Oncogenic osteomalacia (OO) is a rare clinicopathological syndrome characterized by renal phosphate wasting, hypophosphataemia, normal serum calcium levels and decreased serum 1,25-dihydroxyvitamin D3 levels, which are induced by a neoplastic lesion. The clinical signs and symptoms disappear completely after resection of the tumour.1–3 Phosphaturic mesenchymal tumour (PMT) is a rare mesenchymal tumour that causes OO. Fine needle aspiration cytology (FNAC) of PMT has been documented only once before.4 Our case is the first at this site, as a bone tumour in the jaw with diverse differential diagnosis. A 35-year-old man presented with complaints of multiple bone pains and generalized muscle aching of 3 months’ duration. He had a pathological fracture of the neck of left femur 2 years back. Clinically, no neurovascular deficit was noted. Investigations showed a low to normal serum calcium (7.8 mg/dl), low serum phosphorus (1 mg/dl) and elevated serum alkaline phosphatase (289 U/l). Radiographs showed osteopenia. A diagnosis of osteomalacia was made. The patient received injectable vitamin D and was advised to take oral calcium. One year later he presented with a progressively enlarging swelling in the right mandible. His symptoms had worsened and he was unable to walk. Local examination revealed a 6 × 8-cm bony swelling in the right mandible. Computed tomography (CT) showed a well-defined enhancing, expansile, solid and cystic lesion with internal septations situated in the body of the right mandible (Figure 1a). FNAC of the lesion was performed and the smears were received in our laboratory with a clinico-radiological suspicion of ameloblastoma. The smears were alcohol fixed and stained with Papanicolaou stain. (a) Computed tomography image showing expansile, solid cystic lesion with internal septations involving the body of the right mandible. (b) Cellular smear showing three-dimensional clusters and cribriform sheets of plump spindle-shaped and rounded cells (Papanicolaou, ×100). (c) Smear showing cribriform sheets with peripheral cells appearing to ‘fall off’ the cell clusters (Papanicolau, ×200); inset: the cells have plump elongated nuclei, fine granular chromatin and well-defined vacuolated cytoplasm. (d) High-power view showing plump cells with bland nuclei, nuclear crowding and overlapping embedded in eosinophilic osteoid-like stromal matrix (Papanicolaou, ×400). The smears were cellular and haemorrhagic and showed plump spindle and rounded cells with eccentric round vesicular nuclei, fine granular chromatin and scant vacuolated cytoplasm. The cells were arranged in three-dimensional clusters and cribriform sheets with prominent nuclear crowding and overlapping; focal spindling of the cells was seen. Peripherally, the cells seemed to be ‘dropping off’ from the clusters. The cells appeared to be embedded in abundant eosinophilic osteoid-like stromal matrix. No mitoses, necrosis or nuclear pleomorphism was evident (1, 2). A possibility of ameloblastoma was suggested. Histopathological section: haphazardly arranged bland-looking plump spindle cells with microcystic spaces, aneurysmally dilated vascular spaces and focal aggregates of osteoclastic giant cells (haematoxylin and eosin, ×200). The confounding factors responsible for the cytological misdiagnosis were: first, the location of the tumour in the mandible clinico-radiologically mimicking ameloblastoma; second, disregard for the history of osteomalacia; and third, cytological misinterpretation of the cribriform nests with central dyscohesive cells as ‘stellate reticulum’, classically seen in ameloblastoma. A differential diagnosis of low-grade sarcoma was also considered. The presence of plump spindle cells showing mild nuclear pleomorphism arranged in large three-dimensional clusters and cribriform nests were features favouring this diagnosis. Surgical intervention with right hemimandibulectomy was performed. Histopathological examination revealed a tumour composed of sheets of plump spindle and rounded cells with mild nuclear pleomorphism, occasional mitoses (3–4/10 high-power fields) and prominent vascularity with aneurysmal dilatation of the vascular channels. The stroma showed microcystic change, scattered aggregates of osteoclast-like giant cells, focal osteoid metaplasia and amorphous calcification (Figure 2). Histological differential diagnoses that were considered included giant cell tumour (GCT) with secondary aneurysmal bone cyst formation, chondroblastoma, giant cell malignant fibrous histiocytoma (MFH) and haemangiopericytoma. GCT of the mandible is extremely rare and shows a more uniform distribution of giant cells, with nuclei of the giant cells resembling those of the mononuclear cells. Chondroblastoma