Rajni Yadav

Prognostic value of MIB-1, p53, epidermal growth factor receptor, and INI1 in childhood chordomas

BACKGROUND Chordomas are slow-growing tumors and most commonly involve the sacrum and clivus. Multiple recurrences are frequent. Childhood chordomas are rare and often show exceptionally aggressive behavior, resulting in short survival and a high incidence of metastatic spread. OBJECTIVE This study examined the histologic features and immunohistochemical profile of pediatric chordomas and compared them with their adult counterparts. METHODS Nine pediatric and 13 adult cases were included in the study. Childhood chordomas were classified into conventional, atypical, and poorly differentiated types. Immunohistochemistry was performed for cytokeratin, epithelial membrane antigen, vimentin, S100, brachyury, p53, INI1, epidermal growth factor receptor (EGFR), and CD117. Cytogenetic analyses were performed in a subset of tumors for SMARCB1/INI1 locus on 22q chromosome by fluorescent in situ hybridization (FISH) and analysis of the SMARCB1/INI1 gene sequence. RESULTS All tumors showed expression of cytokeratin, epithelial membrane antigen, S100, vimentin, brachyury, and EGFR. Atypical morphology, p53 expression, higher MIB-1 labelling index (LI), and INI1 loss were more frequently seen in pediatric chordomas as compared with adults. None of the tumors showed CD117 expression. No point mutation in the SMARCB1/INI1 gene was noted in the tumors examined; however, 4 pediatric and 1 adult chordoma showed loss of this locus on FISH analysis. CONCLUSIONS A subset of pediatric chordomas with atypical histomorphologic features needs to be identified, as they behave in an aggressive manner and require adjuvant therapy. Pediatric chordomas more frequently show p53 expression, INI1 loss, and higher MIB-1 LI as compared with adults, whereas EGFR expression is common to both.

Updates on the multimodality management of desmoplastic small round cell tumor.

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare and an aggressive malignancy with poor outcome. This tumor can co‐express epithelial, neural, and mesenchymal markers. The molecular hallmark of DSRCT is the EWS–WT1 fusion protein. Despite the diversities in treatment modality, the best results have been seen with radical surgery and adjuvant or neoadjuvant chemotherapy. J. Surg. Oncol. 2012; 105:617–621.

Laparoscopic Management of Juvenile Cystic Adenomyoma: Four Cases

STUDY OBJECTIVE To highlight a recently identified rare cause of severe dysmenorrhea in young patients with poor response to medical management. DESIGN Evidence obtained from several timed series with or without intervention (Canadian Task Force classification II-3). SETTING Tertiary care referral hospital. PATIENTS Four young (age, 16-24 years) nulliparous patients with juvenile cystic adenoma with severe secondary dysmenorrhea with poor response to medical management. INTERVENTION Laparoscopic resection of the cystic adenomyoma. MEASUREMENTS AND MAIN RESULTS Complete resection of the cystic adenomyoma was performed laparoscopically in all patients. The lesion was uncapsulated (unlike myoma) and locally defined to the uterine myometrium (unlike diffuse adenomyosis), and there was chocolate-colored blood in the cavity. No other müllerian anomaly was observed in any patient. Histopathologic analysis revealed features suggestive of adenomyosis in all cases. After surgery, dysmenorrhea resolved completely in all patients. Compared with preoperative visual analog scores, significant improvement was observed at the first menstrual cycle after surgery. Patients are being followed up every 3 months for a minimum of 12 months to detect development of dysmenorrhea or any other menstrual disorder. CONCLUSION Juvenile cystic adenomyosis is a rare cause of severe dysmenorrhea. However, it should be included in the differential diagnosis in patients with dysmenorrhea with poor response to medical treatment. All patients reported in the literature and in our series were younger than 30 years, which can be considered as an arbitrary cutoff point to differentiate juvenile from adult cystic adenomyosis. It can be considered a new type of müllerian anomaly that manifests as a noncommunicating accessory uterine cavity lined with endometrium and with an otherwise normal uterus. Surgery is the only therapeutic option for these patients. Minimally invasive surgery in expert hands is advisable to preserve fertility.

Gallbladder carcinoma: An attempt of WHO histological classification on fine needle aspiration material.

