu, Leech 22: 3, 1952. 6. Dutz, W., and Stout, A. D.: Kaposi's sarcoma in infants and children, Cancer 13: 684, 1960. 7. McLaren, D. S.: Kaposi's sarcoma of the eyelids of an African child, A. M. A. Arch. Ophth. 63: 859, 1960. 8. Sacks, I.: Kaposi's disease manifesting in the eye, Brit. J. Ophth. 40: 574, 1956. 9. Jelliffe, D. B.: Disseminated tuberculous lymphadenitis in Nigerian children, J. Trop. Med. 52: 251, 1949. 10. O'Conor, G. I., and Davies, J. N. P.: Malignant tumors in African children, J. PEDIAT. 56: 526, 1960.