Intestinal atresia accounts for approximately one third of all cases of neonatal intestinal obstruction. There is controversy regarding pathogenesis of congenital atresia and stenosis of small bowel. Studies regarding clinical manifestations and specific histopathological features of neonatal intestinal atresia are scarce in Indian literature.
To understand the histomorphological features and thus suggest pathophysiology of cases with Intestinal Atresia.
Materials and Methods
Out of 147 cases, of intestinal obstruction in newborn studied over a period of 5 years, 39 cases of intestinal atresia were found. Their histomorphological details with clinical manifestations were studied.
Type II was the commonest type of atresia. Associated anomalies noted were gastroschisis, volvulus, anal stenosis, microcolon, annular pancreas, meconium cyst and duplication cyst. Histological changes observed were ulceration, flattening, abnormal villous configuration, luminal obliteration, narrowing, haemangiomatous proliferation of blood vessels, fibrosis, haemorrhage, calcification, and mesenchymal condensation around the blood vessels. Gangrene and perforation has also noted in some cases.
An intrauterine intestinal ischemia due to vascular pathology followed by resorption of the bowel is the possible explanation for the development of intestinal atresia.
Histomorphology; Clinical features; Pathogenesis
The clinical presentation of metastatic renal cell carcinoma to ovary is extremely rare as well as confusing due to its close resemblance to primary ovarian tumors, especially clear cell carcinoma. We present a case of metastatic renal cell carcinoma diagnosed in a 48-year-old female, who had renal cell carcinoma of the right kidney and right sphenoid wing meningioma of transitional type.
Ovary; metastatic renal cell carcinoma; meningioma
Spindle cell rhabdomyosarcoma is a rare variant of embryonal rhabdomyosarcoma that affects young males and most commonly involves the paratesticular region. We report a case of paratesticular spindle cell rhabdomyosarcoma in a 14-year-old boy, who presented with a painless scrotal mass. Left inguinal orchidectomy was performed. Histopathological and immunohistochemical examination of the mass revealed spindle cell rhabdomyosarcoma of the paratesticular region.
Rhabdomyosarcoma; scrotum; immunohistochemistry
Pediatric myelofibrosis is a rare disorder. It is usually secondary to other diseases. Rarely, when no underlying cause is found, it is termed idiopathic. We present here, a rare case of idiopathic myelofibrosis in a 10 year old male child. Bone marrow aspirate was dilute. Bone biopsy showed marrow fibrosis, with grade 2–3 reticulin fibres, with no evidence of granuloma, parasite or infilterative disorder. Acid fast bacillus stain was negative. Iliac lymph node biopsy showed reactive sinus histiocytosis with extramedullary hematopoeisis. Thus, diagnosis of pediatric idiopathic primary myelofibrosis was made. Idiopathic pediatric myelofibrosis should be suspected in a child with progressive pallor, hepatosplenomegaly and dry tap on bone marrow aspiration and marrow fibrosis on bone biopsy, after exclusion of secondary causes.
Pediatric; Idiopathic; Myelofibrosis
The purpose of this study was to evaluate the expression of cdk4 and p16, the proteins implicated in hyperproliferation and arrest in oral lichen planus and to compare their expression in erosive and non-erosive oral lichen planus and with normal mucosa and oral squamous cell carcinoma.
Material and Methods
Analysis of cdk4 and p16 expression was done in 43 erosive oral lichen planus (EOLP) and 17 non-erosive oral lichen planus (NOLP) cases, 10 normal mucosa and 10 oral squamous cell carcinoma (OSCC) cases with immunohistochemistry.
This study demonstrated a significantly increased expression of cytoplasmic cdk4 (80% cases, cells stained - 19.6%), and cytoplasmic p16 (68.3% cases, cells stained - 16.4%) in oral lichen planus (OLP) compared to normal mucosa. cdk4 was much higher in OSCC in both cytoplasm and nuclei compared to normal mucosa. Also, while comparing OLP with positive control, significant difference was noted for cdk4 and p16, with expression being more in OSCC. While comparing EOLP with NOLP; significant differences were seen for cdk4 cytoplasmic staining only, for number of cases with positive staining as well as number of cells stained.
