Profile of Non-Hematological Pediatric Tumors: A Clinicopathological Study at a Tertiary Health Care Centre.

BACKGROUND: Tumors in pediatric age group are biologically different from tumors in adults and emerging as a significant contributor to morbidity/mortality in children. OBJECTIVES: This study evaluated the epidemiological profile of pediatric solid tumors in 0-18 years of age over a period of 5 years in a general hospital in India. RESULTS: A total of 510 cases of tumors were included, which accounted for 5.1% of the total tumor burden of the hospital. Benign tumors were more common than malignant tumors. Most common age group affected was 14-18 years. Among benign tumors, vascular tumors were the most common, while malignant bone tumors outnumbered all other malignant tumors. Neuroblastomas comprised only 0.9% of our malignancies. CONCLUSION: Our study provides an insight into the patterns of childhood tumors for which exists little literature in India. With lack of a dedicated pediatric cancer registry from India, tracking of the diverse histological spectrum of tumors is difficult.

Vitamin B12 supplement alleviates N'-nitrosodimethylamine-induced hepatic fibrosis in rats.

Abstract Context: Altered vitamin B12 levels have been correlated with hepatotoxicity; however, further evidence is required to establish its protective role. Objective: To evaluate the effects of vitamin B12 supplement in protecting N'-nitrosodimethylamine (NDMA)-induced hepatic fibrosis in Wistar rats. Materials and methods: Hepatic fibrosis was induced by administering NDMA in doses of 10 mg/kg body weight thrice a week for 21 days. Another group received equal doses (10 mg/kg body weight) of vitamin B12 subsequent to NDMA treatment. Animals from either group were sacrificed weekly from the start of the treatment along with their respective controls. Progression of hepatic fibrosis, in addition to the effect of vitamin B12, was assessed biochemically for liver function biomarkers, liver glycogen, hydroxyproline (HP) and B12 reserves along with histopathologically by hematoxylin and eosin (H & E) as well immunohistochemical staining for α-SMA expression. Results and discussion: Elevation in the levels of aminotransferases, SALP, total bilirubin and HP was observed in NDMA treated rats, which was concomitant with remarkable depletion in liver glycogen and B12 reserves (p < 0.05). Liver biopsies also demonstrated disrupted lobular architecture, collagen amassing and intense fibrosis by NDMA treatment. Immunohistochemical staining showed the presence of activated stellate cells that was dramatically increased up to day 21 in fibrotic rats. Following vitamin B12 treatment, liver function biomarkers, glycogen contents and hepatic vitamin B12 reserves were restored in fibrotic rats, significantly. Vitamin B12 administration also facilitated restoration of normal liver architecture. Conclusion: These findings provide interesting new evidence in favor of protective role for vitamin B12 against NDMA-induced hepatic fibrosis in rats.

Surgical resection of orbital primitive neuroectodermal tumor without adjuvant chemotherapy.

Primitive neuroectodermal tumor (PNET) of the orbit is a very rare entity. We report a case of a 10-year-old boy having progressive protrusion of the eyeball, restricted ocular motility, ptosis, and ocular pain for 2 months. On examination, a fixed, nontender, firm, hyperemic, nonpulsatile globular mass was palpated in the superomedial quadrant of the orbit. Computed tomography scans of the orbit depicted a well-defined, soft tissue density lesion within the orbit without evidence of any bone defect or erosion. Surgical resection of the encapsulated lesion through anterior orbitotomy followed by histopathology and immunohistochemistry examination confirmed the diagnoses of PNET. The patient has been on regular follow-up for 2 years and has shown no sign of recurrence.

Grossing and reporting of bone tumor specimens in surgical oncology: Rationale with current evidence and recent updates.

