Testicular malignancy in persistent Mullerian duct syndrome: Experience from an apex cancer center with review of literature.

OBJECTIVES: Persistent Mullerian duct syndrome (PMDS) is a rare disorder of sexual differentiation resulting from aberrations in the Mullerian inhibiting factor (MIF) pathway, with consequent failure of regression of fetal Mullerian duct. The concomitant association of undescended testis increases the likelihood of developing testicular tumors in these patients. Due to its rarity, clinic-pathologic and treatment outcome data on testicular cancer in PMDS is sparse. We present our institutional experience and review published literature on testicular cancer in PMDS. MATERIAL AND METHODS: We retrospectively queried our institutional testicular cancer database for all patients with a diagnosis of testicular cancer and PMDS, between January 1980 and January 2022. Additionally, a Medline/PubMed search was performed for English language articles published during the same time period. Data on pertinent clinical, radiologic, and pathologic disease characteristics were abstracted, in addition to treatment received and outcomes. RESULTS: Of 637 patients treated for testicular tumors during the specified time period in our institution, 4 patients had a concomitant diagnosis of PMDS. Testicular tumor was pathologically confirmed as seminoma in 3, 1 had mixed germ cell tumor. All patients in our series presented with stage 2B or higher disease and required chemotherapy, either in the neoadjuvant or adjuvant setting, in addition to surgery. With a mean follow up of 67 months, all patients were disease free. Medline/PubMed search retrieved 44 articles (49 patients) of testicular tumors associated with PMDS, with majority (59%) presenting with a large abdominal mass. Only 5 cases (10%) had a preceding history of appropriately managed cryptorchidism. CONCLUSIONS: Testicular cancer in PMDS usually presents in adults with advanced stage disease resulting from neglected or inadequate management of cryptorchidism. Appropriate management of cryptorchidism in childhood is likely to decrease malignant degeneration, if not, enable early-stage diagnosis.

Adrenal Castleman's Disease: Case Report and Review of Literature.

Castleman's disease is a rare lymphoproliferative disorder of poorly understood etiology. It is most commonly located in the mediastinum. Castleman's disease mimicking adrenal neoplasm is a very rare differential diagnosis and unusual presentation. We report a rare suprarenal unicentric hyaline vascular variant of the disease in a 16-year-old boy with atypical symptoms mimicking an adrenal neoplasm.

Primary Renal Synovial Sarcoma Case Series: Clinical Profile and Management of a Rare Entity.

Synovial sarcomas occur predominantly in the extremities. Primary renal synovial sarcoma is a rare entity. Very few cases have been reported in the literature. Clinical and radiological features are similar to renal cell carcinoma with the diagnosis being established after surgery based on histopathology, immunohistochemistry, and chromosome studies. There are no established guidelines on the role of adjuvant treatment in the management of this disease. We herein present a series of 3 cases managed at 2 institutions. In the current series, all patients had venous thrombus, and surgery was the mainstay of treatment. One patient received neoadjuvant chemotherapy after a preoperative biopsy which was done as she did not respond to chemotherapy for a presumptive diagnosis of Wilm's tumor.

Factors Predicting Nodal Metastasis in Penile Cancer: Analysis from a Tertiary Center.

INTRODUCTION: Lymph node metastasis is an important prognostic marker in penile cancer. Identification of occult metastasis is by lymphadenectomy based on the histological features of primary tumor; however, this leads to unnecessary surgical morbidity in node negative patients. METHODS: A retrospective analysis of all surgically treated penile cancer patients managed at our institute from January 2011 to March 2014 was performed. Patient characteristics, histological factors, and lymph node involvement were identified. Logistic regression analysis was used to compute odds ratio (OR) in univariate and multivariate analysis. RESULTS: Ninety seven patients underwent surgical management at our institute during the abovementioned period. Grade III tumor, presence of lymphovascular invasion, tumor thickness >10 mm, perineural invasion (PNI) and Ki67 >50% were significantly associated with nodal metastasis. On multivariate analysis, only presence of PNI was found to be significant (OR: 6.82) (95% confidence interval: 1.72-27.03) (p = 0.006). CONCLUSION: PNI is a strong independent predictor of occult lymph node metastasis in penile cancers. Its inclusion in stratification of clinically node negative patients will identify high-risk patients who will benefit from prophylactic lymphadenectomy.

Nonbilharzial Squamous Cell Bladder Cancer: An Indian Experience.

Anand RajaBackground Squamous cell carcinoma represents the second most common histological type of bladder cancer. Nonbilharzial squamous cell carcinomas of bladder are rare histological variant with limited experience. Objective We aimed to review our experience to determine various treatment patterns and survival outcomes for this malignancy. Methods Data from patients treated at our center from 1995 to 2016 was collected from patient records and analyzed. Clinicopathological variables, treatment patterns, and follow-up data were extracted. Results A total of 32 patients were included in the study with a median age of 55.5 years. Hematuria was the most common presentation. Overall, 16 patients underwent radical cystectomy, 8 underwent definitive radiotherapy (RT), 4 received palliative RT, and 4 patients defaulted for any treatment. Surgery conferred better survival rates as compared with RT (31.9 vs. 7.45 months). In the surgical group, only pathological TNM staging was a significant prognostic factor. Conclusion In localized nonbilharzial squamous cell bladder cancer, radical cystectomy with bilateral pelvic node dissection appears to be treatment modality of choice. Larger series are needed to validate the role of other perioperative modalities.

