Extranodal lymphoma of the tongue, a very rare entity-report of two cases with literature review.

BACKGROUND: Lymphomas are malignant neoplasms of the lymphocyte cell lines affecting the lymph nodes, spleen and other nonhemopoietic tissues. Of the extranodal lymphomas found in the head and neck region, 3-5% of malignant lymphomas arise in the oral and paraoral region, mainly from Waldeyer's ring. The involvement of the base of the tongue is extremely rare. SUMMARISED CASE: Case 1: 64 year old female who presented initially with an enlarged occipital lymph node which gradually became generalized cervical lymphadenopathy with initial histology confirmed reactive lymphoid hyperplasia. Biopsy of left postero-lateral tongue lesion eventually showed high grade T cell lymphoma. Case 2: 85 year old male presented with history of dysphagia for one year who was found to have a lesion extending from his base of tongue into the nasopharynx. Histology showed a diffuse B cell lymphoma. DISCUSSION: Both patients were noted to have lesion of the tongue, but tongue lesions are noted in the literature to be extremely rare. When tongue lymphomas do occur, most are of B-cell origin; the diffuse large-cell variety is the most common. Extranodal lymphomas of the T cell phenotype tend more to be sinonasal in origin than of the tongue, with T cell lymphomas of the tongue being even rarer than B cell lymphomas. CONCLUSION: With regards to tumours arising in the tongue, squamous cell carcinomas are still classified as the most common. Lymphomas however, should still be kept in consideration as a differential diagnosis with regards to lesions arising from this site.

The distribution of presumptive thoracic paraganglionic tissue in the common marmoset (Callithrix jacchus).

The aortic-pulmonary regions (APR) of seven adult marmosets (Callithrix jacchus) and the region of the right subclavian artery of a further three marmosets were diffusion-fixed with 10% buffered formol-saline solution. In both regions serial 5 microm sections were cut and stained by the Martius yellow, brilliant crystal scarlet and soluble blue method. Presumptive thoracic paraganglionic (PTP) tissue was only observed in the APR. PTP tissue was composed of small groups of cells that varied in size and number. The distribution of the groups of cells was extremely variable, so much so that it would be misleading to attempt to classify their position; they were not circumscribed by a connective tissue capsule, but were always related to the thoracic branches of the left vagus nerve. The cells lay in loose areolar tissue characteristic of this part of the mediastinum and received their blood supply from small adjacent connective tissue arterioles. Unlike the paraganglionic tissue found in the carotid body the cells in the thorax did not appear to have a profuse capillary blood supply. There was, however, a close cellular-neural relationship. The cells, 10-15 microm in diameter, were oval or rounded in appearance and possessed a central nucleus and clear cytoplasm. No evidence was found that these cells possessed a 'companion' cell reminiscent of the arrangement of type 1 and type 2 cells in the carotid body. In conclusion, we found evidence of presumed paraganglionic tissue in the APR of the marmoset which, however, did not show the characteristic histological features of the aortic body chemoreceptors that have been described in some non-primate mammals. A survey of the mediastina of other non-human primates is required to establish whether this finding is atypical for these animals.

The distribution of presumptive thoracic paraganglionic tissue in the common marmoset (Callithrix jacchus)

The aortic-pulmonary regions (APR) of seven adult marmosets (Callithrix jacchus) and the region of the right subclavian artery of a further three marmosets were diffusion-fixed with 10 percent buffered formol-saline solution. In both regions serial 5-æm sections were cut and stained by the Martius yellow, brilliant crystal scarlet and soluble blue method. Presumptive thoracic paraganglionic (PTP) tissue was only observed in the APR. PTP tissue was composed of small groups of cells that varied in size and number. The distribution of the groups of cells was extremely variable, so much so that it would be misleading to attempt to classify their position; they were not circumscribed by a connective tissue capsule, but were always related to the thoracic branches of the left vagus nerve. The cells lay in loose areolar tissue characteristic of this part of the mediastinum and received their blood supply from small adjacent connective tissue arterioles. Unlike the paraganglionic tissue found in the carotid body the cells in the thorax did not appear to have a profuse capillary blood supply. There was, however, a close cellular-neural relationship. The cells, 10-15 æm in diameter, were oval or rounded in appearance and possessed a central nucleus and clear cytoplasm. No evidence was found that these cells possessed a 'companion' cell reminiscent of the arrangement of type 1 and type 2 cells in the carotid body. In conclusion, we found evidence of presumed paraganglionic tissue in the APR of the marmoset which, however, did not show the characteristic histological features of the aortic body chemoreceptors that have been described in some non-primate mammals. A survey of the mediastina of other non-human primates is required to establish whether this finding is atypical for these animals

Quantitative studies of the vasculature of the carotid body in the chronically hypoxic rat.

