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.

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.

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.

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.

Synovial sarcoma of kidney in a child: A rare presentation.

There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms' tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms' tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction.

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.

Inoperable Renal Malignant Glomus Tumor, the answers for all the "W's"?

Glomus tumor, arising from glomus bodies (specialized neurovascular structures involved in thermoregulation), commonly occurs in extremities and rarely in viscera. The spectrum of glomus tumors range from benign tumors to tumors with uncertain malignant potential to tumors of the malignant subtype. A vast majority of visceral glomus tumors are benign. Most common visceral tumors arise in the gastrointestinal tract. Glomus tumors of the kidney are a rare entity of which malignant glomus tumors are exceedingly rare. The index patients in the existing case reports were middle-aged males. We report our experience with malignant glomus tumor of the left kidney in a 60-year-old female, with computed tomography (CT) showing involvement of renal vein and inferior vena cava (IVC). Percutaneous biopsy was performed as imaging did not conform to the appearance of a conventional renal tumor and was reported as malignant glomus tumor after immunohistochemistry. After informed decision, the patient and family elected to proceed with surgery. However, intraoperatively, the left renal mass was found to infiltrate the pancreas, duodenum, aorta, and root of the colonic mesentery due to which surgery was aborted. Biopsy obtained intraoperatively again confirmed diagnosis of left renal malignant glomus tumor. She had an uneventful postoperative recovery. Options of treatment were reviewed by a multidisciplinary board. In light of no proven benefit for systemic therapy, she was referred for supportive care. She was under follow-up and she expired after 7 months due to progressive disease. Our literature review focuses on the clinicopathologic features and the current standard of management of malignant renal glomus tumors.

A Rare Site of Metachronous Metastases from Renal Cell Carcinoma.

Secondary metastatic involvement of the testis is a rare occurrence, particularly in cases of metastasis from renal cell carcinoma (RCC). We present a case of metachronous contralateral testicular metastasis from RCC in a 55-year-old man, occurring 2 years after radical nephrectomy. Following a thorough evaluation that ruled out systemic disease, the patient underwent a Chevassu procedure and right inguinal orchidectomy. Histopathological analysis confirmed metastatic involvement of the right testis by RCC. Metastasis to the testis from RCC is uncommon, with only a few cases reported in the literature. Isolated metachronous metastasis without systemic involvement is even rarer. This case highlights the importance of considering testicular metastasis in patients with a history of RCC, emphasizing the need for comprehensive evaluation and surgical resection when feasible, as it has been associated with prolonged survival.

Single-Stage Free Flap Reconstruction Following Extremity Sarcoma Resection: a Regional Cancer Center Experience.

Surgery remains mainstay modality of treatment of STS of extremity. In majority of patients, primary closure is possible following surgical resection of the tumor. Primary closure of wound may not be feasible in tumors with large area of skin involvement and sometimes following a whoops procedure. We analyzed postoperative complications and oncological outcomes in patients who underwent free flap reconstruction. Thirty-seven patients who required a free flap for reconstruction of the defect following resection of the STS were included in the study. There were 26 men and 11 women with a mean age of 40 years. Seventy-three percent tumors were in lower limb; 62% patients had undergone a whoops procedure elsewhere. Flaps were fasciocutaneous in 19, myocutaneous in 15, and free muscle transfers in 3 patients. Seventeen patients had early surgical site complications. Nine patients had early flap failures. Of the 9, 6 patients had total loss of flap and in 3 the flaps were salvaged. Two patients had partial loss (< 50% of total area) of flap. Four patients had edge necrosis and two had suture diastasis. Nine patients had late surgical site complications. Four had post-radiotherapy wound dehiscence. Late SSI was noted in two. Late joint contracture and chronic lymphedema was seen in one patient each. One died due to sepsis. Statistically significant association was not found between patient, tumor or treatment-related factors and complications or flap failures. Mean duration of surgery was 7.73 h. Twenty-three patients required multiple surgeries due to complications. Mean time to initiate adjuvant therapy from the date of surgery was 47 days. Mean OS of the group was 67 ± 7.3 months. Mean RFS was 58 ± 7.8 months. Single-stage free flap reconstruction following surgical resection of extremity sarcoma is a viable option of reconstruction while salvaging the limb. Though associated with high rate of early surgical site complications, they are manageable.

Single-Stage Combined Approach Sagittal En Bloc Spondylectomy for L3 Vertebral Chondrosarcoma: A Technical Note.

BACKGROUND: Primary malignant tumors of the spine are rare and most commonly occur in lumbar and thoracic vertebrae. We report a rare case of retroperitoneal chondrosarcoma of L3 that was managed with sagittal en bloc spondylectomy following chemoradiation. CASE DESCRIPTION: A 26-year-old woman was evaluated for abdominal pain with contrast-enhanced computed tomography of the abdomen and pelvis, which revealed a soft tissue retroperitoneal mass arising from L3. She underwent laparotomy and biopsy, which revealed chondrosarcoma, and she received chemoradiation over a period of 28 weeks 6 days. After repeat imaging, she underwent single-stage combined approach sagittal en bloc spondylectomy of retroperitoneal chondrosarcoma of L3 with right nephrectomy and spine reconstruction. At 3-year follow-up, there was no evidence of recurrence on contrast-enhanced computed tomography of the abdomen and pelvis. She demonstrated no gait abnormality or spinal deformity. CONCLUSIONS: Sagittal en bloc spondylectomy is a preferred surgical approach for eccentrically placed spinal tumors that offers better oncological and functional outcomes.