Clipping inguinal lymphatics decreases lymphorrhoea after lymphadenectomy following cancer treatment: results from a randomized clinical trial.

BACKGROUND: Post-operative lymphorrhea is a well-known complication of inguinal lymph node dissection. However, the interventions to reduce the duration of drain in situ have not been sufficiently elaborated. OBJECTIVES: We evaluated the potential role of intra-operative mapping of lymphatic leakage with peri-incisional methylene blue injection and clipping of lymphatics after inguinal block dissection in reducing postoperative lymphorrhea. METHODS: We randomized 39 inguinal dissections done for various malignancies such as for carcinoma penis, urethra, malignant melanoma, rectum into 19 dissections (Interventional group) and 20 dissections (Control group). In the interventional group, after the completion of inguinal dissection, two ml of methylene blue dye was injected 4-8cm from the incision to identify the leaking lymphatics and they were clipped. RESULTS: The primary outcome was the decrease in duration of days of drain in situ and was found to have significant reduction of 3.07 days in the interventional arm. (p value-0.02). The secondary outcome was the reduction of 21 ml of mean drain output in the interventional group (p = 0.09). The number of lymphatics clipped was not found to have statistical correlation with the duration of drain in situ and the mean drain output. CONCLUSION: The intraoperative mapping of lymphatic channels using methylene blue after inguinal dissection reduces the number of days of drain in situ.

Nerve-Sparing Postchemotherapy Retroperitoneal Lymph Node Dissection (PC RPLND) for Nonseminomatous Germ Cell Tumour: Experience from a Tertiary Cancer Centre.

Postchemotherapy RPLND remains an integral part of management of testicular tumours. Nerve-sparing techniques can minimize the ejaculatory dysfunction due to the procedure. We report our functional and oncological outcomes for nerve-sparing RPLND in postchemotherapy settings. We analysed data from all patients undergoing nerve-sparing PC RPLND from January 1990 to December 2013 at our institute. Antegrade ejaculation and fertility issues were determined by patient history. Nerve sparing was achieved in 30% of patients undergoing PC RPLND. Of the 33 patients who underwent nerve-sparing PC RPLND, 19 (57.8%) had antegrade ejaculation. The mean time to antegrade ejaculation was 6.8 months. After a median follow-up of 75.61 months, 5-year disease-free survival was 98%. Nerve-sparing RPLND can improve functional outcomes without increasing recurrence rates in post chemotherapy setting.

Intravesical Bacillus Calmette-Guerin (BCG) Therapy for Non-muscle Invasive Bladder Cancers: Long-term Results of a Modified Schedule.

Intravesical BCG therapy is an integral part of management of non-muscle invasive bladder cancers. Our aim is to analyze the non-muscle invasive bladder cancer patients treated at our center with a modified schedule intravesical BCG therapy. Data from patients treated at our center from 2009 to 2017 was collected from patient records and analyzed. A 6-weekly 120-mg induction course followed by 6 monthly 120 mg has been used at our institute for NMIBC. Clinicopathological and treatment variables were collected. A total of 119 patients were treated at our center with a median follow-up period of 4.18 years with the above schedule. Nearly 96% patients were able to complete induction therapy and 79% completed the maintenance therapy. The 5-year recurrence-free survival was 83%. The recurrence and progression rates were 16.8% and 4.2% respectively. About 60% of the patients suffered from side effects of BCG with 11% having class 3 or 4 toxicity. Our regimen of monthly maintenance intravesical BCG for 6 months shows good control rates with high compliance, similar to those of other contemporary series, although with higher incidence of high-grade toxicity.

Inferior Vena Cava (IVC) Wall Penetration by IVC Filter Detected Incidentally During Retroperitoneal Lymphadenectomy.

Anticoagulation is the treatment of choice in deep venous thrombosis; IVC filters can be placed in cases where anti coagulation is absolutely contra indicated. IVC filters are not without complications, some of which can be life-threatening. If detected early and managed timely and appropriately, a crisis can be averted. We report a complication-IVC wall penetration by a retrievable IVC filter, detected incidentally during a retroperitoneal lymph node dissection.

Utility and Safety of Repeat Transurethral Resection of Bladder Tumor Performed at a Tertiary Center.