The carotid bodies of rats made chronically hypoxic by breathing 12% O2 in a normobaric chamber (inspired PO2 91 mmHg) were compared with those of controls. Serial 5-microm sections of the organs were examined using an interactive image analysis system. The total volume of the carotid bodies was increased by 64%. The total vascular volume rose by 103% and was likely due to an increase in size of the large vessels (>12 microm lumen diameter) because the small vessel (5-12 microm lumen diameter) volume did not increase significantly while the small vessel density tended to decrease. The extravascular volume was increased by 57%. Expressed as a percentage of the total volume of the organ, the total vascular volume did not change, but the small vessel volume was significantly decreased from 7.83 to 6.06%. The large vessel volume must therefore have been increased. The proportion occupied by the extravascular volume was virtually unchanged (84 vs 82%). In accordance with these findings, the small vessel endothelial surface area per unit carotid body volume was diminished from 95.2 to 76.5 mm-1, while the extravascular area per small vessel was increased from 493 to 641 microm(2) or by 30%. In conclusion, the enlargement of the carotid body in chronic hypoxia is most likely due to an increase in total vascular volume, mainly involving the "large" vessels, and to an increase in extravascular volume. This is in contrast to our previously published findings indicating that in the spontaneous insulin-dependent diabetic rat the enlargement of the carotid body is due solely to an increase in extravascular volume.

The carotid body of the spontaneous insulin-dependent diabetic rat.

The carotid bodies from adult spontaneous insulin-dependent diabetic rats (strain BB/S) were perfusion-fixed at normal arterial blood pressure with 3% phosphate-buffered glutaraldehyde and compared with the organs from control rats (strain BB/Sc) prepared in the same way. Serial 5-micron sections were cut, stained, and using an interactive image analysis system, were analysed to determine the volumes of the carotid body and its vascular and extravascular compartments. There was no evidence of systemic arterial disease in the carotid stem arteries in either group of animals, and the microvasculature of the organs appeared normal by light microscopy. The volume of the carotid body was unchanged 3 months after the onset of diabetes but was increased at 6 months. The total vascular volume of the organ was unchanged, but the volume of the small vessels (5-12 microns) was increased. In the control group the small vessels comprised 5% of the total volume of the carotid body, or about 44% of the vascular compartment. The percentage of small vessels increased at 3 months in the diabetic group, but had returned to normal at 6 months. The extravascular volume followed the same pattern as the total carotid body volume and so did not change appreciably when expressed as a percentage of the total volume of the organ. The increase in size of the carotid body in diabetic rats is due, therefore, to an augmented extravascular volume. In one diabetic specimen the carotid sinus nerve showed signs of diabetic neuropathy, axonal swelling and intramyelinic oedema. The clinical implications of these results are discussed.

Immunoablation does not delay the neurologic progression of X-linked adrenoleukodystrophy.

We report the results of a near total myeloablation in preparation for bone marrow transplantation in a boy with minimal symptoms of X-linked adrenoleukodystrophy. Severe cerebral X-linked adrenoleukodystrophy developed in the patient after failure of bone marrow transplantation. This experience suggests that immunotherapy alone is not responsible for the improvement observed in some patients with X-ALD after BMT.

Sexual dimorphism in interhemispheric relations: anatomical-behavioral convergence.

