Cystic fibrosis presenting as small bowel obstruction in pediatric patient: a case report

Intestinal obstruction complete or incomplete has been found commonly in patients with cystic fibrosis in all age groups with variable presentation. Prevalence of intestinal obstruction in children with cystic fibrosis is 7-8%. Neonates present with meconium ileus due to inspissated meconium and complete obstruction. This case demonstrates similar presentation in a pediatric patient. A six day old male patient presented in the pediatric surgery department with abdominal distention, vomiting and fever. Patient was operated and intraoperative meconium plug was seen along with dilated proximal bowel, filled with sticky meconium. Resected gut segment was received in the Department of Pathology. Grossly dilated gut segment on cut section was seen filled with thick putty like greenish fecal matter. Histopathological examination showed features consistent with cystic fibrosis. There are multiple causes for intestinal obstruction in neonates, cystic fibrosis can be one of them which needs to be considered by the clinicians. Such cases need to be reported for better understanding of their clinico-pathological presentation and prevalence.

Profile of Erythroblastopenia in Childhood.

Bone marrows of hospitalized children with anaemia were examined to study the incidence and profile of erythroblastopenia in childhood. Forty children aged between 7 months to 12 years with anaemia, reticulocytopenia and isolated erythroblastopenia on Bone marrow aspiration were evaluated during 2 years duration. Depending on duration of illness and their recovery the disease was categorized into acute, subacute and chronic erythroblastopenia. The disease was found to be associated with PEM and nutritional anaemia (55%), gastroenteritis (12.5%) and respiratory tract infections (12.5%) amongst others.

Inflammatory psuedo-tumor of the spleen.

Inflammatory pseudo-tumor of spleen, a benign reactive lesion of unknown etiology and pathogenesis, is extremely rare with isolated case reports in literature. These are usually misdiagnosed preoperatively, both clinically and radiologically; metastasis or lymphoproliferative disorders with pathological studies allow reliable diagnosis of the disease. We report the unusual occurrence of this lesion in the spleen.

HER-2/neu expression in lesions of uterine cervix: Is it reliable and consistent.

This study was conducted to evaluate the expression of HER-2/neu oncogene in the lesions of the uterine cervix and to determine its correlation with histological type of malignancy, grade and clinical stage of presentation. One hundred cervical specimens were included in this study. These comprised cases with diagnosis of benign epithelial lesions, squamous cell carcinoma, adenocarcinoma, carcinoma cervix with glandular differentiation and cervical intraepithelial neoplasia. HER-2/neu immunostaining was performed by streptovidin-biotin peroxidase method. Higher expression of HER-2/neu was noted in malignant lesions as compared to benign lesions. Intensity of staining also correlated with clinical stage of presentation, lymph node metastasis and presence of parametrial extension. The over-expression of HER-2 oncoprotein is associated with poor prognosis, metastatic potential and aggressive biological behavior.

Congenital lymphangioma circumscriptum of the vulva.

Lymphangioma circumscriptum of the vulva is a disorder of lymphatic channels involving deep dermal tissues. Most of these cases are confused with genital warts leading to improper diagnosis and treatment. We present a three years young female child who had multiple skin colored papular lesions over the genitals. Skin biopsy revealed features of lymphangioma circumscriptum.

Immunohistochemical study of the expression of HER-2/neu oncogene in ovarian lesions.

To evaluate the expression of HER-2/neu in various ovarian lesions, 75 cases of ovarian tissues (25 cases of benign lesions and 50 cases of carcinoma) were studied in the department of pathology, Pt. B.D. Sharma PGIMS, Rohtak. Besides conventional H&E staining, representative sections were processed for immunohistochemical staining for HER-2/neu detection. Two (8%) of benign cases were 1+ positive and 19 (38.0%) of malignant lesions were positive for HER-2/neu staining with intensity 1+/2+ in 16 cases (32.0%), 3+ in 2 cases (4%) and 4+ in 1 case (2%). It was observed that HER-2/neu expression was significantly associated with high grade ovarian tumours, however intensity of positivity did not correlate with the grade of tumour.

Transferrin receptor expression in reactive and neoplastic lesions of lymphnodes.

