The importance of a high index of suspicion in rabies.
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Animals↗
Biomedical subjects
M C Sharma
Publications and source records attributed to M C Sharma.
August 2002: 21-year-old male with cystic intracerebral tumor.
The August 2002 COM. A 21-year-old male presented with a single episode of generalized tonic clonic seizures. Radiology revealed a cystic tumor with mural nodule suggestive of a pilocytic astrocytoma. However, histopathological examination and electron microscopy revealed features of an intracerebral schwannoma. Therefore, although rare, in an intracerebral cystic lesion with mural nodule, the possibility of an intracerebral schwannoma should be entertained. This is important because this is a benign tumor with favourable response to resection.
Adult↗
Bone marrow metastasis in anaplastic oligodendroglioma.
Oligodendroglioma is a rare primary brain tumour. These tumours rarely metastasis extraneurally because of the absence of a lymphatic system, the presence of the blood-brain barrier and the patient's poor overall survival. Oligodendroglioma may spread via the cerebrospinal fluid or after surgical intervention. Metastasis to bone marrow is extremely rare. Oligodendroglioma is now considered a chemosensitive disease. If chemotherapy leads to prolonged survival, we may see more extraneural metastases in future.
Antineoplastic Agents↗
A comparative study of classical vs. desmoplastic medulloblastomas.
Classical and desmoplastic medulloblastomas (MBs) have been suspected to be biologically different, though comparative studies on markers of biological aggressiveness in these two variants are sparse in the literature. 87 classical and 43 desmoplastic variants of MB were studied with respect to clinical and histological characteristics, MIB-1 labeling index (MIB-1 LI), apoptotic index (AI), ratio of AI to MIB-1 LI, expression of p53 and Bcl-2 protein and 3-year progression-free survival. The only differences documented between the variants were with regard to age distribution and location. Thus, classical histology cases occurred predominantly in children and 80% were midline in location. In contrast, lateral location was seen more frequently with tumors of desmoplastic histology, which occurred in an almost equal distribution between children (56%) and adults (44%). No difference was noted between the variants with regard to proliferation index, apoptotic index, their ratio on or their molecular controls (p53 and Bcl-2). This was reflected in the clinical outcome wherein no significant difference was observed in the 3-year progression-free survival between the variants. It is concluded that the two histological variants of medulloblastoma are not different with regard to biological parameters of aggressiveness. The growth rate and clinical outcome in medulloblastomas have no correlation with the histological variant.
Adolescent↗
Primary osteosarcoma of the skull. A report of 3 cases.
Osteosarcomas are malignant bone tumors, which commonly affect the long bones of young adults. Primary osteosarcomas of the skull are exceedingly rare. We herein report 3 cases of primary osteosarcoma of the skull involving the calvaria, diagnosed over a period of 8 years at the Department of Pathology of our Institute.
Adolescent↗
Clinicopathological study of spinal teratomas. A series of 10 case.
AIM: Sacrococcygeal teratomas are common in children, but intramedullary spinal teratomas are rare. We report a series of 10 cases of spinal teratomas, which, to the best of our knowledge, is the largest series of intramedullary teratomas. METHODS: During a period of 15 years (1987-2001) 10 cases of spinal teratomas were diagnosed in our department. The clinical profile, radiological data and histopathological slides were reviewed. RESULTS: Age ranged from 10 months to 51 years (mean 23.5 years) with male predominence. Duration of symptoms varied from 5 months to 20 years (mean 4.1 years). The most common symptoms were weakness of lower limbs, backache and urinary bladder involvement. Radiologically, these were heterogenous lesions with fat signal and areas of calcification. Surgery is the treatment of choice. Only 1 case recurred after 9 years of operation. CONCLUSION: Although there are no specific features on imaging of intramedullary teratomas, solid and cystic morphology, fat signal and areas of calcification are some of the helpful features. Most of these lesions are diagnosed on histopathological examination after surgery. Surgery is the treatment of the choice.
Adult↗
Merosin negative congenital muscular dystrophy: a short report.
We report a rare case of an infant with congenital muscular dystrophy who presented at birth with marked generalized hypotonia and normal mental development. Creatinine phosphokinase (CPK) level was markedly raised; however no white matter abnormalities were detected by brain imaging techniques. Immunohistochemical staining for merosin (laminin alpha 2) was negative, thereby confirming merosin-deficient congenital muscular dystrophy.
Female↗
Gamma-sarcoglycanopathy.
The best known muscular dystrophies are X-linked dystrophinopathies. A clinically and genetically heterogeneous group presenting with weakness of the pelvic and shoulder girdles is that of the limb-girdle muscular dystrophies (LGMDs). Sarcoglycanopathies (SGPs) are autosomal recessive LGMDs. We report a rare case of primary gamma-sarcoglycanopathy (SGP) which emphasizes the evolving concept of dystrophinopathy to sarco-glycanopathy.
