Case of the month: September 1998 - 18 year old male with large cystic orbital tumor.
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Adolescent↗
Biomedical subjects
M C Sharma
Publications and source records attributed to M C Sharma.
Interactions of gender, growth hormone, and phenobarbital induction on murine Cyp2b expression.
The interactions of gender, growth hormone, and phenobarbital induction on Cyp2b expression were examined in phenotypically normal (lit/+) and growth-hormone deficient "little" (lit/lit) mice. Using an immunocrossreactive monoclonal antibody designed to identify rat CYP2B1 and 2B2 proteins, we observed three hepatic Cyp2b proteins in control (lit/+) females, but only two proteins, one at trace levels, in control males. Phenobarbital administration to lit/+ mice increased the expression of the two Cyp2b isoforms in the males by 3- to 4-fold, but produced an approximately 75% reduction in the female-expressed proteins. Whereas growth hormone depletion (lit/lit) had no effect on the expression profile of Cyp2b proteins in females, it had a de-repressive effect in males, resulting in the expression of three proteins at concentrations now comparable to those observed in female liver. Generally, phenobarbital had no inductive effects in the lit/lit mice of both sexes. In all groups, transcript levels measured by a CYP2B1 probe were in agreement with the protein findings. In contrast, Cyp2b mRNA identified by an oligonucleotide probe for CYP2B2 were repressed completely by growth hormone in both sexes, and was expressed as a female-predominant transcript in the lit/lit mice. In spite of an apparent high degree of sequence homology between the rat CYP2B and murine Cyp2b gene families, the present findings highlight fundamental differences in their constitutive and gender-dependent expression, growth hormone regulation, and phenobarbital inducibility.
Animals↗
Medullomyoblastoma. A rare cerebellar tumour in children.
Seven patients between the ages of 3 and 24 years were admitted to our hospital in the last 28 years who had a histological diagnosis of medullomyoblastoma. These patients presented with classic symptoms of a posterior fossa midline mass associated with evidence of raised ICP. A CT scan in each patient revealed a uniformly high-attenuating tumour in the posterior fossa with gross hydrocephalus. In all seven patients a ventriculoperitoneal shunt was placed prior to definitive surgery. Radical tumour excision was carried out in all cases 3-5 days after CSF diversion. The histological diagnosis was made on H&E-stained slides. In two cases each, the tumour tissue was subjected to electron microscopy and immunohistochemical studies. Six of the seven patients survived the operation. One patient died 21 days after surgery as a result of shunt block and shunt infection. All surviving patients received cranial and spinal radiation 2-4 weeks after surgery, and also chemotherapy. The cranial radiation dose ranged from 4500 to 5000 rad, while the spinal radiation dose was limited to 1500 rad. Patients were followed up carefully. Three patients died within 6 months, and the remaining three between 2.5 and 3 years after surgery. None of the patients in our study survived longer than 3 years. One patient had developed paraplegia. This study highlights the details of an uncommon entity and reports the largest collection of such cases in the literature.
Adult↗
Aggressive angiomyxoma of the vulva--a case report.
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Adult↗
Gliofibroma: mixed glial and mesenchymal tumour. Report of three cases.
Gliofibromas are rarely encountered astrocytic tumours comprising of astrocytic and benign fibroblastic components. They commonly occur in first two decades of life. However, the exact behaviour is not fully known and their histogenesis is also still debatable. We report three cases of gliofibroma in which we studied proliferative markers (MIB-1) and p53 protein expression. In these tumours, occurrence in adult life is in contrast to that reported in the literature. Depending upon the morphology and proliferative Labelling Index we classified these tumours into low grade (benign) and high grade (malignant/anaplastic).
Adult↗
Taping outpatient consultations: a survey of attitudes and responses of adult patients with ocular malignancy.