An embryogenetic hypothesis states that hemispheric specialization is inversely related to callosal connectivity (Geschwind and Galaburda, 1985). We tested this hypothesis (i) anatomically by relating postmortem planum temporale asymmetry to regional callosal morphology and (ii) behaviorally by relating the right visual field advantage in a lateralized lexical decision task with associative primes to regional callosal morphometry using magnetic resonance imaging (MRI). The postmortem study showed a significant negative correlation between planum temporale asymmetry and the number of small diameter fibers in the isthmus of the corpus callosum, but only for males. The MRI study showed a significant negative correlation between the right visual hemifield advantage for associated words and the cross section size of the isthmus of the corpus callosum, but again only in males. There was no sex difference in either the anatomical asymmetry, the behavioral asymmetry, or the callosal morphology. These convergent results suggest that there is a sexual dimorphism in interhemispheric relations in humans.

Sexual dimorphism in interhemispheric relations: anatomical-behavioral convergencea

An embryogenetic hypothesis states that hemispheric specialization is inversely related to callosal connectivity (Geschwind and Galaburda, 1985). We tested this hypothesis (i) anatomically by relating postmortem planum temporale asymmetry to regional callosal morphology and (ii) behaviorally by relating the right visual field advantage in a lateralized lexical decision task with associative primes to regional callosal morphometry using magnetic resonance imaging (MRI). The postmortem study showed a significant negative correlation between planum temporale asymmetry and the number of small diameter fibers in the isthmus of the corpus callosum, but only for males. The MRI study showed a significant negative correlation between the right visual hemifield advantage for associated words and the cross section size of the isthmus of the corpus callosum, but again only in males. There was no sex difference in either the anatomical asymmetry, the behavioral asymmetry, or the callosal morphology. These convergent results suggest that there is a sexual dimorphism in interhemispheric relations in humans

Pancreatitis in patients with organic acidemias.

STUDY OBJECTIVE: The discovery of pancreatitis in two children with methylmalonic acidemia led us to review the experience with pancreatitis in a large number of patients with organic acidemias to determine whether pancreatitis is an important complication of these disorders. DESIGN: Case series. SETTING: Pediatric metabolism services at five tertiary care centers. PATIENTS: Records of all patients with organic acidemias followed at the five institutions during the past 10 years were reviewed. Pancreatitis was recognized by symptoms and laboratory findings and confirmed by imaging studies, surgery, or autopsy. At three institutions all cases of pancreatitis in children younger than 10 years were reviewed. MEASUREMENTS AND RESULTS: Nine children with pancreatitis (seven with acute and two with chronic cases) were identified among 108 children with branched-chain organic acidemias. They ranged in age from 13 months to 9 years. Five had methylmalonic acidemia, three had isovaleric acidemia, and one had maple syrup urine disease. There were three deaths; acute hemorrhagic pancreatitis occurred in two children, and chronic pancreatitis was found at autopsy in a third. All three patients with isovaleric acidemia and pancreatitis were identified after the occurrence of pancreatitis. The survey of pancreatitis at three institutions found 57 other patients (none with an inborn error) in addition to three patients with inborn errors included in this study. CONCLUSIONS: Acute or chronic pancreatitis may complicate branched-chain organic acidemias and must be considered in the assessment of patients with these disorders who have acute clinical deterioration and vomiting, abdominal pain, encephalopathy or shock, or milder symptoms. Conversely, an inborn error of organic acid metabolism should be considered in children with pancreatitis of unknown origin.

Copper-histidine therapy for Menkes disease.

Menkes disease is an X-linked genetic disorder of copper transport that results in death from severe progressive neurodegeneration by the age of 3 years. We report here our 17 years' experience with the treatment of Menkes disease with subcutaneous administration of copper-histidine. Two patients (16 and 6 years of age) whose therapy was begun within 1 month of birth have done well neurologically. The other five patients have done poorly despite treatment initiated at 2 to 7 months of age. Copper-histidine therapy may be an effective treatment if started early.

Atypical nonketotic hyperglycinemia confirmed by assay of the glycine cleavage system in lymphoblasts.

A 13-year-old girl with early-onset, mild, slowly progressive mental retardation caused by nonketotic hyperglycinemia is described. The plasma and cerebrospinal fluid glycine concentrations were elevated, but the cerebrospinal fluid/plasma glycine ratio was only mildly elevated. The diagnosis was confirmed by demonstration of a defect in the activity of the glycine cleavage system in cultured lymphoblasts.