is a childhood tumour with islands of ‘chicken wire’ calcification and mononuclear cells showing oval nuclei with a longitudinal groove. MFH is a diagnosis of exclusion and has overlapping findings on immunohistochemistry (IHC), the cells being positive with vimentin, smooth muscle actin (SMA) and CD68. Haemangiopericytoma shows proliferation of stag horn-shaped blood vessels surrounded by spindle cells which are CD34 positive. On IHC, the tumour cells were positive for vimentin and negative for epithelial membrane antigen, SMA, CD68 and CD34. The osteoclast-like giant cells were positive for CD68. Taking into account these findings, together with the crucial history of persistent osteomalacia, a final diagnosis of PMT-mixed connective tissue variant was made. Fibroblast growth factor 23 (FGF-23) IHC performed subsequently was, however, negative in this case. The patient’s clinical symptoms improved following surgery. Serum phosphate levels returned to normal at around 4 weeks following surgery. At 1-year follow-up, the patient was asymptomatic with no evidence of tumour recurrence. McCance is credited with describing the first case of OO in 1947 while Prader et al., in 1959, first recognized a neoplasm as the cause of the clinical signs and symptoms of OO.2 These tumours secrete FGF-23, a phosphaturic substance that causes total body phosphate depletion, leading to osteomalacia/rickets. FGF-23 is readily detectable in the plasma or serum of healthy persons and can be markedly elevated in those with OO.5 It can also be demonstrated in tumour tissue by reverse transcriptase-polymerase chain reaction (RT-PCR) and IHC.3 We did not have the facility to measure the serum FGF-23 in our case. Various tumours associated with OO reported in the literature include neurofibromatosis, McCune–Albright syndrome,3 carcinomas (prostatic, breast, small cell), lymphocytic leukaemia, myeloma,6 osteogenic sarcoma, fibrous dysplasia, haemangiopericytoma, haemangioma, chondroblastoma, chondromyxoid fibroma, metaphyseal fibrous defect, GCT, MFH and PMT.7 The term PMT was coined by Weidner and Cruz 8 in 1987. They classified these tumours into four morphological groups: (1) mixed connective tissue variant, common in soft tissue and characterized by a distinctive admixture of spindle cells, osteoclast-like giant cells, microcysts, prominent blood vessels, cartilage-like matrix and bone; the present case had similar features. The other groups resembled bone tumours: (2) osteoblastoma-like; (3) nonossifying fibroma-like; and (4) ossifying fibroma-like variants. Folpe et al.3 proposed an additional group of nonphosphaturic PMT. The tumour is commonly seen in the fourth to fifth decades of life, although no age is exempt and there is no sex predilection. It commonly occurs in bone and soft tissue of the head and neck region and the extremities.7 Rarer sites include the pharynx, breast,4 spinal nerve9 and tongue.10 In most patients, the musculoskeletal symptoms precede the detection of the tumour by several months to years.4,7 In our case, the patient had disease manifestations 1 year prior to the diagnosis of the tumour. Rarely, PMT can recur or metastasize.3,8 The diagnostic utility of IHC in PMT is chiefly to exclude other tumours with overlapping morphology. The tumour cells in PMT are positive for vimentin and occasionally for αSMA,3 with the osteoclast-like giant cells being CD68 positive.6 Granular cytoplasmic positivity for FGF-23 confirms the diagnosis of PMT, but negative staining does not rule it out.3 The role of cytology in the diagnosis of PMT is still evolving. The cytological features of the present case are as follows: (1) three-dimensional clusters of bland-looking plump spindle and rounded cells; (2) nuclear crowding and overlapping; (3) cells embedded in eosinophilic osteoid-like matrix; and (4) peripheral cells appearing to ‘fall off’ from the clusters. The only difference on comparison with the first described case was the presence of cribriform sheets of cells. This could possibly be explained by the microcystic spaces or the prominent vasculature seen on histology. In conclusion, the cytological findings of PMT are quite distinctive. A definitive diagnosis is only possible by the amalgamation of FNAC findings with the clinical history and biochemical data. FNAC diagnosis of PMT, followed by tumour excision, should result in complete cure for the patient. The authors are grateful to Professor Pathmanathan Rajadurai, adjunct professor of Pathology, Meleka Manipal Medical College, for help in performing FGF-23 IHC for this case; and to Mr Ganesh Prasad, Artist at the Department of Pathology, Kasturba Medical College Manipal, for help in compiling the photographs.
