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Background Primary lung sarcoma (PLS) differs in management protocols and prognosis from the more common primary lung carcinoma (PLC). It becomes imperative to raise a high index of suspicion on radiological and pathological features.

Abstract Synovial sarcoma (SS) is one of the commonest non-rhabdomyosarcoma soft tissue sarcoma with limited treatment options in the relapsed and advanced settings. The combination of gemcitabine and docetaxel has demonstrated its role predominantly in leiomyosarcoma and pleomorphic sarcomas but has not been prospectively studied in SS. This trial assesses the efficacy, tolerability and quality of life (QoL) with this regimen in metastatic/unresectable locally advanced relapsed SS. Patients and methods This was a single-arm, two-stage, phase II, investigator-initiated interventional study among patients with metastatic or unresectable locally advanced SS who had progressed after at least one line of chemotherapy. Gemcitabine 900 mg/m2 on days 1 and 8 and docetaxel 75 mg/m2 on day 8 were administered intravenously every 21 days. The primary endpoint was 3-month progression-free rate (PFR); overall survival (OS), progression-free survival (PFS), overall response rate (ORR), safety and quality of life (QoL) constituted the secondary endpoints. Results Twenty-two patients were enrolled between March 2020 and September 2021 and the study had to be closed early due to slow accrual. The study population comprised of 18 (81.8%) patients with metastatic disease and 4 (18.2%) patients with locally advanced, unresectable disease. The most common primary sites of disease were extremity in 15 (68%) and the median number of lines of prior therapies received was 1 (range 1–4). 3-month PFR was 45.4% (95% CI 24.8–66.1) and ORR was 4.5%. Median progression-free survival (PFS) was 3 months (95% CI 2.3–3.6) and median OS was 14 months (95% CI 8.9–19.0). 7 (31.8%) patients experienced grade 3 or worse toxicities, including anemia (18%), neutropenia (9%) and mucositis (9%). QoL analysis demonstrated significant decline in certain functional and symptom scales, while financial and global health scales remained stable. Conclusion This is the first prospective study on the combination of gemcitabine and docetaxel performed specifically in patients with advanced, relapsed SS. Although the accrual of patients could not be completed as planned, the therapy did produce clinically meaningful outcomes and met its primary endpoint of 3-month PFR. This result, along with the manageable toxicity profile and stable global health status on QoL analysis, should encourage further studies. Trial registration This trial was prospectively registered under the Clinical Trials Registry of India on 26/02/2020 (Registration number: CTRI/2020/02/023612).

Pleomorphic myxoid liposarcoma (PML) is a newly recognized entity with aggressive clinical behavior and a tendency to recur. It has histological features of both myxoid and pleomorphic liposarcoma and lacks the molecular and structural chromosomal abnormalities associated with myxoid and pleomorphic liposarcoma. The data about their response to chemotherapy is quite sparse. We report a case of incidentally detected pleomorphic myxoid liposarcoma of the mediastinum in a 32-year-old gentleman. After resection and adjuvant chemotherapy with doxorubicin and ifosfamide, there was no evidence of residual disease at the end of treatment. During a routine follow-up 5 months later, he was found to have a recurrence of the disease with histological confirmation. He received a trabectedin given its activity in myxoid liposarcoma. However, he had toxicities and progression leading to its discontinuation. Subsequently, eribulin was started as the next line of therapy. After 4 cycles of chemotherapy, response assessment was suggestive of partial response, which is still maintained after 7 cycles of eribulin. This is the first report of this entity responding to a newer chemotherapy regimen.

