Siddharth Laskar

Primary carcinoma of the vagina: Tata Memorial Hospital experience

PURPOSE Carcinoma of the vagina is a rare gynecological malignancy comprising approximately 2% of all the gynecological malignancies. We have analyzed the treatment outcome of the patients treated at the Tata Memorial Hospital from January 1984 to December 1993. METHODS AND MATERIALS In this 10-year period, 134 patients of primary vaginal cancers were registered at our hospital. Of these, 75 patients received complete treatment and are analyzed. RESULTS Disease-free survival (DFS) for the whole group is 50%, and overall survival (OAS) is 60%. Most locoregional recurrences and distant failures are noted in the 2 years following treatment. DFS at 5 years is as follows: Stage I (5 patients), Stage IIA (37 patients), Stage IIB (15 patients), Stage III (14 patients), and Stage IV (4 patients); are 40%, 55%, 60%, 50%, and 25%, respectively. The DFS for patients with complete response (42 patients) to external radiation at 5 years is 68%, with partial response (25 patients) is 35%, and with poor or no response (6 patients) is 18% (p = 0.0000). We observed that brachytherapy was an important part of the treatment, and patients who received brachytherapy (59 patients), either with a vaginal intracavitary applicator (30 patients) or interstitial implant (29 patients) had a DFS of 53% and 56%, respectively, while 15 patients who received external radiation alone had a DFS of 30%. Patients receiving brachytherapy within 4 weeks of external radiation had a DFS of 60% as compared to 30% when the interval was more than 4 weeks. CONCLUSION The factors indicating prognosis are: site and extent of involvement, presence of lymph nodes at presentation, technique of brachytherapy, and interval between external radiation and brachytherapy.

Alveolar soft part sarcoma 'revisited': clinicopathological review of 47 cases from a tertiary cancer referral centre, including immunohistochemical expression of TFE3 in 22 cases and 21 other tumours.

Background: Alveolar soft part sarcoma accounts for 0.5–1.0% of soft tissue sarcomas in the United States. At our Hospital, it constitutes 1.8% of the newly diagnosed soft tissue sarcomas. Lately, TFE3 has been found to be a useful immunohistochemical marker for diagnosing this sarcoma. Methods: We reviewed 47 cases of alveolar soft part sarcoma that were either treated at Tata Memorial Hospital, Mumbai, India, or were referred in consultation from various parts of India. TFE3 immunohistochemical staining was performed on 22 alveolar soft part sarcomas and on 21 other tumours. Results: Unlike most other large series, 58% of patients were males and 40% were females. The ages ranged from 2 to 54 years (median 24 years). Tumours were located in the deep soft tissues of lower extremities (54%), upper extremities (13%), head and neck (11%), retroperitoneum (10%), chest wall (6%), pelvis (4%), and were positive for TFE3 (20/22, 91%), desmin (3/18, 16%), myoglobin (1/6, 17%) and smooth muscle actin (1/9, 11%). TFE3 was positive in tumour controls that comprised paragangliomas (3/4), translocation related renal cell carcinoma (1/1), adrenocortical carcinoma (1/3) and granular cell tumour (1/3). Treatment consisted of primary surgical excision, metastatectomy, chemotherapy and radiotherapy. Seven tumours (24%) recurred locally and 21 of 29 (72%) metastasised, mainly to the lungs. Follow-up information (5–108 months, median 27.5 months) was available for 22 patients. No patients died in the relatively short follow-up period. Conclusions: TFE3 is a useful immunohistochemical marker for diagnosis of an alveolar soft part sarcoma. Awareness of other tumours expressing TFE3 is vital. Alveolar soft part sarcoma has a high metastasis rate but relatively good short-term survival. Surgical excision with follow-up forms the present management.

Clinicopathologic study and outcome analysis of thyroid lymphomas: Experience from a tertiary cancer center

The aim of this study was to review clinicopathologic presentations of patients diagnosed with thyroid lymphomas at a tertiary cancer center. Thyroid lymphomas represent less than 2% of all lymphomas.