Ornithine transcarbamylase deficiency presenting with strokelike episodes.

A girl with ornithine transcarbamylase deficiency had a history of recurrent strokelike episodes. The differential diagnosis of unexplained stroke should include primary urea cycle defects.

Neurological/neurosurgical referrals from Queen Elizabeth Hospital, Barbados - abstract

Neurological/neurosurgical cases (NNC) requiring overseas investigation and treatment have become an increasingly difficult problem for Barbados in recent years. All overseas referrals from Queen Elizabeth Hospital (QEH) between January 1, 1984 and November 1, 1987 have been reviewed. Ninety one NNCs were referred; there were 48 males and 43 females, aged 32ñ7 years (meanñSD, range 4 months to 69 years). Only five were older than 60 years. Eleven centres were used. In 1984 referrals to the University Hospital of the West Indies (UHWI), Jamaica, ceased; for the next two years, most NNCs were sent to Port-of-Spain, Trinidad. Several factors, chiefly bureaucratic delays in approval for transfer, forced new lines of referral, outside of the Caribbean. In 1986 and 1987, 63 percent of new cases were referred to North America, chiefly to Mount Sinai Hospital New York. This was made possible by a Government aid scheme. During 1984 - 1986, there were non-surgical than surgical cases. In January 1987, a CAT Scan was installed at QEH and there was a marked fall (by over 60 percent) in referrals in 1987. Each of the 13 NNCs referred in this year was correctly diagnosed as needing surgery. This represents a huge savings in non-surgical referrals for investigation. Lack of neurological/neurosurgical facilities for investigation and treatment has placed an inordinate burden on finances, social services and internists, as well as severely compromising the outcome of NNCs (AU)

A case control study of adult T-cell leukaemia/lymphoma and human T-lymphotropic virus type I in Jamaica - abstract

Ninety-three patients with haematological malignancies were enrolled into the study between Janauary, 1984 and August, 1985. Of these, 23 fulfilled the clinicopathological criteria for the diagnosis of adult T-cell leukaemia/lymphoma (ATL). Eighteen of 23 ATL patients were HTLV-I seropositive, compared with 8 of 46 age- and sex-matched general medical controls, resulting in a claculated odds ratio (estimated relative risk) of 17:1. Other patients with non-Hodgkin's lymphoma had slightly higher seropositivity rates than the controls, but none of the other haematological malignancies were HTLV-I positive. No other risk factors for ATL were conclusively demonstrated. The recently noted association of HTLV-I with tropical spastic paraparesis (Jamaican neuropathy) was supported by a high level of HTLV-I antibodies among patients with certain neurological disorders (AU)

Early deaths in Jamaican children with sickle cell disease.

In Jamaican children with homozygous sickle cell (SS) disease diagnosed at birth two-year survival was 87%, compared with 95% in children with sickle cell-haemoglobin C (CS) disease, and 99% in normal controls. Death among those with SS disease occurred most often between the ages of 6 and 12 months. Principal causes were acute splenic sequestration and pneumococcal infection. Neonatal diagnosis of haemoglobinopathies must be followed by close observation if mortality is to be reduced by early diagnosis and treatment of these complications.

Gamma chain variants in Jamaican newborns.

15,661 cord bloods from Jamaican infants were examined for abnormal hemoglobins using alkaline cellulose acetate electrophoresis for the initial screening, supplemented by acid agar gel electrophoresis for samples exhibiting abnormal hemoglobin bands. Of the 16 electrophoretic variants which were detected, six were fully characterized and found to be: four Hb F Port Royal (alpha2 Ggamma2 125 Glu replaced by Ala) and two Hb F Victoria Jubilee (alpha2Agamma2 80 Asp replaced by Tyr). The Hb F Port Royal samples each constituted about one eighth of the total Hb F as did seven additional samples presumed to be Hb F Port Royal. The infants with this variant exhibited no special hematological characteristics or other consistent associations. Both Hb F Victoria Jubilee samples occurred in somewhat lower proportions of the total Hb F compared with Hb F Port Royal and exhibited an apparent increase of free alpha chains in the whole hemolysate. The data available on detectable gamma chain variants suggest that a specific point mutation may occur in either a HbGgamma or a HbAgamma locus.