Nirmala A. Jambhekar

CT-guided percutaneous core needle biopsy in deep seated musculoskeletal lesions : a prospective study of 128 cases

ObjectivesAlthough large lesions of the limbs can easily be biopsied without image guidance, lesions in the spine, paraspinal area and pelvis are difficult to target, and benefit from CT guidance to improve the accuracy of targeting the lesion for biopsy purposes. A prospective study of CT-guided core needle biopsies for deep-seated musculoskeletal lesions was conducted at a referral cancer institute over a 4-year period with the aim of assessing the safety and efficacy of the procedure.Patients & methodsFrom January 2000 to December 2003, 136 consecutive CT-guided biopsy sessions were undertaken for musculoskeletal lesions in 128 patients comprising 73 males and 55 females. The following data was recorded in all patients: demographic data, suspected clinicoradiological diagnosis, data related to core biopsy session (date, site, approach, total time required in minutes, number of cores, surgeon satisfaction with adequacy of cores), patient discomfort, complications, histopathology report and number of further sessions if material obtained during the first biopsy session was not confirmatory. The sample obtained during the biopsy session was considered inconclusive if, in the opinion of the pathologist, inadequate or non-representative tissue had been obtained. The diagnosis was considered inaccurate if the final histopathological diagnosis did not match with the biopsy diagnosis, or if subsequent clinicoradiological evaluation at follow up did not correlate with the biopsy diagnosis in those patients who were treated with modalities other than surgery.ResultsIn 121 patients, a single session was sufficient to obtain representative material, whilst for six patients two sessions, and for one patient three sessions were necessary. The time taken for biopsy, including the pre-biopsy CT examination time, varied from 15 min to 60 min (median 30 min). For 110 bony lesions 116 sessions were required, and for 18 soft-tissue lesions 20 sessions were required. 108 biopsy sessions yielded a diagnosis, whilst 28 were inconclusive (diagnostic yield of 79.41%). Of 108 diagnostic biopsies, five were considered inaccurate (accuracy rate of 95.37%). The overall diagnostic yield and accuracy rate for bony lesions were 81.03% and 95.74%; and those for soft-tissue lesions were 70% and 92.85%. There were two complications with no permanent sequelae.ConclusionCT-guided core needle biopsy is a safe, easy, and effective technique for the evaluation of deep-seated musculoskeletal lesions, with a high rate of diagnostic yield and accuracy. It facilitates definitive therapy without the patient having to undergo a major surgical procedure for diagnosis.

Revisiting Chordoma With Brachyury, a “New Age” Marker: Analysis of a Validation Study on 51 Cases

CONTEXT Chordoma is a rare, notochordal tumor with a characteristic histomorphology and immunohistochemical profile. At times, it presents a diagnostic challenge, especially in small biopsies. Brachyury, a nuclear transcription factor, is a recently described immunohistochemical marker for diagnosing chordomas. OBJECTIVE To study the sensitivity and specificity of brachyury in diagnosing chordomas by comparing its expression in axial chordomas with nonchordomatous tumors. DESIGN Fifty-one axial chordomas, accessioned during a 10-year period, and 58 nonchordomatous tumors were subjected to brachyury staining by immunohistochemistry. RESULTS The 51 chordomas occurred in 36 men and 15 women. Sitewise, 34 cases (66.7%) occurred in the sacrococcyx, 9 (17.6%) in the spine, and 8 (15.7%) in the skull base. Histologically, 34 cases (66.7%) were classical chordomas, 13 cases (25.5%) had a dominant chondroid component, and 2 cases each (3.9%) were chondroid chordomas and dedifferentiated chordomas, respectively. Brachyury staining was positive in 46 of the 51 chordomas (90.2%) and negative in all 58 nonchordomatous tumors. The dedifferentiated area in 2 chordomas was negative for brachyury staining. Fourteen of 15 chordomas with chondroid component showed positive brachyury staining. Immunohistochemical expression of other markers, included cytokeratin (positive in 23 of 23 cases; 100%), epithelial membrane antigen (positive in 22 of 22 cases; 100%) and S100 protein (positive in 18 of 21 cases; 85.7%). CONCLUSION Exclusive brachyury expression in more than 90% of chordomas indicates its value as a unique, specific marker with other sensitive markers like cytokeratin, epithelial membrane antigen, and/or S100 protein in substantiating a diagnosis of chordoma, including on small biopsies.

Small cell carcinoma of the esophagus: the Tata Memorial Hospital experience

BACKGROUND Small cell carcinoma of the esophagus is a rare disease, characterized by aggressive progression. It has a high incidence of metastatic disease at presentation and a poor overall prognosis. Treatment protocols are not well established because of the paucity of cases and a lack of large studies. METHODS We performed a retrospective review of all patients with small cell carcinoma of the esophagus diagnosed at the Tata Memorial Hospital between 1985 and 2001. We retrieved and analyzed data regarding demographic details, diagnosis, staging, type of treatment, and overall survival. RESULTS Eighteen patients with a mean age of 62 years (range 48 to 80 years) diagnosed as having small cell carcinoma of the esophagus were analyzed. The group included 13 men and 5 women. Seven of the 18 patients (39%) presented with metastatic disease including 5 patients (28%) with liver metastases. Four patients were treated with surgery, with or without chemotherapy or radiotherapy. Three patients were treated with combination chemoradiotherapy, 2 patients with chemotherapy alone, and 5 patients with radiotherapy alone. Four patients with advanced disease and poor general condition were not offered any treatment. The overall median survival of our patients was 6 months. Patients treated with surgery and chemotherapy had a better overall survival. CONCLUSIONS Small cell carcinoma of the esophagus should be regarded as a systemic disease with a high distant failure rate. Treatment strategies hence must incorporate systemic chemotherapy along with radical surgery or radiotherapy as part of a multimodality approach.