Interstitial brachytherapy in soft tissue sarcomas

BackgroundSoft tissue sarcomas are relatively rare tumors with an aggressive natural history associated with a high propensity for local recurrence following conservative surgery. It accounts for 1.8% of all cancers seen at the Tata Memorial Hospital, Mumbai. Organ preserving surgery and tumor bed brachytherapy have revolutionized the management of soft tissue sarcomas.Patients and MethodsOne hundred and seventy-seven patients with histologically proven non-metastatic cases of soft tissue sarcomas in the age group of 16 to 79 years (median 41 years) were treated at the Tata Memorial Hospital between January 1983 and December 1992. One hundred and fifty-one patients who had completed a minimum of 24 months of treatment were studied. There were 100 males (66%) and 51 females (33%). The majority had recurrent lesions (70.3%). Extremities were involved in 75% of patients. Spindle cell sarcoma was the major histologic variant (30%). The patients underwent function preserving surgery and temporary afterloading Ir-192 tumor bed brachytherapy with or without external radiotherapy.ResultsIn patients receiving brachytherapy only, 25 out of 33 (75%) were locally controlled after a median follow-up of 30 months. After successful salvage of local failures the overall local control improved to 82%. Similarly in the patients who received both interstitial, brachytherapy and external irradiation, the local control rate after a median follow-up of 40 months was 71% which improved to 86% after successful salvage. The overall treatment related complication rate was less than 1%. The only marginally significant prognostic factor for local control was tumor grade (p=0.06).Conclusion sThe sequential combination of limited surgery and tumor bed brachytherapy with or without external radiotherapy has been established as an effective alternative to more ablative procedures like amputation. Histologic grade has proven to be a significant factor determining local control.ZusammenfassungHintergrundWeichteilsarkome sind relativ seltene Tumoren, die jedoch einen aggressiven natürlichen Verlauf mit hoher Lokalrezidivrate nach konservativer Chirurgie aufweisen. Etwa 1,8% aller Tumoren im Tata Memorial Hospital sind Weichteilsarkome. Die organerhaltende chirurgische Resektion und anschließende Brachytherapie des Tumorbettes haben die Behandlung der Weichteilsarkome revolutioniert.Patienten und Methoden177 Patienten mit histologisch nachgewiesenem, nichtmetastasierten Weichteilsarkom im Alter zwischen 16 und 79 Jahren (Median 41 Jahre) wurden zwischen 1983 und 1992 in unserem Hospital therapiert. 151 Patienten wiesen eine minimale Nachbeobachtungszeit von 24 Monaten auf und bildeten die Studiengruppe. Es handelte sich um 100 Männer (66%) und 51 Frauen (33%). Die Mehrzahl der Patienten hatte rezidivierte Läsionen (70%). Bei 75% der Patienten waren Extremitätensarkome vorhanden. Die häufigste histologische Typisierung war mit 30% das Spindelzellsarkom. Alle Patienten wurden funktionserhaltend operiert, anschließend mit einer temporären Ir-192-Brachytherapie des Tumorbettes und ggf. mit externer Radiotherapie nachbehandelt.ErgebnisseNach alleiniger postoperativer Brachytherapie war bei 25 von 33 (75%) der Patienten eine anhaltende lokale Tumorkontrolle zu erreichen. Nach erfolgreicher Rettungschirurgie der Lokalrezidive erhöhte sich die lokale Tumorkontrolle auf 82%. Für Patienten, die eine kombinierte interstitielle und externe Radiotherapie postoperativ erhalten hatten, betrug die lokale Kontrollrate 71%, einschließlich Rettungschirurgie 86%. Die behandlungsbedingte Komplikationsrate lag bei unter 1%. Der einzige, marginal signifikante Prognosefaktor für den Endpunkt lokale Kontrolle war das Tumorgrading (p=0,06).SchlußfolgerungDie Kombination aus extremitätenerhaltender Chirurgie, Brachytherapie des Tumorbettes und externer Radiotherapie ist eine sichere und wirksame Alternative im Vergleich zur Amputation.

Primary non-metastatic Ewing sarcoma of the jaw in children: results of surgical resection and primary reconstruction.

The rarity of Ewing sarcoma (ES) of the jaw coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data on surgical management of these tumors. The purpose of this study is to describe the results of surgical excision and reconstruction of primary non‐metastatic ES of the mandible and maxilla in children.