Biopsy, Needle↗
Diagnostic intraocular aspiration cytology of choroidal melanoma.
Fine-needle aspiration cytology (FNAC) was performed on an intraocular mass in a 32-yr-old Indian woman and a cytologic diagnosis of malignant melanoma was made, supported by immunocytochemistry. The cytologic features included spindle-shaped tumour cells with minimal pleomorphism and the presence of nuclear grooves. No intracellular pigment was identified; however, positivity for HMB-45 allowed a rapid and reliable diagnosis. The utility of FNAC in an atypical presentation of choroidal melanoma is discussed.
Adult↗
A melanotic desmoplastic medulloblastoma: report of a rare case and review of the literature.
A 28-year-old man had a desmoplastic medulloblastoma in the vermis and left cerebellum. This tumor was composed of nodular, reticulin-free zones (pale islands) surrounded by densely packed, highly proliferative cells that produced a dense intercellular reticulin network. Some of the cells were heavily pigmented, and this pigment proved to be melanin. Adult age, desmoplastic nature, and melanin pigmentation are some of the rare features of this tumor that need documentation. Further, this pigment was in the primitive cells, unlike in the published cases, in which it was present in the tubular or tubulopapillary component. To the best of our knowledge, this is the first published case of desmoplastic pigmented medulloblastoma, and the patient is the oldest reported to have this tumor.
Adult↗
Chronic subdural haematoma and the enigmatic eosinophil.
The membranes from 50 cases of chronic subdural haematomas were examined histologically and correlated with the duration of the lesion. Cases were divided into three groups based on duration from time of trauma and/or onset of clinical symptoms to date of surgery - Group I: 1 to 30 days, Group II: 31 to 90 days and Group III: >90 days. Infiltration with eosinophils was observed in the vascularised and hyalinised granulation tissue of the subdural membrane in 30 of the 50 cases (60%). There was a trend to correlation both of the frequency and the extent of eosinophilic infiltration with duration of haematoma. Thus, eosinophils were encountered in about half the cases with duration up to 3 months which increased to 80% in cases with duration more than 3 months. The extent of eosinophilic infiltration (mild, moderate or severe) also appeared to correlate with duration of haematoma in that mild infiltration was more common in Group I cases while moderate to severe infiltration were more frequently observed in Group II and III cases. No correlation was observed of the eosinophilic infiltrate with age and sex of the patients or with presence of other cellular inflammatory components of the membrane. Interestingly, a finding hitherto unreported in English literature was the demonstration of mast cells in 7 of 16 membranes (44%) which had been stained using toluidine blue. It is possible that the eosinophils appear at this unusual site due to chemotactic stimulus abetted by these mast cells as well as lymphocytes and haemosiderin pigment. The eosinophils may have an important role in the repair and healing process of these membranes.
Adolescent↗
Intramedullary tuberculoma of the spinal cord: a series of 10 cases.
OBJECTIVE: Involvement of vertebral column is common in tuberculosis but intramedullary tuberculomas are rare. We report a series of ten cases of intramedullary tuberculomas, which, to the best of our knowledge, is the largest series of biopsy proven intramedullary tuberculomas in English literature. METHODS: During a period of 16 years (1985-2000), ten cases of intramedullary tuberculomas were diagnosed in our department. Of these, eight cases were histologically proven intramedullary tuberculomas. The clinical profile, radiological data and histological slides were reviewed. RESULTS: Age ranged from 18 to 45 years (mean 29.7 years) and there was slight male preponderance (six men, four women). Duration of symptom varied from 3 to 20 months (mean 11.5 months). All of them presented with motor weakness and sensory impairment. Most common site of involvement was dorsal cord followed by cervical, cervicodorsal and dorsolumbar regions. Three patients had associated involvement of lungs, cervical lymphnodes, and brain, and one patient had past history of tuberculous meningitis. Two patients were treated conservatively but surgical excision was done in eight cases followed by medical treatment. CONCLUSION: Radiologically, intramedullary tuberculomas should be differentiated from other space occupying lesions (SOL) to avoid unnecessary surgery especially in those patients with tuberculosis of the other organs. The incidence of intramedullary tuberculomas is likely to increase with a rise in the incidence of AIDS.
Adolescent↗
Carcinoma cervix with metastasis to deltoid muscle.
Skeletal muscles are rare metastatic sites despite their rich blood supply and the fact that the muscular mass of the body accounts for a large percentage of the total body weight. They account for less than 1% of all malignant metastases of haematogenous origin. Menard and Parache, Ann Med Interne Paris 1991;142:423-428. Skeletal muscle metastasis in cervical cancer have been infrequently reported. In this case report, we detail a case of cervical cancer who developed a painful swelling of the right shoulder which on investigation, was found to be skeletal muscle metastases.