Influence of Accuracy and Precision of Measurements of Long Bone Tumors in Imaging Studies-A Retrospective Study in Musculoskeletal Oncology.

The aim of this study is to evaluate the level of accuracy and precision of bone scan (BS), MRI, and digital radiography (DR) to measure long bone tumors to design custom-made prosthesis (CMP)/modular prosthesis (MP) in limb salvage surgery (LSS) with the help of phantom and patient's study. There are two separate groups: one is the phantom study and another one is the patient's study. The phantom study is done with the Jaszack Phantom for the Gamma camera and the indigenous phantom for the MRI and DR. Three independent imaging professionals (nuclear medicine physicians and radiologists) measured the distance between standardized, preselected points on the Jaszack phantom in the Gamma Camera (GC) and indigenous phantom on the coronal and sagittal view of the MRI scan and in digital radiography. The measured values were compared with the known values for phantom measurement. A total of 36 patients, which include 24 males and 12 females, 3 independent imaging professionals measured the patient's long bone in a bone scan, MRI and DR and compared it with histopathological specimen measurement after limb salvage surgery (LSS). Descriptive statistics using appropriate measures of central tendency and dispersion were employed to describe the data. Karl-Pearson correlation coefficient was used to establish the association between continuous covariates. Paired t-test was utilized to test the differences in paired values for statistical significance. A near-perfect positive correlation was evident between all three pairs of bone scan, MRI scan, and digital radiography values, and a positive agreement within 1 mm of the bone scan, MRI scan, and DR values of all three pairs was around 95%. For the phantom study, we conclude that Gamma camera and MRI measurements are equal in physical measurements (MCF-1). DR measurements were found to be near equal physical measurements and multiplication correction factor (MCF)-0.9104 and three observer's measurements values were also near normal. For the patient's study, we conclude that the bone scan, MRI, and DR measurements of 3 independent imaging professionals are near normal, and it was confirmed with pathological specimen after LSS, to confirm reliability, repeatability, reproducibility, and accuracy of the tumor length to do custom-made prosthesis or modular prosthesis for the patients who are affected by osteosarcoma and Ewing's sarcoma.

Factors that Impact the Outcomes in Ewing's Sarcoma: Experience from a Regional Cancer Center in Southern India.

Ewing's sarcoma family of tumors (EWSFT) is common in the second decade of life. Achieving good outcomes in EWSFT requires a multimodality approach. We report the clinico-pathological features, treatment, and survival outcomes of patients with EWSFT treated at our center. Patients diagnosed and treated for EWSFT at our center from 2009-2017 were included in this study. Data was collected from the patient's case records. Event-free survival (EFS) and overall survival (OS) were estimated using the Kaplan-Meier method. The study included 173 patients among whom 44 (25%) patients were metastatic at diagnosis. The median age of patients was 16 years. The most common site of the primary tumor was the pelvis (16.1%), followed by long bones. The median follow-up was 75 months and the 5-year EFS and OS were 43.7% and 45.1% respectively for the overall cohort whereas for the localized disease were 56.6% and 57.2% respectively. Metastatic disease, tumor volume > 200 ml, tumor diameter > 8 cm, pelvic site, hemoglobin < 10 gms%, elevated lactate dehydrogenase, positive margin, and necrosis less than 90% were significantly associated with inferior OS on univariate analysis. On multivariate analysis, metastasis disease, tumor diameter > 8 cm, and necrosis < 90% were significantly associated with inferior OS. Large tumors, advanced disease, and poor response to chemotherapy are associated with poor outcomes in EWSFT. Whether the use of dose-dense chemotherapy and/or autologous stem cell transplant would improve outcomes without increased toxicity in resource-limited settings needs to be explored. Supplementary Information: The online version contains supplementary material available at 10.1007/s13193-023-01817-6.

Distally Based Pedicled Fibula Flap for Reconstruction of Distal Tibia After Oncological Resection: A Case Series and Review of Literature.

Reconstruction of distal tibial defects pose a difficult challenge because the bone is subcutaneous and close to the tendons and neurovascular bundles. Distally based pedicled fibula with retrograde flow can be used for the reconstruction of distal tibial defects. This is based on the communicating branch of the peroneal artery to the posterior tibial artery. We present three cases of distal tibia primary tumours which were resected and reconstructed using recycled autograft plus distally based pedicled fibula and ankle arthrodesis. This pedicled retrograde fibula flap is a novel technique for the reconstruction of distal tibial defects after oncological resections. It provides a vascularized graft without the need for microvascular surgery and without violating the normal limb. Meticulous dissection of and preservation of the communicating branches between the peroneal artery and the posterior tibial artery with confirmation of retrograde flow before dividing the proximal peroneal pedicle is sine quo non for the success of this graft. This flap overcomes the drawback of the limited arc of rotation and limited reach of proximal pedicle-based flap for distal tibial reconstruction. Long-term functional outcomes, limb shortening associated with this flap, and its effect on functional outcomes remain to be ascertained.