Background: Carcinoma of the gallbladder (CaGB) is common in India and its prognosis depends primarily on the extent of the disease and histological type. We aim to study the role of guided fine needle aspiration cytology (FNAC) for diagnosis of CaGB and to evaluate the feasibility of applying world health organization (WHO) classification on fine needle aspiration (FNA) material to predict the outcome of the tumor. Materials and Methods: Retrospective cytomorphologic analysis was performed in all cases of CaGB diagnosed by ultrasound (US) guided FNAC over a period of 2 years. A specific subtype was assigned according to WHO classification based on characteristic cytologic features. These included papillary or acinar arrangement, intra and extracellular mucin, keratin, rosettes and columnar, signet ring, atypical squamous, small, clear, spindle and giant cells. Correlation with histopathology was performed when available. Results: A total of 541 aspirations with clinical or radiological suspicion of primary CaGB were studied. Of these, 54 aspirates were unsatisfactory. Fifty cases were negative for malignancy. Remaining 437 aspirates were positive for carcinoma. Histopathologic diagnosis was available in 32 cases. Adenocarcinoma was the most frequent diagnosis in 86.7% of cases. Mucinous, signet ring, adenosquamous, squamous, small cell, mixed adenoneuroendocrine and undifferentiated carcinoma including spindle and giant cell subtypes were diagnosed identifying specific features on FNAC. Correlation with histopathology was present in all, but one case giving rise to sensitivity of 96.8%. No post-FNA complications were recorded. Conclusions: US guided FNAC is a safe and effective method to diagnose CaGB. Although, rare, clinically and prognostically significant variants described in WHO classification can be detected on cytology.

Leiomyosarcoma of inferior vena cava: A case series of four cases

Vascular leiomyosarcomas are rare tumors, arising most frequently from inferior vena cava (IVC). They are mostly seen in sixth decade, with a female predominance. Their diagnosis is often challenging, as patients may present with non-specific complaints such as dyspnea, malaise, weight loss, abdominal pain, or back pain, preceding the diagnosis by several years. Leiomyosarcoma of the IVC most frequently occurs in the middle segment. The final diagnosis can be made by an ultrasound or computed tomography guided biopsy. Because of limited experience with this disease, optimal management of IVC leiomyosarcoma is unknown. Curative surgical resection remains the current treatment of choice for primary leiomyosarcoma of IVC. Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates. Nonetheless, when complete resection is not possible, debulking combined with radiation therapy still provides good palliation. We, hereby, report four cases of this rare entity with emphasis on management.

Comparison of quality of life in patients undergoing transhiatal esophagectomy with or without chemotherapy

Background/Aim: To compare the quality of life (QOL) in patients undergoing transhiatal esophagectomy (THE) with or without chemotherapy, who were admitted to the Post Graduate Institute of Medical Education and Research, Chandigarh and enrolled in the study, from July 2004 to October 2005. Patients and Methods: Thirty patients of esophageal carcinoma by purposive sampling were randomized into two groups i.e., patients undergoing THE after chemotherapy and patients undergoing THE without chemotherapy. Two QOL questionnaires, one generic i.e., EORTC-QLQ C-30 (European Organization for Research and Treatment of Cancer) and other esophageal cancer-specific i.e., EORTC OES-18 were utilized to assess the QOL. Result: Physical functional scales were better in patients, who received neoadjuvant chemotherapy. The role and social aspects of functional scales deteriorated after completion of treatment in both groups. This was primarily due to the effect of surgery. However, they were better from an emotional and cognitive point of value after surgery and radiotherapy. Fourteen out of 30 patients experienced vomiting and diarrhea due to radiotherapy. Conclusion: THE in esophageal carcinoma improves global health scales and majority of symptom scales in all patients. QOL improvement in general was better in patients who were administered neoadjuvant chemotherapy along with surgery.