Transferrin, the major iron binding protein in human plasma transports iron to various tissues. The first step in cellular iron uptake is binding of transferrin complex to the cell surface membrane by specific molecule known as transferrin receptors. Transferrin receptors are found in limited sites in normal tissues, in contrast, the receptors are widely distributed in majority of carcinomas and sarcomas. Presence of increased transferrin receptors implies a stage of moderate or less differentiation corresponding to elevated proliferative activity and therefore, has a prognostic value. Demonstration of transferrin receptors and its distribution pattern within a tumour as well as its quantitative determination can provide data helpful for, both, an additional understanding of tumour biology and as an approach for planning therapy. In present study, we analysed 60 cases, 30 each of reactive lymphadenitis and lymphomas for transferrin receptors using immunohistochemical technique (DAKO, Code-K0673). Grade II and Grade III intensity was recorded in the germinal centers and the histiocytes in sinus histocytosis indicating the proliferating cells and activated histocytes. Most of the low grade non-Hodgkin lymphomas (83.66%) showed weak (Grade I) positivity for transferrin receptors. Intermediate grade lymphomas showed moderate (Grade II) to high intensity (Grade III) for transferrin receptors (57.14% and 42.85%) respectively. Seventy five percent of high grade lymphomas showed strong (Grade III) positivity. All the 9 cases of Hodgkin lymphoma (100%) showed grade III positivity. Proportion of the cells within a tumour expressing transferrin receptors in high density are therefore likely to represent the growth fraction of the tumour.

Infected primary intramuscular echinococcosis of thigh.

An unusual case of infected primary echinococcosis involving quadriceps muscles of the thigh and presenting as a cystic mass in a child is being reported. The diagnosis was made preoperatively with ultrasound examination and serology. After a cover of medical treatment the cyst was excised. Various clinical possibilities and management strategies are discussed.

Extraskeletal chondroma of the hand--a case report.

Extraskeletal chondroma is a rare entity. A fairly benign condition, it is usually seen in adults. It presents as an enlarging mass, most commonly in the hand. Local excision is the treatment of choice. We present a case of extraskeletal chondroma of hand in a 12 year male child. Its variable histological appearance not infrequently leads to a mistaken diagnosis of chondrosarcoma.

Malignant Brenner tumour--a case report.

Malignant Brenner tumour is a rare pathological entity. Apart from identification of typical benign, metaplastic and/or proliferating components, stromal invasion must be observed for diagnosis of Brenner tumour. A case of malignant Brenner tumour is described along with a brief review of strict criteria of diagnosis and its biological behaviour.

Cystic lymphangioma of the spleen--a case report.

Splenic cystic lymphangioma is a very rare condition. It occurs commonly in children in whom it is often an incidental imaging finding. In the absence of histologic confirmation, it usually mimics hydatid disease and other cystic vascular proliferations of the spleen. We present a case of cystic lymphangioma of the spleen in 15 years old female.

Pancreatoblastoma (infantile pancreatic carcinoma)--a case report.

Pancreatoblastoma or infantile pancreatic carcinoma is a rare pancreatic tumor with distinct acinar and squamoid cell differentiation that generally affects infants and young children. Ultrasound and CT scan may be useful but preoperative diagnosis is often quite difficult. The outcome is generally favourable. A such case of 10 years old boy with an abdominal mass is being presented.

Significance of mucin stain in differentiating benign and malignant lesions of prostate.

In an attempt to assess the significance of mucin stains in benign and malignant lesions of prostate, 200 prostatic biopsies in the department of Pathology, Pt. B.D. Sharma PGIMS, Rohtak were studied. All the biopsies were subjected to PAS and alcian blue staining along with H&E stain. Neutral mucin (PAS positive) was more frequently observed in benign prostatic lesions (93.3%) as compared to carcinoma (36%) while acid mucin (AB positive) was found more in carcinoma prostate (68%) as compared to benign prostatic lesions (16%). The positivity for acid mucin was more in well differentiating tumour decreasing significantly in high grade malignancies. With both types of mucin, luminal positivity was slightly more as compared to cytoplasmic positivity.

Solitary amyloid tumour of breast--a case report.

45 years old woman with solitary amyloid tumor of breast is described. Clinically, the breast mass was hard and was suspicious of carcinoma. Microscopically there was no neoplasia. Instead there was extensive fibrosis with amyloid deposition in perivascular periductal areas and also in intervening stroma. The presence of amyloid was confirmed by Special stains and by Polarised microscope. Investigations revealed no evidence of systemic amyloidosis or amyloid related illness.

Mesenchymal hamartoma of the liver--a case report.

Mesenchymal hamartoma of the liver is a rare benign tumor of infancy and childhood. A case of mesenchymal hamartoma of liver seen in a 37 day old child is reported and the available literature on the subject is briefly reviewed.

Congenital fibrolipoma of anal canal.

Although rare, congenital lipomatosis presents during first few months of life as large sub-cutaneous fatty masses on chest with extension into skeletal muscle bundles. Only a few such cases have been reported in the literature. A rare case of congenital fibrolipoma of anal canal in a 3-day-old male child is being documented in the present report.

Odontogenic myxoma of the mandible: a case report.

A case of myxoma of mandible in a 23 years old female patient is described. The case was treated with enbloc resection and bone graft from iliac crest. No recurrence is reported after 4 years follow up.

Primary malignant lymphoma of the breast.

Primary malignant lymphomas of the breast (PBL) are uncommon constituting only 0.04 to 0.53% of malignant breast neoplasms. We wish to report the clinical, cytological and histologic findings of PBL diagnosed in a 52 years old female.