Primary bone tumors, including sarcomas, are rare tumors and require a multidisciplinary approach, including inputs from a radiologist, pathologist, medical oncologist, and surgical and radiation oncologist, for optimal management. Over the years, there has been a paradigm shift toward the treatment of bone sarcomas, from radical resections to conservative surgical procedures, to achieve improved clinical and functional outcomes. This has led to receiving and processing various types of specimens in orthopedic oncopathology. Grossing and reporting of bone tumors require expertise. This review focuses upon the types of biopsies, grossing techniques of various specimens in orthopedic oncology and reporting, with rationale and recommendations from pathologists, actively involved in reporting and pursuing a special interest in bone tumors, based on current evidence. Furthermore, there is a section on some of the updates in the diagnosis of bone tumors, based on the recent fifth edition of the World Health Organization classification of tumors of soft tissues and bone.

Cytohistomorphological grading of breast carcinoma with special reference to apoptotic rates and lymph node metastasis.

To analyze the significance of comparative evaluation of cytohistomorphological grading of infiltrating ductal carcinoma with specific reference to lymphnode metastasis status and apoptotic index. 50 patients who underwent FNAC and mastectomy for infiltrating ductal carcinoma were included in the study. Concordance between cytological and histological grades was calculated. Cytological smears were also evaluated for apoptotic rates and lymph node metastasis and then compared with the histological grades using regression analysis. Histological and cytological grades were comparable and statistically significant difference was found in the lymphnode metastasis rate and apoptotic index in the three cytological grades of the tumor. Moreover, by considering the apoptotic rates, the sensitivity of cytological grading significantly rose in relation to histological grade. With histological grade taken as the standard, cytology was found to be comparable but less sensitive for grading infiltrating ductal carcinoma. However, by considering lymphnode status and apoptotic rates as calculated on cytology, the sensitivity of cytological grading rose significantly in relation to histological grade. Therefore, apoptotic index incorporated with cytological grade may provide relevant information on the aggressiveness of invasive ductal carcinoma of breast and could be a useful parameter to take into consideration when selecting neo-adjuvant therapy.

Giant primary mesenteric liposarcoma: a rare case report.

Primary mesenteric liposarcoma is a rare malignant tumor of mesenchymal origin. These tumors are often found to be of substantial size upon first clinical presentation. We report a case of primary mesenteric liposarcoma in a 55 year old man who underwent laparotomy for the clinical/radiological impression of a mesenteric cyst. FNAC was inconclusive due to suboptimal cellularity. On laparotomy there were 2 masses (diameter- 19 and 14 cms) weighing approximately 8.5 kilograms together. Histopathology showed features of atypical lipomatous tumor / well differentiated liposarcoma (ALT/WDLS) with mixed histological pattern (e.g. lipoma like, sclerosing and myxoid areas) varying from area to area. Prognosis of ALT/WD liposarcoma depends upon its anatomic location and most aggressive histological subtype. This case is being presented because of its huge dimensions, rarity of the site and mixed histological pattern.

Significance of AgNOR score in benign and malignant soft tissue tumours.

AgNOR staining was employed on FNAC and histopathological sections obtained from patients with soft tissue tumours. The study comprised of 20 normal appearing soft tissues, 74 benign and 36 malignant soft tissue tumours. The slides were stained with AgNOR in order to differentiate between benign and malignant soft tissue tumours. The mean AgNOR count in normal appearing soft tissues, benign lesions and malignant lesions was 1.04+/-0.10 (0.94-1.14), 1.51+/-0.21 (1.1-2.1) and 4.96+/-1.33 (2.57-7.21) respectively. The mean AgNOR count was found to be higher in benign soft tissue tumours as compared to normal appearing soft tissues and the difference was found to be statistically significant. The mean AgNOR count in soft tissue sarcomas was found to be higher as compared to both normal appearing soft tissues and benign soft tissue tumours and the results were found to be statistically significant. The increased AgNOR score in both benign and malignant soft tissue tumours as compared to normal appearing soft tissues indicates high proliferative activity. Thus AgNOR staining is a simple and useful method for estimating tumour cell proliferation thereby differentiating normal appearing soft tissues from benign and malignant soft tissue tumours.

Recurrent Dermatofibrosarcoma Protuberance and its Management with Radical Excision and Interval Skin Grafting: A Case Report.