Overexpression of cytoplasmic cdk4 and p16 was registered in oral lichen planus, however considerably lower than in squamous cell carcinoma. Erosive oral lichen planus demonstrated overexpression of cytoplasmic cdk4 and premalignant nature compared to non-erosive lesion. Therefore there is an obvious possibility for cytoplasmic expression of cdk4 and p16 to predict malignant potential of oral lichen planus lesions.
cell cycle checkpoints; immunohistochemistry; lichen planus
Intracranial fungal granulomas can be misdiagnosed clinically and radiologically as neoplastic lesions. They also rarely occur without any history of immunodeficiency or diabetes. We report two such cases of fungal granulomas that were unsuspected clinically and radiologically but were detected on intraoperative squash cytology (SC) and later confirmed on histopathology. Timely intervention was hence possible and patient was saved from the hazards of unnecessary removal of eloquent areas of brain and was shifted on proper medical management. SC remains an indispensible tool for the neurosurgeon to get a provisional intraoperative diagnosis and in such surprising scenarios, change the surgical management of the patient, save the resection of eloquent brain areas and begin immediate postoperative medical management. These cases are being presented for their rarity and for highlighting the importance of SC as a regular tool for intraoperative neurosurgical consultation of intracranial mass lesions.
Fungal mass lesions; Granuloma; Intraoperative cytology; Neurosurgery; Squash cytology
Eccrine syringofibroadenoma (ESFA) is a rare, benign tumor of eccrine sweat gland origin that usually presents as a nodule on the extremities of an elderly person. It can also present as an ulcerative plaque, verrucous lesion, papular or nodular lesion or as palmoplantar keratoderma. Although the clinical features are variable, histology is characteristic in the form of anastomosing strands, cords and columns of epithelial cells embedded in a fibrovascular stroma. We report the case of a 62-year-old male with a nodular lesion on the extremity that caused secondary involvement of the nail in the form of complete nail dystrophy. The histology showed features consistent with ESFA. Nail involvement by ESFA is a rare presentation and is rarely described in the literature.
Eccrine syringofibroadenoma; fibroepithelioma of Pinkus; schopf syndrome
Port-wine stains (PWSs) are congenital vascular lesions caused by progressive ectasia of blood vessels located in the vascular plexus of the dermis. Acquired PWSs develop later in life but are identical in morphology and histology to the congenital PWSs. Less than 75 cases of acquired PWSs have been reported in the published literature, of which there has not been a single report from India so far. Various factors have been proposed for its pathogenesis like trauma, actinic exposure, drugs, tumors, and herpes zoster infection. We report an acquired port-wine stain in a 41-year-old male. The causative factors, treatment, and previous reports of this uncommon entity have also been reviewed.
Adult; acquired; port-wine stain
Frictional lichenoid dermatitis.
Frictional lichenoid dermatitis (FLE) is an entity that is probably under diagnosed and has been variably associated with either friction and/or atopy with a distinctive seasonal variation.
Aims and Objectives:
To study correlation of FLE with UV index and to assess its association with atopic dermatitis.
Materials and Methods:
A cross sectional analysis of children with FLE was done, over a period of 6 years in two tertiary hospitals. A detailed history and examination was done to assess the features of atopic dermatitis. The number of cases seen per month was compared with the mean monthly UV index. Two-tailed significance tests using Pearson's coefficient of correlation and T-test were used to interpret the data. (P < 0.05).
One hundred seventy-four patients were studied using the UKC criterion 17.2% of the patients had AD while xerosis (40.3%) was the predominant cutaneous finding. The number of patients seen in summer was more than in winter (P < 0.05) but there was no statistical difference between the cases in winter and spring. There was a significant correlation of the number of cases per month with UV index (P = 0.019). Almost 42% of patients gave a history of recurrence.
FLE is probably not associated with atopic dermatitis and is likely to be related to the ambient UV index though a larger cohort with meticulous follow up may be needed to draw a final conclusion.
Statistical Analysis Used:
The Pearson's coefficient of correlation was used for comparing the cases per month with the UV index. The tests of hypothesis used included the paired T-tests. F-test of variance, Welch test, Wilcoxon rank sum test and the Kolmogorov-Smirnov Test. P < 0.05 was considered significant.
Atopic dermatitis; frictional lichenoid dermatitis; seasonal variation; summer; Sutton's prurigo; UV Index; UV light
Clear cell sarcoma of kidney (CCSK) is an aggressive renal neoplasm. We report two boys aged three and half, and three years with CCSK, one of whom had a disease free survival of four years and eight months. These patients were managed with surgery, chemotherapy and radiotherapy. One of the patients discontinued treatment early and lost to follow up. Aggressive multimodality therapy is the keystone to improved outcome.