Study of β-catenin, E-cadherin and vimentin in oral squamous cell carcinoma with and without lymph node metastases

BackgroundDespite great improvement in the surgical treatment and adjunctive therapy for oral squamous cell carcinoma (OSCC), prognosis remains dismal in advanced cases. Regional metastatic disease is known to reduce recurrence free survival and disease specific survival significantly. The present study was conducted to evaluate the role of cell adhesion molecules β-catenin, E-cadherin and vimentin in predicting tumour metastasis of OSCC.MethodsA total of sixty cases of oral squamous cell carcinoma were included for the study which comprised of 30 cases with lymph node metastases and 30 cases without metastases. Immunohistochemistry was performed for β-catenin, E-cadherin and vimentin on both the test groups along with 30 controls from normal buccal mucosa and inflammatory lesions each.ResultsThere was no significant difference between the immunoreactivity for β-catenin, E-cadherin and vimentin between OSCC with and without lymph node metastases. Vimentin immunopositivity was noted with varying intensity in all cases of OSCC.ConclusionsE-cadherin and β-catenin are probably not the key determinants for regional metastases in OSCC. The role of vimentin expression in OSCC and metastases is controversial and needs to be studied further.Virtual slidesThe virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/6506095201182002.

Utility of transient elastography (fibroscan) and impact of bariatric surgery on nonalcoholic fatty liver disease (NAFLD) in morbidly obese patients

BACKGROUND Controlled attenuation parameter (CAP) is a novel, noninvasive technique for assessing hepatic steatosis. However, its role in morbidly obese individuals is unclear. The effect of bariatric surgery on inflammation and fibrosis needs to be explored. OBJECTIVES To assess the utility of CAP for assessment of hepatic steatosis in morbidly obese individuals and evaluate the effect of bariatric surgery on hepatic steatosis and fibrosis. SETTING A tertiary care academic hospital. METHODS Baseline details of anthropometric data, laboratory parameters, FibroScan (XL probe), and liver biopsy were collected. Follow-up liver biopsy was done at 1 year. RESULTS Of the 124 patients screened, 76 patients were included; mean body mass index was 45.2 ± 7.1 kg/m2. FibroScan success rate was 87.9%. The median liver stiffness measurement (LSM) and CAP were 7.0 (5.0-9.5) kPa and 326.5 (301-360.5) dB/m, respectively. On liver histopathology, severe steatosis and nonalcoholic steatohepatitis were present in 5.3% and 15.8%; significant fibrosis (≥stage 2) and cirrhosis in 39.5% and 2.6%, respectively. Area under receiver operator characteristic curve of LSM for prediction of significant fibrosis (F2-4 versus F0-1) and advanced fibrosis (F3-4 versus F0-2) was .65 (95% confidence interval [CI]: .52-.77) and .83 (95% CI: .72-.94), respectively. The area under receiver operator characteristic curve of CAP for differentiating moderate hepatic steatosis (S2-3 versus S0-1) and severe hepatic steatosis (S3 versus S0-2) was .74 (95% CI: .62-.86) and .82 (95% CI: .73-.91), respectively. At 1-year follow-up, 32 patients underwent liver biopsy. In these patients, there was significant improvement in hepatic steatosis (P = .001), lobular inflammation (P = .033), ballooning (P<.001), and fibrosis (P = .003). Nonalcoholic steatohepatitis was resolved in 3 of 4 (75%) patients. LSM and CAP significantly declined. CONCLUSIONS LSM and CAP are feasible and accurate at diagnosing advanced fibrosis and severe hepatic steatosis in morbidly obese individuals. Bariatric surgery is associated with significant improvement in LSM, CAP, steatohepatitis, and fibrosis.

Cytomorphology of neuroendocrine tumours of the gallbladder

Gallbladder neuroendocrine tumours (GB‐NETs) are rare and account for 0.5% of all NETs. GB‐NETs have an aggressive behaviour, which depends on the tumour grade. The cytomorphological spectrum of these tumours has never been described in detail. The present study evaluates the cytological features of GB‐NETs and grades them according to the World Health Organization (WHO) classification. Furthermore, the expression of thyroid transcription factor‐1 (TTF‐1) has not been investigated previously in GB‐NETs, although found in a subset of extrapulmonary NETs.

Natural history of primary precursor B lymphoblastic lymphoma of the ovary: Report of a rare case

The involvement of the ovaries in lymphomatous processes is a relatively rare phenomenon. Secondary involvement as a part of systemic disease is common as compared to de novo primary lymphoma. Mostly, primary ovarian lymphomas are diffuse large B cell type, whereas the precursor lymphoblastic lymphomas are extremely rare and only four cases have been reported previously. We herein describe a case of primary precursor B lymphoblastic lymphoma involving both ovaries in a 28‐year‐old woman which was detected incidentally and spread into the blood after 7 months; consequently she succumbed to the disease.