PURPOSE: To study the attitudes and responses of cancer patients to audiotaped consultations as a communication aid in an ophthalmic clinic. METHODS: Ninety-four patients attending an ocular oncology clinic were given an audiotape of their initial consultation. They were then administered a structured interview with questionnaire regarding their views on the tape when they attended an outpatient clinic for follow-up after surgery or radiotherapy. RESULTS: 91% of patients had listened to the tape at least once, often prior to their treatment or follow-up visit. Most patients had shared the tape with immediate relatives, including their spouse (58%) or other family members (45%). Of those who had listened to the tape, 94% described it as very useful or quite useful in helping them understand their condition. The majority had found it valuable in helping them understand their treatment and its side-effects, in reducing their fears and anxieties and in their emotional and psychological adjustment to their illness. Most commented on the tape as a valuable communication aid to the consultation process. CONCLUSIONS: Taping of the consultation as a method of improving communication is popular among cancer patients. The vast majority benefit greatly from being able to hear their consultation again in their own time.
Adult↗
A study of proliferative markers in central neurocytoma.
To gain a better insight into the biological behavior of central neurocytomas, various proliferative indices were studied in these tumors and correlated with the histological features as well as the clinical outcome. Twenty cases of neurocytoma were selected over a 16 year period (1980-1995), which accounted for 0.28% of all intracranial tumors reported at this centre. Treatment consisted of surgical resection (total 14, subtotal six) followed by radiotherapy. Except for five patients who died of surgical complications, the remaining 15 were all alive and well during the follow-up period, varying from six months to 72 months (average 32 months). Thirteen tumors showed benign histological characteristics (Group I) while seven showed mitoses + necrosis (Group II). The proliferative index was assessed in formalin-fixed paraffin-embedded tissue of 17 cases using the silver nucleolar organiser region (AgNOR) technique and immunohistochemical staining for proliferating cell nuclear antigen (PCNA-PC10 antibody) and Ki-67 antigen (MIB-1 monoclonal antibody). The AgNOR counts ranged from 1.2 to 2.6 (mean 1.9 +/- 0.4), PCNA labeling index (LI) from 0.1 to 5.5 (mean 2.5 +/- 1.8) and MIB-1 LI from 0.1 to 3 (mean 0.8 +/- 0.02). There was no significant difference in any of these parameter values between histological Groups I and II, except that MIB-1 LI tended to be higher in Group II tumors. Further, there was no significant correlation between these proliferative indices and the mitotic rate of the tumors as well as the survival of the patients. A longer follow-up will be required to determine the relationship between proliferative markers and outcome as well as to bring out any heterogeneity in their biological behavior. Since these are relatively rare tumors, multicentric pooling of data will be required to reach a definitive consensus regarding their biological aggressiveness and consequentially, the use of radiotherapy in their treatment. The present report is a contribution in this direction.
Adolescent↗
Hypothalamic hamartoma: report of a case with unusual histologic features.
A rare case of hypothalamic hamartoma with unusual radiologic and histopathological features is described, possibly the first of its type in English literature. A 1.5-year-old female child presented with precocious puberty. MR scan of the brain revealed a pedunculated hypothalamic mass, most of which was isointense with normal brain on T1- and T2-weighted images. However, a sizeable component of the lesion was hyperintense on T1-weighted images, suggestive of adipose tissue. Microscopically, the lesion was a hamartoma composed of an admixture of neuroectodermal elements, namely glial cells, neurons, and nerve bundles along with mesenchymal elements in the form of fibroadipose tissue.
Brain Diseases↗
Giant cell phlebitis as a cause of large intestinal stricture.
Although arteritis of the gastrointestinal tract is well known, an isolated phlebitis without associated arteritis of the colon and cecum is rare. We describe a distinct form of giant cell phlebitis in a 16-year-old girl causing ischemic stricture of the large intestine. She presented with subacute intestinal obstruction and was suspected of suffering from tuberculosis. However, histopathologic examination showed giant cell phlebitis, the arterioles and arteries being spared. Although this is an extremely rare form of nonprogressive vasculitis, it should be considered in the differential diagnosis of strictures in the large intestine, especially in the young.
Adolescent↗
A comparison of cytochrome P450-dependent testosterone 2alpha-hydroxylase in rat (P4502C11) and mouse (P4502alpha).