Clear cell sarcoma; Kidney; Wilm's tumor
One-third of schwannomas occur in head and neck region, mostly in the parapharyngeal region. Cystic change is seen in only 4% of schwannomas. The diagnosis of such tumors remains a challenge due to the rarity of these lesions and limited utility of fine needle aspiration cytology. When cystic, branchial cleft cyst is an important differential diagnosis more so due to radiological resemblance. We present a case of 42-year-old male with left sided neck mass masquerading as branchial cleft cyst clinically and radiologically. Multiple sections examined from the cystic areas of the mass revealed lymphoid aggregates beneath the cyst wall in addition to the schwannomatous areas.
cystic schwannoma; branchial cyst; lymphoid aggregates
Griscelli syndrome 2 is a rare autosomal recessive disorder of pigmentary dilution of hair, skin, splenohepatomegaly, pancytopenia, immune and neurologic dysfunction. Clinical course is characterized by recurrent infection triggered by uncontrolled T-lymphocyte and macrophage activation, called hemophagocytic syndrome. Since the primary presentation is with depigmented hair, we attempt to highlight diagnostic difficulties in such cases in developing countries like ours where pigmentary changes in hair and skin are commonly attributed to severe malnutrition. We also evaluated phenotype of all 10 cases of genotype (c.C550T; p.R184X), collected from published literature worldwide and emphasize the potential role of above mutation as hotspot in Southeast Asian region.
Griscelli syndrome; Kala Azar; mutation
Rhabdoid tumors of extrarenal origin are uncommon. This is the first report of rhabdoid tumor presenting as a perineal mass. Furthermore, it is a disease of childhood, with adult presentation being rare. We report a case of extrarenal rhabdoid tumor and discuss its presentation and possible treatment options.
Rhabdoid tumor; Extrarenal rhabdoid; Perineal tumor
SNNL is a rare, benign lesion which may arise from trauma, a sclerosing haemangioma or it may represent a burned out phase of various conditions like parasitic infections, or chronic infections like tuberculosis. Histopathology provides the definite diagnosis, which thus helps clinicians in allaying unnecessary anxiety of the patient and in planning proper management.We are reporting two cases here, which had solitary necrotic nodules of liver (SNNL), with emphasis on the aetiology and morphology of the lesions. SNNL was incidentally detected during cholecystectomy done in both young female patients. The lesion was solitary in one case and it was multiple in the other. The suspected aetiology in one of the cases was parasitic, whereas it was tuberculosis in the other. The clinical features in both cases were suggestive of neoplasms.
Solitary necrotic nodule; Liver
This is a rare case of a 35 year old multiparous lady with complaints of postcoital bleeding and foul smelling discharge for 3 months. On per speculum examination, a large irregular friable growth was seen which used to bleed on touch. A provisional diagnosis of carcinoma cervix was made, but the cervical biopsy revealed granulomatous inflammation with caseation, consistent with the diagnosis of cervical tuberculosis. The patient responded well to 6 months of antitubercular treatment. Hence, tuberculosis should be kept as a differential diagnosis of carcinoma cervix.
Carcinoma; cervix; tuberculosis
Leiomyosarcomas of soft tissue are the rare tumors and the retroperitoneum is the most common site involved. We report a case of primary leiomyosarcoma of the peritoneal cavity which clinically presented with suprapubic, freely mobile, nontender mass which measured 10×10 cm in size. Contrast enhanced computed tomography revealed well defined heterogenous hypodense solid cystic mass. The mass was surgically excised out in its entirety. The histopathological examination revealed spindle cells arranged in alternating fascicles having pleomorphic nuclei, indistinct margin and eosinophilic cytoplasm with foci of haemorrhage, necrosis and 5-6 mitosis/HPF. The spindle cells were immunoreactive for smooth muscle actin, desmin and negative for S-100, CD-34 and c-kit. Histopathology and immunohistochemistry were helpful in making the final confirmatory diagnosis. Leiomyosarcomas are aggressive tumors, with poor prognosis and often difficult to treat. The survival rates are lowest among all soft tissue sarcomas.
tumor; sarcoma; soft tissue
Multifocal carcinoid is a known phenomenon. We present a rare combination of an ovarian carcinoid synchronous with gallbladder carcinoid. This rare combination has not been reported so far. An asymptomatic 45-years-old perimenopausal lady was diagnosed to have a metastatic ovarian cancer, but on laparotomy she was found to have a primary synchronous metastatic gall bladder as well. On histopathological evaluation she was found to have two separate primary carcinoids. Subsequently the patient received chemotherapy and is completely asymptomatic on follow up. Further research needs to be undertaken and guidelines need to be formulated for management of these cases.
