Shirley Sundersingh

Role of dynamic sentinel node biopsy in carcinoma penis with or without palpable nodes

Introduction: We aimed to evaluate the role of dynamic sentinel node biopsy (DSLNB) in patients diagnosed with carcinoma penis and clinically N0 disease using superficial inguinal dissection as the standard staging modality. Materials and Methods: Twenty consecutive men (40 groins) with carcinoma penis having clinically N0 status were enrolled in the study. Patients underwent DSLNB if fine needle aspiration cytology from the groin nodes was negative, followed by injection of radiocolloid and blue dye. The sentinel lymph node(s) were harvested. The inguinal incision was then extended and a modified superficial inguinal dissection was performed and all nodes were labeled separately and sent for frozen section. A completion deep inguinal with pelvic dissection was performed if any of the nodes were reported positive for malignancy. Results: The median age of the patients was 52.5 years. Ten patients were smokers. Phimosis was present in five patients. Lesions were present over the glans penis and shaft in 18 and two patients, respectively. Wide local excision, partial penectomy and total penectomy were performed in one, 15 and four patients, respectively. Clinically palpable nodes were found in 19 groins. Median follow-up was 26 months. Nodes were positive in 10 groins. DSLNB missed the sentinel node in one groin. The accuracy and false-negative rate of DSLNB was 97.5% and 10%, respectively. Conclusion: DSLNB is a useful and reliable technique to identify the involved node(s) in patients diagnosed as having carcinoma penis with clinical N0 status (with or without palpable nodes). It helps to avoid the morbidity associated with a staging inguinal dissection in these patients.

Pediatric Hodgkin Lymphoma Treated at Cancer Institute, Chennai, India: Long-Term Outcome

Purpose Pediatric Hodgkin lymphoma (HL) is a highly curable malignancy. Outcomes for pediatric HL may vary between developed and developing countries for multiple reasons. This study was conducted to ascertain the outcomes of children with HL at our center and to identify risk factors for recurrent disease. Methods We analyzed the outcomes of 172 consecutive, previously untreated patients with pediatric HL presenting at our center from 2001 to 2010. Patients were treated with either adriamycin, bleomycin, vinblastine, and dacarbazine or adriamycin, bleomycin, vinblastine, cyclophosphamide, vincristine, prednisone, and procarbazine chemotherapy initially, and radiation to bulky sites or a single site of residual disease when appropriate. Results The median duration of follow-up was 77 months. The median age of the patients was 10 years; 127 (74%) of the 172 patients were male. The extent of disease was stage I and II in 59% of the patients. B symptoms were present in 32% of the patients, and 27% had bulky disease. The most common histologic subtype was mixed cellularity (45%). The 5-year overall survival (OS) and progression-free survival (PFS) of the entire cohort were 92.9% and 83.1%, respectively. The 5-year OS rates for patients with stage I, II, III, and IV were 96%, 94.7%, 84%, and 69.8%, respectively. On univariate analysis, advanced stage, response on interim radiologic assessment, and presence of B symptoms significantly predicted inferior PFS and OS. On multivariate analysis, only interim radiologic response significantly predicted PFS (P < .001) and OS (P < .001). Conclusion Overall, the outcomes of patients treated at our center are comparable to those observed in other centers in India and globally.

Synovial Sarcoma in a Neonate.

BackgroundMalignant tumors in neonates are rare.Case characteristicsA tumor was detected in the left biceps of a 3-day old neonate. Tumor biopsy and molecular study confirmed the diagnosis of synovial sarcoma. The child received multi-modality treatment with surgery and chemotherapy.OutcomeThe child is disease-free on follow-up period of 12 months.MessageSynovial sarcoma can rarely occur in a neonate.

Lymphangitis Carcinomatosa: Report of a Case and Review of Literature

Lymphangitis carcinomatosa describes a condition of diffuse infiltration of the lymphatics of both the lungs by malignant cells. It may be the only manifestation of an occult malignancy and should be included in the differential diagnosis of dyspnoea, especially in young patients in whom this diagnosis is usually not suspected. The diagnosis rests on a high index of suspicion in the setting of progressive respiratory failure. In this article we review the clinical presentation and the diagnostic methods. We also review the relevant literature pertaining to this rare entity.

Primary B-cell lymphoblastic lymphoma of the ovary

Sir, An 18‐year‐old unmarried girl presented with a 1‐month history of lower abdominal pain without fever, loss of weight or appetite, or menstrual irregularity. At a local hospital, computerised tomography imaging showed a large left adnexal mass measuring 10.6 cm × 7.1 cm × 8.2 cm, for which she underwent laparotomy and ovariectomy and came to us for further management. On evaluation, she had an Eastern Cooperative Oncology Group (ECOG) performance status of one with unremarkable general physical and abdominal examination. The ovariectomy specimen reviewed at our center showed high‐grade non‐Hodgkin’s lymphoma (NHL) confirmed as B‐lymphoblastic lymphoma by immunohistochemistry [Figures 1‐5]. The malignant cells were positive for LCA, CD20, C79a, CD10, and TdT and negative for staining with LMO, EMA, CD3, and CD68. The Ki‐67 was strongly expressed in over 90% of the cells [Figures 1‐5]. Serum alpha‐fetoprotein (2.09 ng/ ml), beta‐human chorionic gonadotropin (2.39 mIU/ml), and lactate dehydrogenase levels (381 U/L) were normal. Her baseline hemogram and peripheral smear were normal, and there was no evidence of tumor lysis. Imaging at our center showed no residual tumor in the abdomen. Bone marrow aspiration, bone marrow trephine biopsy, and cerebrospinal fluid analysis were negative for disease Primary B-cell Lymphoblastic Lymphoma of the Ovary