Prognostic factors in primary nonmetastatic Ewing sarcoma of the rib in children and young adults

BACKGROUND The rarity of Ewing sarcoma of rib has resulted in paucity of data, particularly on the prognostic factors and pattern of relapses. We analyzed the recurrences in patients with primary nonmetastatic Ewing sarcoma of the rib and examined prognostic factors of poor outcome. METHODS From January 2004 to January 2011, 37 patients were treated. After induction chemotherapy, complete (from costal cartilage to vertebra) or partial excision of involved rib with or without adjacent ribs was performed. Postoperative radiotherapy was administered for positive margins, poor response to chemotherapy, and large primary tumors with significant soft tissue component at presentation. RESULTS Disease relapsed in 16 patients: at the local site (n = 5), both local and distant (n = 2), and distant site only (n = 9). The projected 5-year cause-specific, relapse-free survival and local control were 50%, 44%, and 72%. Poor response to chemotherapy (>5% residual tumor) and resection of adjacent lung parenchyma (a surrogate for tumor extension) were adverse prognostic factors for relapse-free survival in multivariate analysis. CONCLUSION Relapses occurred more often at distant sites and had a poor outcome. In this study, poor histologic response to chemotherapy (P = .04) and the infiltration of adjacent lung parenchyma (P = .01) are adverse prognostic factors.

Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document

Rhabdomyosarcoma (RMS) is a highly malignant tumor which is thought to originate from the pluripotent mesenchyme. It is the most common soft-tissue sarcoma of childhood. This review article summarizes the recent and older published literature and gives an overview of management of RMS in children. RMS can arise in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph nodal spread, therapeutic response and long term outcome, hence requiring physicians to be familiar with site-specific staging and treatment details. Most common primary sites include the head and neck region, genitourinary tract, and extremities. Prognosis for children and adolescents with RMS has recently improved substantially, especially for patients with local or locally extensive disease because of the development of multi-modal therapy incorporating surgery, dose-intensive combination chemotherapy, and radiation therapy. Despite aggressive approaches the outcome for patients who present with metastatic disease remains unsatisfactory. Clinical trials are ongoing to reduce toxicity and improve outcomes of such patients; newer agents in combination are being investigated.

Extra-renal Wilms’ tumour—is it different?

Nephroblastomas (Wilms’ tumour) are the most common solid malignancies in childhood, but extra-renal Wilms’ tumours are rare entities, and the diagnosis is made only after surgical intervention. However, the embryogenesis and the exact mechanism of occurrence of primary ERWTs is controversial, thereby presenting a number of unanswered questions of great theoretical interests. We present three well-proven cases of extra-renal Wilms’ tumour together with a review of the literature based on its histogenesis, its behaviour and its management policies.

Management of Wilms Tumor: ICMR Consensus Document

Wilms tumor (WT) is the most common renal tumor of childhood. Although multidisciplinary care including surgery, chemotherapy and radiotherapy have greatly improved the survival rates in WT, there is a scope for further improvement in India and other resource-poor settings. In resource-limited settings, the majority of patients present with large tumors, which may either be unresectable or risky to resect; making preoperative chemotherapy followed by delayed surgery the preferred approach. Histology and staging are used for risk stratification. The imaging procedure of choice is Contrast Enhanced CT scan (CECT) of thorax/ abdomen and pelvis, which is to be done at presentation, as well as for re-evaluation. Surgery is the cornerstone of treatment in WT and Radical Nephroureterectomy and Lymph node sampling is the procedure of choice, to be performed at week 5 in Non Metastatic WT and week 7 in Metastatic WT. WT is an extremely chemosensitive and radiosensitive tumor. Preoperative chemotherapy for Non Metastatic WT consists of 4 wk of Vincristine /Actinomycin and 6 wk of Vincristine /Actinomycin/ Adriamycin for Metastatic WT, with post-operative chemotherapy depending on stage and histology. Radiation therapy is recommended mainly in Stage III and Stage IV WT, with other indications given in the text. Other recommendations, such as treatment of WT in special situations and for supportive care are also detailed in the text.

Clinical characteristics and treatment outcomes of primary and recurrent malignancy involving the salivary glands in children

Malignant salivary gland neoplasms are rare in children. The purpose of this study was to present our experience in the treatment of primary and recurrent salivary gland neoplasm and contribute to the fractional available data.