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.

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.

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.

Primary Synovial Sarcoma of the Kidney with Inferior Vena Caval Thrombus.

Synovial sarcomas occurs primarily in the soft tissues, mostly in para-articular regions of the extremities. These tumors have been described in other unusual locations like the pleura, lungs, mediastinum, and kidneys. Primary synovial sarcoma of the kidney is a recently described entity; to date, a total of approximately 35 cases have been reported. Only three cases of primary synovial sarcoma of kidney with IVC thrombus have been reported so far. Here we present the case of a 44 year old gentleman, with primary synovial sarcoma of the left kidney with caval thrombus.

Metastatic malignant melanoma of the small bowel--report of two cases.

CASE REPORT: We report two cases of malignant melanoma metastasizing to the ileum and jejunum in a 48-year-old female and 62-year-old male, respectively. The female patient was a known case of vaginal melanoma who on follow-up developed pain abdomen 4 years after excision of the primary, whereas the male patient who was initially referred as pleomorphic spindle cell sarcoma of the groin presented with complaints of bleeding per rectum and melena 6 years later. RESULTS: After preliminary investigations both underwent laparotomy and resection of segments of ileum and jejunum with tumor. Histopathological examination with immunohistochemistry showed features suggestive of metastatic malignant melanoma. DISCUSSION: Metastasis should be suspected in patients with malignant melanoma who develop gastrointestinal symptoms such as abdominal pain, anemia, melena, fatigue, constipation, small bowel obstruction, or perforation. This helps in avoiding a delay in the diagnosis and complications that arise due to metastatic disease. CONCLUSION: Our first patient with primary vaginal melanoma died of multiple metastases 11 months following surgery for the ileal metastasis while the second patient with jejunal metastasis developed recurrent disease in the small bowel and iliac lymph nodes 10 months after surgery. However, in a patient with isolated gastrointestinal metastasis, diagnosed early, with good general condition surgical management should be encouraged when a complete resection of the disease is feasible as no other treatment option is as good for relief of symptoms and prolongation of life.

A rare case of synchronous adrenocortical carcinoma and renal cell carcinoma.

We present here a rare case of synchronous adrenocortical carcinoma (ACC) and renal cell carcinoma (RCC). A 27-year-old woman presented with gradual abdominal distension, hematuria, and loss of weight of 3-months duration. She gave a history of treatment for hypertension. The computed axial tomography (CT) scan revealed a large retroperitoneal mass. Her urinary VMA was slightly elevated. Her 24-h urinary metanephrine level was normal. The patient underwent left adrenalectomy, left nephrectomy, spleenectomy, and distal pancreactomy with segmental colonic resection. Postoperative pathology revealed ACC of left suprarenal measuring 22 × 19 × 18 cm(3) and RCC involving the left upper pole of kidney measuring 3 × 2 × 1 cm(3).

Risk factors for lymph node metastasis in clinically node-negative penile cancer patients.

OBJECTIVES: To analyze the effects of pathological T stage, grade, extent of surgery for primary tumor, and age group on the risk of developing lymph node metastasis in clinically node-negative penile cancer patients. METHODS: We performed a retrospective analysis of 200 clinically node-negative penile cancer patients who were kept under surveillance, after treatment of the primary tumor in our institution. The primary outcome parameter was cytologically or histologically proven lymph node metastasis. Logistic regression analysis was used to compute odds ratios in univariate and multivariate settings. RESULTS: Lymph node metastasis occurred in 31 patients at a median time of three months. Histological grade 3 and grade 2 tumors had a statistically significant increased odds ratio for lymph node metastasis, (7.1[P < 0.001] and 2.7 [P = 0.04], respectively), compared with grade 1 tumors. Although increasing pT stage was associated with increasing odds ratios, the differences were not statistically significant. Nor did the extent of surgery of the primary tumor or the age group significantly influence the risk of developing lymph node metastasis. CONCLUSIONS: Histological grade is the most significant parameter influencing the risk of lymph node metastasis in clinically node-negative penile cancer patients on surveillance. Patients with grade 3 and grade 2 tumors may benefit from elective inguinal lymphadenectomy.