We have discussed the case of a solitary polypoid ganglioneuroma in a 45–year–old male patient, at the base of tongue, which mimicked a malignancy. The interest of this case lay in the rarity of its incidence at the base of tongue and its gross resemblance to the more common malignant polypoidal growths at this site. The presence of neural elements and ganglion cells at this site makes it important for an inexperienced histopathologist to differentiate it from other neural lesions of the tongue, in order to avoid a misdiagnosis and this can prevent the clinician from administering an inappropriate treatment. Exhaustive search revealed only a very few case reports on the tongue.
Polypoidal; Tongue; Ganglion; Immunohistochemistry
Isolated soft tissue cysticercosis of the trunk in the absence of concurrent central nervous system involvement is uncommon and may be difficult to diagnose. We report 4 such cases in the pediatric age group. Preoperative diagnosis of soft tissue cysticercosis was considered only in 1 patient, the rest were diagnosed only after biopsy. Complete excision (without rupture) was done. All of them underwent a CT scan head along with ophthalmic examination to rule out the more common sites of occurrence of cysticercosis. No further treatment was undertaken as the evaluation was negative. In endemic areas like ours we must suspect this entity not only in the limb muscles, but also in the subcutaneous tissues of the trunk. If diagnosed accurately, it can be treated medically, eliminating the need for surgery.
Cysticercosis; Taenia solium; Parasitic cyst
A 22 year old male psoriatic patient presented with multiple reddish scaly plaques all over body. After hematological and biochemical investigations the patient was started on oral methotrexate 15 mg weekly. PASI score at the start of treatment was 26.2. After 3 months PASI dropped to 11.5, the dose of methotrexate was tapered to 7.5mg weekly and the patient was maintained on this dose and kept under monthly follow up. Four months later, the patient presented with reddish to hyperpigmented raised firm nodules having a central crater over the healing plaques of psoriasis. Few lesions showed self resolution over a period of 6-12 weeks. Histopathology of the lesion confirmed it to be Keratoacanthoma. We believe the most likely etiologic factors for the multiple KAs in our patient could be a genetic susceptibility stimulated by multiple causes.
Keratoacanthoma; Psoriasis; Spontaneous; Healing
Pityriasis rosea (PR) is an acute or subacute inflammatory skin disease characterized by erythematous papulosquamous eruptions localized on the trunk and arms. The eruptions are self-limiting and usually disappear gradually in 2-10 weeks, without any treatment. Typical PR is much easier to diagnose than the rare atypical forms. There is a passing mention of PR with erythema multiforme-like lesions in the literature, but no extensive case series have been published till date. We present a series of five patients for whom we believe atypical PR is the likely diagnosis.
Erythema multiforme; papulosquamous eruption; pityriasis rosea
A case of hydatid cyst within a congenital cystic adenomatoid malformation (CCAM) of the right lower lobe of lung in an 8-year-old girl is reported. Presence of CCAM was confirmed on histopathology of the lung tissue attached to the specimen.
Hydatid cyst; Congenital cystic adenomatoid malformation; Pericystectomy
Gastrointestinal stromal tumours (GIST) are rare in paediatric patients and have a discrete clinicopathological and molecular divergence from that observed in adults. In the present report we present a case of a 2-month-old female in whom colonic gastrointestinal stromal tumour acted as a lead point of colocolic intussusception. Laparoscopically assisted reduction of the intussusception and resection of tumour was done.
Gastrointestinal stromal tumour; Colonic intussusception; Child
Fetus in fetu is an uncommon anomaly in pediatric age group. There have been controversies regarding its differentiation from well differentiated teratoma. While presence of axial skeleton has been considered mandatory for diagnosis, there are many reports of fetus in fetu without any vertebral column. It has been suggested that the two entities form a spectrum of lesions depending upon the timing of embryological insult. The present case is another example of a lesion that fits in this spectrum of interesting lesion.
Fetus in fetu; Retroperitoneal teratoma
A child with isolated renal mucormycosis who was successfully treated with systemic anti-fungal therapy and nephrectomy is reported.
Amphotericin B; isolated renal mucormycosis; nephrectomy; renal zygomycosis