Introduction Repeat transurethral resection of bladder tumor (ReTURBT) has become an integral part of the management of superficial bladder cancers at various urological centers around the world. Early detection of residual disease, leading to upstaging in some cases, leads to decrease in recurrence rates. Our study aimed to analyze the impact of ReTURBT in detecting residual tumor and tumor recurrences, hence validating the benefits of procedure as a routine. Materials and Methods A total of 152 patients with superficial bladder cancer who were treated at Cancer Institute (WIA) between January 2005 and December 2013 were analyzed and followed up for 3 years. Results Of the 152 cases who underwent ReTURBT, 47 patients had residue in the final histopathology of the resected specimen (31%). The overall rate of upstaging to muscle-invasive disease following ReTURBT was 3.3%. The mean follow-up period was 47.13 months, during which 25 (17%) out of 147 patients who underwent ReTURBT had disease recurrence. There was no additional morbidity due to ReTURBT as compared with the primary procedure. Conclusion ReTURBT is an effective procedure in treating recurrent tumors also as long as they remain superficial. The procedure when performed with utmost care in experienced hands remains a very safe procedure to be followed as a routine and standard.

Gastrointestinal Stromal Tumours (GIST): Indian Experience of Rare Malignancy.

GISTs are rare tumours of the GI tract arising from the intestinal cells of Cajal. Though various risk stratification systems have been proposed, none has been universally accepted. We audited the survival and recurrence patterns in our patients and evaluated clinicopathological features to identify prognostic factors affecting survival. We conducted a retrospective analysis of patients treated at our hospital from 1999 to 2012. Patient variables, clinicopathological factors and treatment variables were collected. Sixty-three patients were evaluated and treated at our institute of which 38 were non-metastatic. The most common site of origin was the stomach. On univariate analysis, presence of metastasis, male gender, high mitotic rate, non-gastric primary and epithelioid histology were significantly associated with poor overall survival. Tumour size > 10 cm, mitotic rate > 10/50 hpf and presence of necrosis significantly affected disease-free survival for non-metastatic patients. Multivariate analysis showed higher mitotic rate and non-gastric primary to correlate with worse outcome. In our experience, a high mitotic rate and non-gastric primary independently predicted a poor prognosis in GIST.

A retrospective analysis of patients undergoing postchemotherapy retroperitoneal lymph node dissection and metastasectomy in advanced nonseminomatous germ cell tumors.

INTRODUCTION: Postchemotherapy retroperitoneal lymph node dissection (PC-RPLND) and metastasectomy play an important role in the management of advanced-stage nonseminomatous germ cell tumors (NSGCT). We aimed to analyze preoperative parameters that could predict postoperative histology. MATERIALS AND METHODS: We analyzed the data of 72 patients who underwent PC-RPLND and 14 patients who underwent metastasectomy after receiving cisplatin- or carboplatin-based chemotherapy for advanced stage NSGCT at our institute from 1994 to 2015. Clinical and pathological parameters such as the histology of orchidectomy, RPLND and metastasectomy, serum tumor markers, and the pre and post chemotherapy retroperitoneal lymph node size were recorded. RESULTS: Seventy-two patients with a mean age of 28 years underwent PC-RPLND. Of the various variables evaluated, only percentage change in nodal size was found to be statistically significant in predicting necrosis (P = 0.004). A decrease of 75% was found to predict the necrosis with a specificity of 100%. There was 84.6% concordance between the histology of RPLND and that of metastasectomy. CONCLUSION: A 75% reduction in tumor size is highly predictive of absence of viable tumor or teratoma, however larger series are required to confirm these findings. RPLND histopathologies have a high concordance with metastasectomy histology and thus can be used as a guide to tailor further management.

Usefulness of narrow-band imaging in transurethral resection of bladder tumor: Early experience from a tertiary center in India.

BACKGROUND: The current standard for diagnosis and treatment of urinary bladder cancer is transurethral resection of bladder tumor (TURBT) using white light guidance. Narrow band imaging (NBI) has emerged as a promising method for identifying additional bladder lesions. Various studies have been published to evaluate its sensitivity in identifying new lesions and its impact on decreasing recurrences. In this study, we evaluated our early experience using NBI in TURBTs. AIMS AND OBJECTIVE: The aim of the study is to determine the accuracy of NBI in identifying additional malignant lesions during TURBT. MATERIALS AND METHODS: We retrospectively collected data for all patients who underwent either TURBT or repeat TURBT with white light and NBI from November 2016 to July 2017 at Cancer Institute (WIA). The number of additional lesions identified using NBI was evaluated along with its correlation with the final histopathology. RESULTS: Forty patients were analysed of which 20 underwent TURBT and 20 underwent repeat TURBT. Of these, 36 patients had complete resection of tumour. Additional lesions were detected in 6 patients (14%) by NBI of which 2 (33%) were malignant histology. The additional lesions detected were carcinoma in situ and no patient was upstaged. CONCLUSION: The inclusion of NBI to conventional white light TURBT increases the sensitivity for identifying additional lesions. The limitation of NBI is high false positivity and its availability. Long term follow up studies with larger subset of patients are required to evaluate its role in decreasing recurrences and justification in routine clinical practice.