Background: Li-Fraumeni syndrome (LFS) is a rare autosomal dominant hereditary cancer syndrome. Due to the high risk of occurrence of multiple cancers, families with LFS may have an overwhelming psychosocial burden. Methods: This cross-sectional study was conducted at a tertiary care center using face-to-face interviews through a grounded theory approach. Statistical analysis was done using Smith’s Interpretative Phenomenological Approach. Themes and sub-themes were extracted, and a thematic schema was developed. Results: A total of five themes were identified. The extracted themes were psychological experiences, behavioural responses, stressors, coping strategies and perceived needs. The interlay of the themes deepened the impact of LFS on the affected ones and brought into light the turmoil of emotions and difficulties that these individuals were going through in the face of the disease. Conclusions: LFS-affected individuals had a range of experiences with this rare and little-known disease. The lack of information seems to be a precursor to the denial of diagnosis. Their experience with the illness sheds light on the grey areas like guilt and helplessness that demand immediate attention. Future policies need to be developed in accordance with the identified perceived needs to potentially guide the treatment and rising needs of LFS-affected individuals.

Background Aggressive fibromatosis (AF) is a benign tumor that usually has a locally aggressive and recurrent disease course. Reports of association between AF and malignancies have been reported infrequently. Case We report a case of a 49-years lady who had papillary thyroid carcinoma associated with a distinct desmoid tumor occurring concurrently on the right side of the neck. Initial management comprised of total thyroidectomy followed by radio-iodine therapy and desmoid tumor resection. Recurrent AF developed at the same site as before after 2 years of resection. The recurrent tumor was managed with sorafenib, the patient responded with a resolution of symptoms, and the tumor remained stable. Beta-catenin mutation done by Sanger sequencing was negative in the tumor specimen. Conclusion AF can occur as a separate tumor in association with PTC. If symptoms are not life-threatening medical management may be a better choice in management.

Abstract Background Neurofibromatosis type 1 is an inherited cancer predisposition syndrome that is caused by a mutation in the NF1 gene that encodes neurofibromin. Patients with neurofibromatosis type 1 have a higher risk of gastrointestinal stromal tumor. This study reports the case of a patient with gastrointestinal stromal tumor who was later diagnosed to have neurofibromatosis type 1 and, unlike usual features, had some uncommon features such as occurrence at an early age and unusual site of origin. Case We report the case of a 29-year-old Indian female diagnosed to have gastrointestinal stromal tumor originating from the greater curvature of the stomach. Gastrointestinal stromal tumor was wild type, negative for c-kit and platelet-derived growth factor receptor, and had an aggressive clinical course not responding to oral tyrosine kinase inhibitors. On later evaluation, we found that the patient had germline mutation in NF1. This case has some unusual features compared with gastrointestinal stromal tumor cases reported in neurofibromatosis type 1. Firstly, the age of onset for gastrointestinal stromal tumor in neurofibromatosis type 1 is earlier in our case compared with previous cases reported in literature. Secondly, the site of occurrence is in the stomach, without involving other parts of the intestine. Gastrointestinal stromal tumor in neurofibromatosis type 1 is usually multifocal, and small intestine is the common site of occurrence. When occurring in the stomach, it is usually associated with other lesions in the small intestine. Lastly, the clinical course is aggressive compared with previous case reports and series. Conclusion Our patient had germline NF1 mutation and cutaneous stigmata of neurofibromatosis. Our patient had unicentric gastrointestinal stromal tumor occurring at younger age and involving greater curvature of the stomach, with spindle cell type histology and high-risk features. If gastrointestinal stromal tumor occurs at young age, we should look into neurocutaneous markers.

Aim: Hemangioendotheliomas (HEs) are malignant vascular tumors with sparse descriptions in literature owing to their rarity. Study design: Ours is a retrospective study among patients of advanced HEs registered between September 2015 and April 2021. Results: There were 13 patients with median age 34.6 (range: 4–69 years), male preponderance (69%) and predominant subtype of epithelioid HE (76.9%). Common primary sites were viscera (46.2%) and bone (30.8%). Tyrosine kinase inhibitors (TKIs) yielded objective responses in 30% patients whereas chemotherapy only produced disease stabilization in 7.7%. Conclusion: We recognize an aggressive subset of HEs with manifestations such as acute liver failure and splenic rupture. Currently no biomarkers predict the efficacy of TKIs over chemotherapy; however, TKIs showed promising outcomes in this series.