Sarcomatoid Carcinoma Metastasis to the Colon from a Small Renal Mass: Case Report with Review of Literature.

A third of patients with renal cell carcinoma (RCC) present with metastatic disease. Metastasis in RCC from small renal mass (SRM) (≤4 cm) is rare. We report a case of stage cT1a clear-cell RCC with low-risk features on pathology presenting with disproportionately large synchronous solitary metastasis to the transverse colon. He underwent resection of the mass with the involved transverse colon and adjoining mesocolon. Intestinal continuity was restored, following which partial nephrectomy was performed for the right renal tumor. Final pathology of the right renal mass confirmed clear-cell RCC. The large mass after immunohistochemistry profile confirmed metastasis from the renal tumor.

Limb Salvage Surgery in a Rare Case of Recurrent Parosteal Osteosarcoma with Vascular Graft Thrombosis.

Recurrent parosteal sarcomas with vascular involvement are rare and present unique challenges in their diagnosis and management. We report the case of a 21-year-old woman with parosteal osteosarcoma of the left distal femur, encasing the popliteal vessels. En bloc transarticular resection of the distal femur and popliteal vessels was performed, followed by reconstruction using a modular prosthesis and a saphenous vein autograft for both the artery and vein. On the 1st postoperative day, the patient developed an arterial thrombus requiring reintervention with a jump polytetrafluoroethylene (PTFE) graft. Histopathology confirmed parosteal osteosarcoma. After a disease-free survival of 41 months, the patient experienced local recurrence involving the PTFE graft, leading to graft compression, erosion, and subsequent thrombosis. Despite these complications, limb salvage was possible due to adequate collateral blood supply. This case highlights the feasibility of limb salvage surgery in select cases of parosteal osteosarcoma with vascular involvement.

Outcomes with chemotherapy in carcinoma penis: Experience from a tertiary cancer center.

Background: Carcinoma penis is more common in India compared to the West. The role of chemotherapy in carcinoma penis is ambiguous. We analyzed the profile and outcomes of patients with carcinoma penis treated with chemotherapy. Methods: We analyzed the details of all patients with carcinoma penis treated at our institute between 2012 and 2015. We collected particulars regarding demography, clinical presentation, treatment details, toxicities, and outcomes of these patients. Event-free and overall (OS) survival were calculated from the time of diagnosis until documentation of disease relapse/progression or death for the patients with advanced carcinoma penis who were eligible for chemotherapy. Results: There were 171 patients with carcinoma penis treated at our institute during the study period including 54 (31.6%) patients with stage I, 49 (28.7%) patients with stage II, 24 (14.0%) patients with stage III, 25 (14.6%) patients with stage IV, and 19 (11.1%) patients with recurrent disease at presentation. The present study included 68 patients with advanced carcinoma penis (stages III and IV) who were eligible for chemotherapy, with a median age of 55 years (range: 27-79 years). Sixteen patients received paclitaxel and carboplatin (PC) and 26 patients cisplatin and 5-FluoroUracil (CF). Neoadjuvant chemotherapy (NACT) was given to four patients with stage III and nine patients with stage IV disease. Of the 13 patients given NACT, we observed a partial response in five (38.5%), stable disease in two (15.4%), and progressive disease in five (38.5%) evaluable patients. Six (46%) patients underwent surgery after NACT. Only 28/54 (52%) patients received adjuvant chemotherapy. After a median follow-up of 17.2 months, the 2-year OS rates were 95.8, 89, 62.7, 51.9, and 28.6% for stages I, II, III, IV, and recurrent disease, respectively. The 2-year OS of patients who were given chemotherapy versus those who were not given chemotherapy were 52.7 and 63.2%, respectively (P = 0.762). Conclusions: We report the real-world outcomes of two chemotherapeutic regimens used in consecutive patients with advanced carcinoma penis. Both PC and CF seemed effective and safe. However, approximately half of patients with advanced carcinoma penis do not receive the planned/indicated chemotherapy. We need further prospective trials regarding the sequencing, protocols and indications of chemotherapy in this malignancy.

Paratesticular Osteosarcoma-A Rare Tumor with Distinctive Imaging Findings.

Extraosseous osteosarcoma (EOO) is a rare mesenchymal malignancy representing 4% of all osteosarcomas and 1% of soft tissue sarcomas. The testes, its supporting structures, that is, paratestes, and the spermatic cord are among the rarest sites for EOO, with only 11 published English language reports to date. We report our experience with a 73-year-old male presenting with left hemiscrotal swelling, noted to have extensive amorphous intratumoral calcification on imaging. He underwent left high inguinal orchidectomy with en bloc hemiscrotectomy, with a final pathologic diagnosis of primary paratesticular osteosarcoma. Our literature review corroborates this distinctive, hitherto overlooked imaging feature.

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.

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.