Dermatofibrosarcoma protuberans (DFSP) is very rare tumor of dermis layer of skin with the incidence of only 1 case per million per year. DFSP rarely leads to a metastasis (Less than 5% have metastasis), but DFSP can recur locally. We publish a rare case of a recurrent dermatofibrosarcoma protuberans and its management with radical excision and interval skin grafting.

Myopericytoma of lip: a rare lesion in an unusual location.

Myopericytoma is a rare benign tumor with perivascular myoid differentiation. It shares histomorpholoic features with the so-called hemangiopericytoma, myofibroma and glomus tumor. We hereby report the case of a 14-year-old boy who presented with a slowly growing, painless, firm mass on upper lip, diagnosed as myopericytoma on the basis of histopathology and immunohistochemistry. To the best of our knowledge, this is only the second such reported case.

Cytodiagnosis of yolk sac tumor.

To analyze cytomorphologic features of yolk sac tumors of childhood. Four cases of pediatric yolk sac tumor (YST), diagnosed by fine needle aspiration cytology were reviewed (1998-2002). Age of patients ranged from 1(1/2) to 5 years. Three cases presented clinically with an intra-abdominal mass while one case presented with a testicular mass. Fine needle aspirates had been obtained directly as well as under radiologic (USG/CT) guidance. Smears were stained with H & E and Papanicolaou stain. In all cases values of serum alpha-fetoprotein and hCG were available preoperatively. Histopathologic diagnosis was correlated with cytologic findings in all the cases. Cytologic examination showed richly cellular smears with a combination of morphological patterns. Characteristically, tumor cells were arranged in papillary groups, tight cell clusters and formed acinar structures. Cells showed enlarged, moderately pleomorphic, hyperchromatic nuclei and moderate amount of cytoplasm, some of which displayed cytoplasmic vacuolation, displacing the nuclei eccentrically. Preoperatively, serum alpha-fetoprotein level was raised in all cases. Histopathology confirmed the cytologic diagnosis. Yolk sac tumor is common among the germ cell tumors of pediatric age group which presents a spectrum of cytomorphologic features having important differences with other germ cell neoplasm, e.g. embryonal carcinoma. Clinicoradiologic features and tumor markers are additionally helpful for an accurate cytologic diagnosis.

Coexistence of giant cell fibroblastoma and encephalocele.

Giant cell fibroblastoma (GCF) is a rare soft tissue tumour that occurs almost exclusively in children younger than 10 years of age and is mostly located in the superficial soft tissues of the back and thighs. We present a rare case of GCF with encephalocele in a 1.5-year-old boy who presented with a swelling in the occipital area of the scalp since birth. CT scan suggested encephalocele without any suspicion of a mass lesion. On histopathology, an ill-defined proliferation of fibroblasts in a heavily collagenised and focally myxoid stroma was seen containing numerous multinucleated cells having a floret-like appearance along with mature glial tissue bordering a cystic space. Immunohistochemically, the stromal cells were positive for both, vimentin (diffuse) and CD34 (focal) thereby confirming the histological diagnosis of GCF. This case highlights the unusual coexistence of GCF with congenital defects and its histogenetic resemblance to dermatofibrosarcoma protuberans.

Reticular schwannoma mimicking myxoid sarcoma.

Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft tissue is extremely rare. We report a case of reticular/microcystic variant of schwannoma in a 55-year-old Indian woman who presented with a painless slow-growing swelling in the right forearm for the past 6 months. MRI findings suggested myxoid sarcoma. However, histological and immunohistochemical findings in the excised specimen were consistent with reticular/microcystic variant of schwannoma. This case report emphasises that the diagnosis of reticular/microcystic schwannoma should always be considered in cases where myxoid sarcomas are suspected as it can mimic malignant myxoid sarcomas, clinically and radiologically, thereby avoiding aggressive intervention and overtreatment.

Sclerosing stromal tumour of the ovary presenting as precocious puberty: a rare neoplasm.