Hepatic P450 2C11 in the rat and P450(2alpha) in the mouse are unique in being the only isoforms in their respective species with testosterone 2alpha-hydroxylase activity. Comparing gender differences, tissue distribution and physicochemical properties, we investigated whether this uncommon catalytic activity shared by the two isoforms is dependent upon a high degree of homology. Using additional substrates (e.g. androstenedione, hexobarbital), we observed that P450(2alpha) and P450 2C11 produced no metabolites in common. Moreover, concentrations of antisera prepared against purified P450(2alpha) that inhibited 95% of P450(2alpha)-dependent testosterone 2alpha-hydroxylase activity had only a minimal inhibitory effect (< 20%) on P450 2C11-dependent testosterone 2alpha-hydroxylase and were similarly unreactive to the rat isoform isolated on Western blots. Comparison of the isoforms' N-terminal amino acid residues and two internal peptide fragments indicated almost no sequence homology (< 4%). Gender-dependent tissue expression levels of P450(2alpha) and P450 2C11 revealed additional dichotomies. Whereas hepatic P450(2alpha) was moderately female-predominant (M/F; 0.62), hepatic P450 2C11 was clearly male-specific (M/F; 32.9). Murine P450(2alpha) mRNA was equally and substantially expressed in liver, kidney and brain; by contrast earlier studies reported that rat P450 2C11 was exclusively expressed in liver. The present results indicate that the unique testosterone 2alpha-hydroxylase activities of P450(2alpha) and P450 2C11 are expressed by two very different proteins exhibiting minimal homology.
Amino Acid Sequence↗
Adrenal lipoma. A case report.
Lipoma of the adrenal gland is an extremely uncommon tumor detected incidentally at postmortem or during life when the patient undergoes investigations for other reasons. All tumors reported in the literature were very small in size. We report a case of lipoma of the adrenal gland in a 45-year-old male patient who was hypertensive and presented with pain in the abdomen. The symptomatic presentation, large size, laparoscopic removal and rarity of this tumor merit documentation in the literature.
Adrenal Gland Neoplasms↗
Calcified filum terminale paraganglioma causing superficial siderosis.
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Brain Diseases↗
Diagnostic yield in computed tomography guided stereotactic biopsies.
Fifty three patients underwent computerised tomography (CT) guided stereotactic biopsies from different CT defined zones of attenuation with the Leksell stereotactic apparatus from October 1993 through January 1995. Multiple lesions were seen in 16 cases and 3 of them had multiple rim enhancing lesions. Astrocytoma was the most common histological diagnosis and thalamus was the commonest site of these tumours. The overall positivity rate was 98.2%. Positive yield from the centre of the lesion, peripheral and perilesional areas was 92.1%, 54.7% and 17.6%, respectively. The definite pathological diagnosis was made in 81.1% of cases. Post-operative neurological worsening was seen in 6 patients, of which 2 recovered without any surgical treatment, in 1 patient ventriculo-peritoneal shunt was done post-biopsy whereas in another evacuation of hamatoma was done which relieved headache and vomiting while 2 patients (3.7%) died.
Adolescent↗
Assessment of proliferative potential of meningiomas using PCNA LI and AgNOR counts.