Infection due to Human immunodeficiency virus (HIV) can cause damage to both the central and peripheral nervous systems and result in disorders of communication. Progressive decline in speech behaviours in HIV infected individuals have been documented in Western literature (Flower and Sooy, 1987). This study aims to create a database of speech impairments seen in individuals with HIV infection to reflect the need for assessment and management of communication skills. 15 males with HIV infection between the age ranges of 18 - 40 years were included in the study. The deviant speech characteristics was profiled on the parameters in Frenchay Dysarthria Assessment. 93.3% of the participants demonstrated speech impairments which ranged across the participants. Most affected were parameters of tongue and laryngeal functions followed by reflex, respiration, lip functions and intelligibility parameters. Jaw and soft palate functions were not affected in any of the participants. It can be concluded that HIV infection results in speech impairments in the affected individuals. But this conclusion has to be generalized with caution since only 15 participants were involved in this study. Further research considering the effects of medication, opportunistic infections and disease duration is suggested. Key words: HIV, speech disorders.
Objective The diagnosis of voice disorders is based on perceptual and acoustic paradigms. Modern acoustic analysis systems are relatively inexpensive and user friendly. One aspect of laryngeal function that is of great interest is the extent of vocal fold closure. Soft phonation index (SPI) is the parameter in Multi Dimensional Voice Program, which reflects the approximation of vocal folds. High values of SPI are stated to correlate with incomplete vocal fold adduction and are a better indicator of breathiness than EGG. This study aims to determine the sensitivity of this acoustic parameter of SPI, as a reflective indicator of incomplete vocal fold adduction in male patients diagnosed with unilateral vocal nodules. Methodology 60 participants were included in the study; 30 with normal vocal fold functioning in the control group and 30 with unilateral vocal nodules in the experimental group. The phonation sample of vowel /a/ was recorded into CSL 4150 of Kay Elemetrics, in a sound treated room. The readings of SPI on the MDVP analysis was extracted and subjected to statistical analysis using independent samples ‘t-test’ using SPSS Version 11. Results The results of the study reveal that there was a statistically significant difference between the means of the SPI values between the control and the experimental groups. Discussion It can be understood that SPI is a sensitive parameter to detect abnormalities in vocal fold approximation in the considered population of individuals with vocal nodules. This is in coherence with a study done on patients with vocal fold palsy and cordectomy, wherein SPI was found to be a good indicator of breathiness. The SPI mean obtained for participants in the control group was higher than the Western normative mean specified in MDVP, which highlights the importance of establishing normative values for Indian population. Conclusion It can be concluded that SPI was sensitive to detect changes affecting vocal fold closure in unilateral vocal nodules. But this conclusion has to be generalized with caution keeping in mind that only one population with vocal pathology was studied. The factors of vocal fold physiology and mechanical properties of vocal tract, which may contribute to individual variations in SPI values, have to be considered for further research.
HIV-1 infects brain cells, and this results in damage to the functioning of both the central and peripheral nervous systems. Progressive decline in communication behaviours can be seen from early stages of infection. Among the communication problems observed in those infected with HIV are speech, language, cognition and swallowing. This study was undertaken with the aim of creating a profile of the vocal impairments in individuals affected with HIV/AIDS, which would in turn support evidence-based practices in the assessment and management of such individuals. Eight participants were included in the study. Subjective analysis was carried out by a speech pathologist and objective analysis of acoustic parameters, by the use of Kay Elemetrics MDVP software. The results of the study reveal that all participants had affected parameters in the voice analysis, which ranged across subjects from one parameter to all parameters. Even in the absence of direct pathology to the voice production mechanism, almost all of the parameters of voice are affected in HIV-infected persons. As this is a preliminary study, it is too early to conclude that there is a correlation between vocal deviancies and medical conditions.