A 7-year-old Indian girl presented with symptoms of excessive development of breasts, early menarche, growth of pubic hairs, accelerated growth and abdominal distension. On clinical examination, a large right abdominopelvic mass was palpable. MRI revealed a large, heterogeneous, solid and cystic tumour in the right adnexal region, suggestive of an ovarian neoplasm. The hormonal profile showed markedly elevated oestradiol and low follicle-stimulating hormone levels. Clinical diagnosis of precocious puberty with right ovarian mass was concluded. Right-sided salpingo-oophorectomy was performed. Histopathology showed features consistent with sclerosing stromal tumour of the ovary. Postoperatively, signs and symptoms of precocity gradually regressed and her serum oestradiol level came down to normal. This is the first reported case from India.

Incidentally discovered goblet cell carcinoid clinically presenting as acute intestinal obstruction: a case report with review of literature.

Goblet cell carcinoid (GCC) is a rare variant of carcinoid tumor that exclusively involves the appendix. It usually occurs in 5th-6th decade with the most common clinical presentation being acute appendicitis. The natural history of this tumor is intermediate between carcinoids and adenocarcinomas. We here report a case of GCC diagnosed incidentally in a patient presenting with acute intestinal obstruction. Ultrasonographic examination supported the clinical diagnosis of acute intestinal obstruction, following which the patient underwent laparotomy and resection of ileum along with appendix was done. On gross pathological examination, a nodular growth was present on the tip and body of appendix that was yellow in color with a semi-solid to mucoid consistency on cut section. On microscopy, lakes of mucin with few acinar structures floating in them were seen. The submucosa as well as serosa were infiltrated by clusters of goblet cells and well-formed acini, with little atypia. Glands and nests were positive for periodic acid Schiff and immunohistochemistry showed focal chromogranin positivity in glandular structures, thereby confirming the diagnosis of GCC. Although the prognosis of GCC is better than adenocarcinomas, it is one of the carcinoids having a poorer outcome when compared with other variants of carcinoid tumor. Therefore, it is important to rule out other differential diagnoses of goblet cell carcinoid, the most important being mucinous adenocarcinomas.

Primary low-grade diffuse small lymphocytic lymphoma of the central nervous system.

A 45-year-old Indian woman presented in neurosurgery outpatient with seizures, headache and vomiting for the past 1 month. MRI of the brain was suggestive of a malignant central nervous system (CNS) tumour. Histological and immunohistochemical examinations of stereotactic biopsy of the tumour were diagnostic of a low-grade diffuse small lymphocytic lymphoma of the CNS. No evidence of any occult systemic lymphoma was observed, confirming its 'primary' origin in the CNS. The diagnosis of a low-grade primary CNS lymphoma (PCNSL) is difficult as clinical and neuroradiological features are wide and variable. The clinical course is more indolent than a high-grade PCNSL and thus, a less aggressive and localised targeted treatment could be sufficient rather than the high dose, neurotoxic methotrexate-based chemotherapeutic treatment, recommended for high-grade PCNSL. Histological and immunohistological confirmation is therefore mandatory for early, appropriate treatment and prognostic implications.

Hydatid cyst: unusual presentation as 'breast lump'.

Hydatid cyst in the breast is rare. We report a case of hydatid cyst in the breast in a 30-year-old Indian rural woman who presented with a lump in her left breast. MRI findings showed a cystic lesion in the breast. Histopathological examination showed features consistent with hydatid cyst with surrounding adherent pericystic breast tissue showing fibrocollagenisation and inflammatory response. ELISA for echinococcal antigen was also found to be positive. This case emphasises that preoperative diagnosis of hydatid cyst in the breast is difficult and often missed as the breast is a rare site of echinococcal infection and clinical and imaging findings are wide and non-specific. Cytological diagnosis by fine-needle aspiration may not always be possible. Therefore, hydatid cyst in the breast should always be considered in the differential diagnosis of a breast lump and histopathological confirmation is necessary for early and appropriate treatment.

Sebaceous carcinoma with apocrine differentiation: a rare entity with aggressive behavior.