Seventy-six cases of international meningiomas were studied using staining for Proliferating Cell Nuclear Antigen (PCNA) and silver nucleolar organizer regions (AgNORs) in order to find out any correlation of these parameters to the histological typing of the tumours and their biological behaviour. Histologically, 35 of the 76 cases were benign, 12 atypical and 19 malignant. Ten cases were recurrent. A male preponderance with a male: female ratio of 1.6:1 was noted. Five of 76 cases were in children below 15 years of age. Majority of the tumours were convexity meningiomas. Overall PCNA labeling index (LI) values ranged from 0.1% to 11.0%. Benign group had an LI of 0.9 +/- 1.42 whereas atypical, malignant and recurrent group had LIs of 4.06 +/- 2.33, 2.91 +/- 2.66 and 3.36 +/- 3.76 respectively. One way analysis of variance test showed a significant difference in the distribution of LI between benign versus atypical, malignant and recurrent group (P < 0.05). A highly significant difference was also observed between PCNA LI of recurrent benign group versus non-recurrent benign group (p < 0.01, wilcoxon Rank Sum Test). On further classifying the tumours based on LI values, it was observed that 30 of 41 (73%) cases of combined atypical, malignant and recurrent group (i.e. biologically more aggressive group) had LI of > 1%, whereas 26 of 35 cases (74%) belonging to the benign group had LI of < 1%. Overall AgNOR counts ranged from 1.27 to 3.11. No statistically significant difference was found in AgNOR counts amongst the different groups of meningiomas. There was no correlation between PCNA LI and AgNOR counts. It was thus concluded that PCNA LI but not AgNOR counts in the primary tumour could be of potential value for more accurate assessment of biologic behaviour of meningiomas in conjunction with the conventional A histological parameters.
Adolescent↗
The unusual imaging appearance of primary retroperitoneal teratoma: report of a case.
Primary retroperitoneal (RP) teratoma is a rare entity which has a distinctive imaging appearance. We describe herein the case of a 25-year-old man in whom a RP teratoma was found to have an extremely unusual imaging morphology by ultrasound and computed tomography (CT). The tumor was resected and histopathological examination confirmed the diagnosis of primary benign RP teratoma.
Adult↗
Diurnal periodicity of Leishmania amastigotes in peripheral blood of Indian Kala-azar patients.
Peripheral blood smears of 20 patients with Indian Kala-azar (KA) were collected throughout the day and night and examined for presence of Leishmania amastigotes. Amastigotes were detected in 46% of peripheral blood samples taken during the day (08:00-17:00 h) and 66% and during the night (18:00-07:00 h). This difference was statistically highly significant (P < 0.0001). The confirmation of amastigote diurnal periodicity is of interest, and the high percentage of parasitemic patients at night is likely to reflect the biting habit of the sandfly vector of Kala-azar Phlebotomus argentipes.
Animals↗
Solitary giant skull base schwannomas--report of four cases.
BACKGROUND: Solitary intracranial schwannomas not related to major nerves or neurofibromatosis as well as paranasal schwannomas are rare. Schwannomas simultaneously involving the paranasal sinuses and intracranial cavity are even rarer. METHODS: We report four cases of schwannomas simultaneously involving the intracranial cavity and paranasal sinuses. They were successfully managed by surgery. The literature on such tumors is reviewed. RESULTS: All patients were young adults; the male to female ratio was 1:3. In two patients, the tumor was predominantly intracranial with extension into the sphenoid and ethmoid sinuses, whereas in the other patients, the tumor was located predominantly in the paranasal sinuses and nasopharynx with intracranial extension. Radiologically, bone destruction was seen in three cases. The tumors were totally removed piecemeal with repair of the basal dura. Histopathologic examination confirmed the diagnosis of schwannoma in all four cases. Three patients are alive and well; one of them was reoperated for a recurrence. CONCLUSIONS: These tumors should be excised completely if possible. Radiologically, bone erosion or destruction are suggestive of malignancy but histopathology clinches the diagnosis. Therefore, drastic surgery should be avoided in these cases. Surgery is generally curative in these massive schwannomas.
Adolescent↗
Unilateral meningeal thickening: a rare presentation of tuberculous meningitis.
A rare case of tuberculous meningitis (TBM) causing unilateral thickening of meninges, is described here. The 50 year old male is presented with hemicranial headache and unilateral multiple cranial nerve involvement without any long tract signs. Magnetic resonance imaging (MRI) scan including enhanced studies showed a hemicranial thickening of the meninges. Biopsy of the meninges revealed tuberculosis. The patient improved gradually on antitubercular treatment (ATT). Follow-up at 6 months revealed significant improvement in neurological status. Though tuberculous involvement of the central nervous system (CNS) is very common in India, hemicranial meningeal involvement has never been reported.
Antitubercular Agents↗