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.

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.

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.

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.

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.

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.

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.

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.

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.

Role of Bone Scan and MRI in Designing of Customized Prosthesis for Limb Salvage Surgery of Long Bone Tumors: 4 Years of Single Institution Analysis.

The purpose of this study is to evaluate the role of bone scan (BS) and MRI measurement data in designing Custom Made Prosthesis (CMP) in Limb Salvage Surgery (LSS) in orthopedic oncology patients. The study was done on 126 patients, which include 88 males and 38 females. Pre-operative planning including determination of the osteotomy plane and dimension of the prosthesis was performed based on Tc99m MDP bone scan and MR images comparing with the histopathology examination (HPE) values. 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. Bone scan and HPE values are positively correlated; p value is 0.913 (p < 0.001). However, mean difference between the HPE and bone scan is statistically significant (p < 0.001). MRI scan and HPE values are positively correlated 0.920 (p < 0.001) and mean difference is also statistically not significant (0.920; p < 0.001). If we apply ± 20% window, 94/126 patients (75%) bone scan measurements and 94/112 patients (84%) MRI measurements are matching with the HPE measurements. We conclude that bone scan measurements were found to be more or less equal to MR imaging for determining the tumor extent for the fit of the prosthesis and excellent functional results.

Expert survey on management of prostate cancer in India: Real-world insights into practice patterns.

To gain insights on the diverse practice patterns and treatment pathways for prostate cancer (PC) in India, the Urological Cancer Foundation convened the first Indian survey to discuss all aspects of PC, with the objective of guiding clinicians on optimizing management in PC. A modified Delphi method was used, wherein a multidisciplinary panel of oncologists treating PC across India developed a questionnaire related to screening, diagnosis and management of early, locally advanced and metastatic PC and participated in a web-based survey (WBS) (n = 62). An expert committee meeting (CM) (n = 48, subset from WBS) reviewed the ambiguous questions for better comprehension and reanalyzed the evidence to establish a revote for specific questions. The threshold for strong agreement and agreement was ≥90% and ≥75% agreement, respectively. Sixty-two questions were answered in the WBS; in the CM 31 questions were revoted and 4 questions were added. The panelists selected answers based on their best opinion and closest to their practice strategy, not considering financial constraints and access challenges. Of the 66 questions, strong agreement was reached for 17 questions and agreement was achieved for 22 questions. There were heterogeneous responses for 27 questions indicative of variegated management approaches. This is one of the first Indian survey, documenting the diverse clinical practice patterns in the management of PC in India. It aims to provide guidance in the face of technological advances, resource constraints and sparse high-level evidence.