Adult↗
Lipomedulloblastoma in a child: a controversial entity.
Lipomedulloblastoma is regarded as a distinct entity that occurs exclusively in adults and has a low proliferative potential and a favorable outcome. We describe a rare case of lipomedulloblastoma in a 6-year-old female child showing a high labeling index that needs documentation. The various hypotheses of adiposal change are discussed.
Adipose Tissue↗
Recurrent astrocytic tumours--a study of p53 immunoreactivity and malignant progression.
Recurrence and progression to higher grade lesions are characteristic of the clinical course of astrocytic tumours. Though p53 gene mutation is an important initiating event in astrocytic tumourigenesis, its role in malignant progression remains controversial. We have therefore analysed p53 protein expression in paired histological samples from 48 cases of astrocytic tumours and their recurrences--29 diffuse astrocytoma, 10 anaplastic astrocytoma and 14 glioblastoma multiforme (GBM). Malignant progression at recurrence was noted in 93% of diffuse and 64% of anaplastic astrocytomas. An association was observed of p53 protein immunopositivity and malignant progression at recurrence. Thus, 27 of 48 (56%) primary tumours were initially p53 positive, while in recurrent tumours associated with malignant progression this frequency increased to 71% (34/48 cases). This was because seven of the 13 cases (4/8 diffuse and 3/5 anaplastic astrocytoma) that were initially p53 negative acquired immunopositivity on malignant progression at recurrence. In contrast, none of the 19 tumours that recurred to the same grade showed any change of p53 status at recurrence. Furthermore recurrence was associated with increase in the percentage of p53 immunopositive cells (p53 labelling index), which was also higher in tumours with progression. This new acquisition of p53 immunopositivity on progression at recurrence has not been documented in earlier studies in English language literature, though increase in p53 LI has been documented. Thus, this study conclusively indicates the role of p53 in malignant progression of astrocytic tumours. Also, it suggests a potential role of p53 LI in predicting malignant progression at recurrence because the highest initial LI was noted in those tumours which progressed to GBM as compared with those which recurred to the same grade or progressed to anaplastic astrocytoma. No correlation could, however, be demonstrated between p53 immunoreactivity and interval to recurrence.
Adolescent↗
Intramedullary neurenteric cyst presenting as infantile paraplegia: a case and review.
The authors report a case of a 3-month-old male child with paraplegia in whom magnetic resonance imaging (MRI) revealed a nonenhancing intramedullary cystic lesion extending from the level of D1 to D7 without any other associated anomaly. Intraoperatively, these findings were confirmed and the spinal cord was found to have splayed circumferentially into a papery thin rim. The patient underwent marsupialization of the cyst with subtotal excision of the cyst wall. Histopathological examination revealed ciliated pseudostratified columnar epithelium consistent with the diagnosis of a neurenteric cyst. Intramedullary neurenteric cysts are rare developmental malformations, and out of the 13 previously reported cases, only 3 were evaluated by MRI. This is the first case report in the literature of an intramedullary neurenteric cyst presenting as infantile paraplegia. In the present report, the embryology, etiopathogenesis, radiological imaging and management of this rare clinical entity are discussed and a detailed literature review is presented.
Diagnosis, Differential↗
Tuberculosis of the maxillary sinus manifesting as a facial abscess.
Tuberculosis of the maxillary sinus is rare. Likewise, an acute onset that necessitates incision and drainage is also very uncommon. We report the case of a 15-year-old girl who came to us with an abscess on the left side of her face. She was found to have tuberculosis of the left maxillary antrum.
Abscess↗
Osteochondroma of the spine: an enigmatic tumor of the spinal cord. A series of 10 cases.
BACKGROUND: The aim of this study was to evaluate clinical, radiological and pathological features of vertebral osteochondromas with compressive myelopathy and to review the relevant English literature. Osteochondro-mas are common benign bony lesions of long bones but involvement of spine by solitary osteochondroma and its presentation as compressive myelopathy is rare. Most of the literature is in the form of case reports. METHODS: During a period of 20 years (1980-1999), 10 cases of osteochondromas of the spine were encountered. Clinical, radiological and pathological features were reviewed. RESULTS: The age ranged from 13 to 45 years (mean 25.3 years) and all except 1 were males. In 8 cases the pathology involved the cervical spine and in two cases dorsal spine was involved. All patients presented with progressive motor sensory deficit of 6 months to 30 years duration (mean 3.9 years). Decompressive laminectomy was carried out in all the patients. Fortunately, gradual and complete recovery was observed in all of them. CONCLUSIONS: Osteochondromas of the spine are not as rare as reported in the literature. In a young patient of compressive myelopathy this possibility should be considered. Magnetic resonance imaging, computed tomography and CT myelogram are useful in evaluating the size and extent of the lesion for subsequent surgical planning.
Adolescent↗