Osteosarcoma of the head and neck region: Lessons learned from a single-institution experience of 50 patients

Osteosarcoma of the head and neck region is a rare tumor and is a therapeutic challenge because of its aggressive nature and complex anatomical location. Standard management guidelines are lacking due to paucity of published data.

Decision making in primary sacral tumors

BACKGROUND CONTEXT Primary tumors of the sacrum are extremely rare lesions. Their management is governed by an interplay of complex factors. Appropriate decision making is crucial to obtain the best possible outcome in terms of maximizing disease control while attempting to minimize neurological dysfunction. PURPOSE Our study presents the results of a group of patients with primary tumors of the sacrum who were surgically treated by the same multidisciplinary team at a specialist oncology center over a relatively short period of time (5 years). STUDY DESIGN/SETTING Patients were identified by a retrospective review from a prospectively maintained database. PATIENT SAMPLE Between January 2000 and December 2005, 17 primary sacral tumors were surgically treated at our institution, a referral center for oncology. OUTCOME MEASURES We evaluated the outcome in terms of local disease control, residual neurological dysfunction, and complications as a result of surgical intervention. METHODS There were 12 males and 5 females. The diagnosis included chordoma in six patients, giant cell tumor in seven patients, aneurysmal bone cyst in two patients, and a chondrosarcoma and an osteoblastoma in one patient each. Sixteen of these patients were analyzed. Four lesions had their upper extent at S1, six lesions had their upper extent at S2, four lesions had their upper extent at S3, and two lesions were below S3. Ten cases were treated with wide excision and underwent partial sacral amputations. Five cases had a midline sacral amputation through S1, three through S2, and two through S3. Six benign lesions were treated with curettage. None of the patients received chemotherapy. Four cases received postoperative radiation. The follow-up duration ranged from 18 to 44 months with a mean of 31 months. RESULTS None of the six patients who presented with loss of bladder and bowel control regained it after surgery. Of the 10 patients who had intact bladder and bowel control preoperatively only 4 retained bladder and bowel control postoperatively. Of the six patients who lost bladder and bowel control postoperatively, four patients had a wide excision where bilateral S2 roots were sacrificed. The other two cases in whom the disease extended up to S1 had curettage. Local recurrence occurred in 4 of the 10 lesions treated with wide excision. All the patients who had inadequate margins recurred. Local recurrence occurred in two of the six lesions treated with curettage. Three of the four cases who received postoperative irradiation developed recurrence. Our wound complication rate was 13%. CONCLUSION Wide resection with adequate margins gives the best chance of local control and should be the surgery of choice for all malignant primary sacral tumors and in benign lesions involving lower segments when preservation of both S3 roots is possible. Intralesional curettage has a higher risk of local recurrence without providing the certainty of retaining neurological function. To retain bladder and bowel control and minimize neurological dysfunction, it may be worthwhile managing benign sacral tumors that extend above S3 with serial embolization. The administration of parenteral bisphosphonates may prove beneficial in cases of giant cell tumor managed with serial embolization.

Morphologic spectrum, immunohistochemical analysis, and clinical features of a series of granular cell tumors of soft tissues: a study from a tertiary referral cancer center

A granular cell tumor (GCT) is relatively uncommon and objectively diagnosed with neural markers on immunohistochemistry (IHC). Recent studies have described additional markers for a GCT. Herein, we present morphologic spectrum of 12 GCTs of soft tissues and skin, including 10 benign and 2 malignant subtypes with an optimal diagnostic IHC panel. Eleven cases occurred in women and 1 in a man, with a mean age of 45.5 years. Six cases occurred in soft tissues and skin of extremities, 4 in the breast soft tissues, and 1 case each in the back and preauricular region, respectively. Cytologic smears in 2 cases displayed polygonal cells with abundant, eosinophilic, granular cytoplasm. On histopathology, all cases invariably revealed a nonencapsulated infiltrating tumor comprising groups and nests of granular cells with vesicular nuclei. In 5 cases, granular cells were noted in close proximity to the nerves. Two malignant GCTs revealed necrosis, mitoses, and nuclear atypia. Immunohistochemistry in 10 cases (83.3%) showed diffuse S-100 positivity in all 7 benign and 2 malignant cases: cytoplasmic CD68 positivity (all 10 cases) and membranous vimentin staining (all 4 cases). Inhibin showed cytoplasmic positivity in 3 of 6 cases. A single malignant GCT showed focal desmin reactivity. All were surgically managed. A GCT is a discrete tumor entity and can be identified from other granular lesions by its proximity to nerves and objective identification with diffuse S-100 positivity, CD68 positivity, and membranous vimentin positivity that form an optimal IHC panel in limited resource settings, irrespective of benign or malignant types.