Sebaceous carcinomas are rare neoplasms but have aggressive behavior. Although they can be found anywhere in the body ocular region is the most common site which comprises 75% of all cases of sebaceous carcinomas. Due to their rarity, varied histopathological features, and diverse clinical presentation, their diagnosis is often delayed, sometimes by a year. They are divided on the basis of histological differentiation into well and poorly differentiated. Apocrine differentiation is a still rarer finding and only two cases have been reported in the literature so far. We report a case of sebaceous carcinoma with apocrine differentiation in a 60-year-old male who presented with a painless swelling in right upper eyelid for 2 months which was gradually progressive. Computed tomography (CT) scan was performed and a provisional diagnosis of hemangioma was made. The mass was excised and histopathological examination revealed it to be sebaceous carcinoma. However, there were areas with decapitation secretions and granular eosinophilic cytoplasm. These were positive for cytokeratin (CK) 7 and CK 19 which confirmed their apocrine nature. Therefore, a final diagnosis of sebaceous carcinoma with apocrine differentiation was made. Thus, it can be concluded that ocular sebaceous carcinomas with apocrine differentiation are extremely rare and have significant clinical importance since they can mimic a benign lesion and the nature of surgical intervention and follow up is more aggressive than that of simple sebaceous carcinoma alone.

Role of fine needle aspiration, imprint and scrape cytology in the evaluation of intraoral lesions.

AIM: The primary goal of our study was to evaluate the value and accuracy of fine needle aspiration cytology (FNAC) in the diagnosis of various intraoral lesions and to correlate the cytological diagnosis with final histopathological findings. MATERIALS AND METHODS: Fine needle aspiration was performed in 229 patients presented with different lesions of the oral cavity at our institution. Cytological findings were then compared with final histopathological diagnosis. RESULTS: With a male to female ratio of 1.79:1, 229 patients presented with different lesions of the oral cavity were aspirated. Histopathological correlation was available in 86.9% of cases while inadequate material was obtained in 13.1% cases. The diagnostic accuracy of fine needle aspiration in diagnosing benign, pre-malignant and malignant lesions were 95.8%, 84.6% and 97% respectively. Overall the diagnostic accuracy of FNAC in diagnosing intraoral lesions was 94.9% with sensitivity and specificity of 93.2% and 96.8% respectively. CONCLUSION: FNAC of intraoral lesion is recommended as a valuable procedure for the initial evaluation of all intraoral lesions as it is simple, inexpensive, convenient and comfortable to the patient. Moreover, it can offer a rapid and accurate diagnosis for further management of the patient.

Benign mediastinal teratoma with intrapulmonary and bronchial rupture presenting with recurrent hemoptysis.

Mediastinal teratomas are usually asymptomatic tumors, located most commonly in the anterior mediastinum. Very rarely, such tumors may rupture into the tracheobronchial tree, lung, pleura or pericardium. Computed Tomography (CT) is helpful in the diagnosis and differentiation of ruptured and unruptured tumors. We report a case of ruptured anterior mediastinal teratoma in a 20-year-old female presenting with recurrent hemoptysis and cough; thus, mimicking a lung malignancy or tuberculosis. CT demonstrated a heterogeneous fat containing lesion in the anterior mediastinum with extension into the lingular lobe. Subsequent fine needle aspiration cytology (FNAC) yielded plenty of anucleate squames and debris, and a clear cut diagnosis could not be made. Total excision of the tumor was performed and histopathology of the surgically excised mass confirmed the CT diagnosis.

A case of pure endophytic squamous cell carcinoma of the gallbladder: a rare entity with aggressive behaviour.

Pure squamous cell carcinoma of gallbladder is a rare tumor accounting for only 3% of the malignancies of gallbladder. A 35-year-old female presented with pain and lump in right hypochondrium. Ultrasonography and computed tomography revealed a thickened gallbladder filled with stones without any obvious growth and a large hepatic mass. Histologically, a pure type of well differentiated squamous cell carcinoma of gallbladder was diagnosed after excluding all possible differential diagnoses owing to different histogenesis and aggressive biological behaviour. Early diagnosis is the most important parameter for improving the survival indices among the patients with squamous cell carcinoma of the gallbladder.