Symptomatic peritoneal deciduosis is a rare event during pregnancy and has rarely been documented in the literature. A single case of peritoneal deciduosis in pregnancy presenting as intestinal obstruction has been reported. We would like to report an additional case. INTRODUCTION Ectopic decidua (deciduosis) has been commonly described in the ovary, cervix , uterus, bowel and appendiceal serosa, omentum, renal pelvis and para-aortic and pelvic lymph nodes.1 Peritoneal deciduosis, however, is a less frequent event during pregnancy and is usually asymptomatic.2 We present an unusual case of peritoneal deciduosis in a primigravida with features of subacute intestinal obstruction. CASE REPORT A 25-year-old primigravida at 28 weeks’ gestation presented with constipation of 1 month’s duration, abdominal pain for 10 days and vomiting of 3 days’ duration. X-ray and ultrasound of the abdomen showed multiple air-fluid levels suggestive of acute intestinal obstruction. The patient was taken up for emergency laparotomy. Intraoperative findings revealed a stricturous terminal ileum. The distal ileum was resected and an ileal re-anastomosis was done. PATHOLOGICAL FINDINGS The specimen consisted of a stricturous portion of ileum received as 2 separate segments, together weighing 12g. Cut section showed an edematous mucosa with shaggy serosal surface (Fig. 1). Microscopic examination of the ileal mucosa showed focal ulceration with dense infiltration of the lamina propria by chronic inflammatory cells. The submucosa and muscular layer showed edema and congested vessels. The serosa was edematous and showed clusters of large polygonal decidual cells with abundant amphophilic cytoplasm, round-oval nucleus and prominent nucleoli surrounded by fibrous tissue (Fig. 2&3). Figure 1 Figure 1: Stricturous terminal ileum Peritoneal Deciduosis Presenting as Subacute Intestinal Obstruction – Case Report 2 of 3 Figure 2 Figure 2: Decidual cells lining serosa (HE 58: 196-199. 3. Heidegger H, Humpfner A, Hugo R, Schulz W. Peritoneal deciduosis: cause for mechanical ileus in pregnancy. Geburtshilfe Frauenheilkd 1991; 51: 307-309. Peritoneal Deciduosis Presenting as Subacute Intestinal Obstruction – Case Report 3 of 3 Author Information Mary Mathew, MD Department of Pathology, Kasturba Medical College Pankaj Bir Singh Ahluwalia, DCP Department of Pathology, Kasturba Medical College MS Susmitha, MD Department of Pathology, Kasturba Medical College Garima Goel, MD Department of Pathology, Kasturba Medical College Rajgopal Shenoy, MS Department of Surgery, Kasturba Medical College
Gelatinous transformation of the marrow (GTBM) has been associated with various conditions. We present a unique case of GTBM in a patient with myeloma following treatment with Melphalan.
John, George T.; Mathew, Mary; Snehalatha, Elizabeth; Anandi, V.; Date, Anand; Jacob, C. K.; Shastry, J. C.M. Author Information
Ninety adult Indian typhoid and paratyphoid fever (enteric fever, EF) patients and 91 controls were tested for glucose-6-phosphate dehydrogenase (G6PD) deficiency using the fluorescent spot test (FST) and the quantitative methaemoglobin reduction test (QMRT). There was a threefold higher incidence of G6PD deficiency in North Indian EF patients (10·6%) than in controls (3·6%) (P = 0·15) which may be attributable to the greater morbidity of the G6PD-deficient EF patients; six of nine had haemolytic anaemia. A transient depression of mean erythrocyte G6PD activity was observed in a subgroup of 49 non-deficient EF patients in whom the spectrophotometric G6PD assay was done. It did not appear to be related to reticulocyte count, chloramphenicol therapy, or differences in leucocyte contamination of the haemolysate used for the G6PD assay. If this depression of G6PD activity occurs in deficient patients as well, it may help to explain the haemolysis seen in them during EF. Of the three tests used, the QMRT and the spectrophotometric assay clearly identified G6PD deficiency in males during haemolysis, whereas the FST was unreliable in this situation.
217 cases of tetanus admitted to Christian Medical College and Brown Memorial Hospital, Ludhiana, from June 1977 to April 1980 were studied. Three treatment regimes were used during this period. The addition of intrathecal horse antitetanus serum (ATS) (Group II) to the routine treatment (Group I) reduced the mortality from 63% to 53% but this was not statistically significant. Following a further addition of parenteral betamethasone to the regimen (Group III) the mortality decreased to 27% which was significantly lower than in Groups I and II. A similar trend in mortality was demonstrated when the severe cases were analysed separately. Groups I and III were comparable in terms of various prognostic factors and the nursing care received but Group II had a higher incidence of unfavourable prognostic factors, which may have obscured the benefit of intrathecal ATS. The combination of parenteral betamethasone and intrathecal ATS seems to be beneficial in the treatment of tetanus.
Thiacetazone has been used as a standard antituberculous drug in several countries. Side effect& which include various VM of skin eruptions have been observed in about 10% of patients taking thiacetazone. We are reporting two' cases of lichen planus-like lesions presumably due to thiacetazone. Of these, one case had preexisting lichen planus hypertrophicus but the other one, did not have any past history of lichen planus.