Perioperative Interstitial Brachytherapy for Soft Tissue Sarcomas: Prognostic Factors and Long-Term Results of 155 Patients

BackgroundThe goal of this study was to evaluate the efficacy of temporary interstitial brachytherapy (BRT) for patients undergoing combined modality management of soft tissue sarcomas (STS).MethodsFrom January 1990 to December 2003, 155 adults 18–88 years of age (median = 42 years) with STS who had received BRT as part of locoregional treatment were included in this review. Sixty-four percent were males. Sixty-nine percent had primary lesions. Sixty percent had lesions involving the lower extremities. Spindle cell sarcoma (28%) and synovial sarcoma (16%) were the most common histologic types and 51% had grade III lesions. Treatment included wide local excision of primary tumor with BRT with or without external beam radiotherapy (EBRT).ResultsAfter a median followup of 45 months, the local control (LC), disease-free survival (DFS), and overall survival (OS) for the entire cohort was 71%, 57%, and 73%, respectively. DFS was superior for superficial tumors compared with that for deep tumors (96% vs. 54%, P =.02). Patients with a tumor less than 5 cm had superior OS (88% vs. 63%, P =.05). Cumulative radiotherapy dose greater than 60 Gy had a significant positive impact on LC (P = .003), DFS (P =.003), and OS (P =.048). Subcutaneous fibrosis (21%) was the major complication.ConclusionsTemporary perioperative iridium-192 interstitial BRT with or without EBRT after function-preserving surgery results in satisfactory outcome in patients with STS. Both low dose rate and high dose rate BRT are equivalent in terms of disease control and complications when used alone or in combination with EBRT. BRT results in fewer complications compared with the combination of BRT and EBRT.

Follicular dendritic cell tumor of the parapharyngeal region.

Follicular dendritic cell (FDC) tumors are rare. A majority of the reported cases were confined to the lymph nodes. We report a case of FDC tumor occurring in the parapharyngeal region in a 45‐year‐old woman.

Frequency of EGFR Mutations in 907 Lung Adenocarcioma Patients of Indian Ethnicity

Background During the past decade, the incidence of EGFR mutation has been shown to vary across different ethnicities. It occurs at the rate of 10–15% in North Americans and Europeans, 19% in African-Americans, 20–30% in various East Asian series including Chinese, Koreans, and Japanese. Frequency of EGFR mutations in India however remains sparsely explored. Methodology/Principal Findings We report 23% incidence of Epidermal growth factor receptor (EGFR) mutations in 907 Non small cell lung cancer (NSCLC) patients of Indian ethnicity, in contrast to 10–15% known in Caucasians and 27–62% among East Asians. In this study, EGFR mutations were found to be more common in never-smokers 29.4% as compared to smokers 15.3%. Consistent with other populations, mutation rates among adenocarcinoma-males were predominantly lower than females with 32% incidence. However unlike Caucasians, EGFR mutation rate among adenocarcinoma-never-smoker females were comparable to males suggesting lack of gender bias among never smokers likely to benefit from EGFR targeted therapy. Conclusions/Significance This study has an overall implication for establishing relevance for routine EGFR mutation diagnostics for NSCLC patients in clinics and emphasizes effectiveness for adoption of EGFR inhibitors as the first line treatment among Indian population. The intermediate frequency of EGFR mutation among Indian population compared to Caucasians and East Asians is reminiscent of an ancestral admixture of genetic influence from Middle Easterners, Central Asians, and Europeans on modern- Indian population that may confer differential susceptibility to somatic mutations in EGFR.

Giant cell tumor of bone in children and adolescents.

Background: There are very few series that document giant cell tumor of bone (GCT) in the immature skeleton, and the reported incidence in literature varies from 1.8% to 10.6%. The purpose of this study was to document the incidence of GCT in patients with open physis in the Indian population and study the course of the disease with respect to its adult counterpart to see if it behaved any differently. Methods: Between January 2000 and December 2005, 17 (6%) of 285 surgically treated patients with histologically proven GCT had open physis on imaging. Treatment was directed toward local control without sacrificing joint function, with most lesions treated with intralesional curettage. Results Fourteen (82%) patients were girls. The most common site was around the knee (53%). Of 15 lesions in tubular bones, 13 were epiphysiometaphyseal in location. An open physis did not prevent GCT from penetrating the epiphyseal cartilage. Histologically, the tumors were typical of GCT. Of 15 patients available for follow-up, 3 (20%) developed local recurrence. Conclusions: Although the overall incidence of GCT may be higher in the Asian population, the percentage of skeletally immature patients or those nearing skeletal maturity is similar to that described in literature. The biological behavior of the disease is similar to that seen in adults, except a marked female preponderance, principles of treatment, recurrence patterns, and course of the disease mirror the behavior of its adult counterpart. Level of Evidence: Level IV, case series.