Refractory ALK-Positive Anaplastic Large Cell Lymphoma: Long Term Survival with Crizotinib

Anaplastic large cell lymphoma, ALK positive is characterized by increased expression of the ALK protein. Though two-thirds can be cured with chemotherapy, patients with refractory disease have limited options [1]. ALK has been successfully targeted by crizotinib in lung cancer and reports show success in ALK? lymphomas also [2]. A 38year female presented with right iliac fossa pain in June 2014. She had a large soft tissue mass in the right ilio-psoas region extending from the level of L1 to L5 vertebra with bony destruction of L3 vertebra (Fig. 1a, b). Biopsy from the mass showed ALK? ALCL. She started CHOP chemotherapy but progressed after cycle 1. With a more intensive therapy (LMB-89, group B protocol), she initially responded, but again progressed after cycle 5. The lymphoma continued to progress on salvage DHAP chemotherapy. She developed intense pain in the iliolumbar region which was poorly controlled with morphine and resulted in spasm of the iliopsoas muscles and fixed flexion at right hip. She received radiation with partial relief of pain but remained bed bound in poor general condition. At this point, we started oral therapy with crizotinib 250 mg twice daily. Within 2 weeks, there was complete resolution of pain and patient was able to walk. Follow up CT scans showed progressive improvement and PET-CT scan done after 6 months of crizotinib showed complete metabolic response (Fig. 1c, d). Patient was unwilling for stem cell transplant and hence crizotinib was continued. She has completed 30 months of crizotinib till now and remains in complete anatomic and metabolic response. This report highlights a patient with chemo-refractory ALK? ALCL showing a dramatic and sustained response to ALKtargeted therapy. Complete response to crizotinib is not uncommon in ALKL? ALCL [2, 3]. The fusion protein NPM-ALK promotes tumorigencity by binding to and constitutively activating a host of cellular signalling proteins, including those in the signalling pathways of signal transducer and activator of transcription 3 (STAT3), MEK/ERK, mammalian target of rapamycin (mTOR) and phosphoinositide 3 kinase (PI3K)/Akt leading to cell proliferation and resistance to apoptosis. Crizotinib exerts its therapeutic effect by inhibiting the phosphorylation of the ALK tyrosine kinase and thus blocks the downstream effects of this oncogene [4]. The duration of therapy is currently unclear and the longest duration of reported in literature is 37 months [5]. Though crizotinib may be considered as a reasonable option in patients with chemo refractory & Prasanth Ganesan prasanth.ganesan@cancerinstitutewia.org

Outcomes in Lung Cancer: 9-Year Experience From a Tertiary Cancer Center in India

Purpose Lung cancer is the most common cause of cancer mortality in the world. There are limited studies on survival outcomes of lung cancer in developing countries such as India. This study analyzed the outcomes of patients with lung cancer who underwent treatment at Cancer Institute (WIA), Chennai, India, between 2006 and 2015 to determine survival outcomes and identify prognostic factors. Patients and Methods In all, 678 patients with lung cancer underwent treatment. Median age was 58 years, and 91% of patients had non–small-cell lung cancer (NSCLC). Testing for epidermal growth factor receptor mutation was performed in 132 of 347 patients and 61 (46%) were positive. Results Median progression-free survival was 6.9 months and overall survival (OS) was 7.6 months for patients with NSCLC. Median progression-free survival was 6 months and OS was 7.2 months for patients with small-cell lung cancer. On multivariable analysis, the factors found to be significantly associated with inferior OS in NSCLC included nonadenocarcinoma histology, performance status more than 2, and stage. In small-cell lung cancer, younger age and earlier stage at presentation showed significantly better survival. Conclusion Our study highlights the challenges faced in treating lung cancer in India. Although median survival in advanced-stage lung cancer is still poor, strategies such as personalized medicine and use of second-line and maintenance chemotherapy may significantly improve the survival in patients with advanced-stage lung cancer in developing countries.

Acinar Cell Carcinoma of Pancreas: a Case Report and Review of Literature

Acinar cells are the predominant cells of the pancreas but malignant transformation is rare with acinar cell carcinoma (ACC) accounting for 1–2% of all pancreatic neoplasms in adults and about 15% in the paediatric population [1]. Although grouped under exocrine neoplasms of the pancreas, ACCs have distinct clinical, morphological and pathological characteristics [2]. We report a case of ACC of pancreas in a 57-year-old gentleman who presented with a lesion in the tail of pancreas with liver metastasis. The management and brief review of literature is presented.

Fibroblastic Reticulum Cell Tumor of the Breast: A Case Report and Review of Literature

IntroductionFibroblastic reticulum cell tumor (FRCT) is a rare tumor arising from the antigen-presenting dendritic cells.MethodWe present a case of FRCT of breast in a 62-year-old female, who presented with a right breast lump. Computed tomography scan revealed a well-circumscribed right breast mass. There was no axillary lymph node enlargement or distant metastasis.ResultThe patient had localized disease and underwent simple mastectomy followed by adjuvant chemotherapy. The patient is well and disease free at the end of 2 years of follow-up. Only two cases of FRCT of breast have been reported in the literature.ConclusionFRCT can be misdiagnosed as phyllodes tumor, anaplastic large cell lymphoma and carcinoma. There is no consensus on the role of adjuvant chemotherapy or radiotherapy in FRCT of breast due to paucity of data.