Abstract Background Carney’s triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25–30% cases, with most patients presenting with two out of three parts of the syndrome. Wild-type succinate-dehydrogenase-deficient gastric gastrointestinal stromal tumor forms the most common component of Carney’s triad and is usually multicentric and multifocal. It usually demonstrates indolent behavior and resistance to imatinib; hence, the management remains predominantly surgical. Pulmonary chondromas are commonly unilateral and multiple with slow-growing nature, which allows for conservative management. Adrenocortical adenomas are found in 20% of patients and are usually detected as incidentalomas. Case presentation A 49-year-old Asian male presented with upper gastrointestinal bleed and was diagnosed with multiple gastric succinate-dehydrogenase-deficient gastrointestinal stromal tumors. On evaluation, he was found to have left pulmonary chondroma and non-secretory adrenal adenoma, thus completing the Carney’s triad. He underwent surgery with sleeve gastrectomy and excision of the antral tumor nodule, while the adrenal and pulmonary tumors have been under close follow-up. Conclusion Literature regarding Carney’s triad is scarce, especially from the Indian setting. Our report aims to highlight the various manifestations of this syndrome with emphasis on management of wild-type succinate-dehydrogenase-deficient gastrointestinal stromal tumor. Radical gastric surgeries do not offer a survival advantage in this condition; hence, more conservative modalities of resection can be adopted.

Aim: Clinicopatholgical findings and outcomes in epithelioid sarcoma (ES) patients. Materials & methods: ES patients registered in sarcoma clinic from 2015 to 2021. Results: There were 20 patients with median age of 26 years. Majority had distal ES (70%) and advanced disease (85%). In patients with advanced disease lymph nodes were involved in 65%, lungs in 58% and others in 35%. Among 14 patients who underwent biopsy outside our institute, nine (64.2 %) had been initially misdiagnosed. Response rates to doxorubicin (n = 12), pazopanib (n = 6), gemcitabine/docetaxel (n = 5), tazemetostat (n = 3) and immunotherapy (n = 2) used in various lines were 16, 16, 20, 33 and 0%, respectively. Conclusion: Our patients had an advanced-stage and distal ES, with a modest response to chemotherapy.

Context Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor most commonly occurring in deep intramuscular plane of lower extremities of adolescents and young adults. It is a highly vascular, slow growing tumor with malignant potential having lung as the most common site of metastases at the time of presentation. Aims The aim is to review the imaging findings of ASPS and determine characteristic imaging features of this rare tumor. Materials and Methods Sixteen patients having histopathological diagnosis and preoperative imaging of ASPS attending the dedicated sarcoma clinic at our institute were included in the study. The demographic, clinical, and imaging data were retrieved from the case records and then evaluated for characteristic imaging features which may raise suspicion of ASPS. Results The patients ranged from 3 to 72 years of age and with a slight male preponderance. Of the eight CECTs evaluated, 62.5% tumors showed well-defined lobulated margins, 87.5% cases showed intense enhancement with presence of feeder vessels. On CEMRI of 10 patients, 70% had well circumscribed lobulated margins with intense enhancement and tortuous flow voids in most of them. All cases showed T2 hyperintense signal. Fourteen of 16 (87.5%) patients had metastatic disease with lung as the most common site (92.8%). Conclusion ASPS is a rare soft tissue sarcoma seen in children and young adults. Imaging may mimic a vascular malformation due to the presence of tortuous feeders. Misdiagnosis at an early stage may lead to later metastatic presentation of the disease, thus emphasizing the need to suspect it on imaging.

Background Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor predominantly affecting the pelvis and perineum of females. Due to its variable clinical presentation, it is often misinterpreted and treated as other pathologies like Bartholin gland cyst, vulvar abscess, and hernia. The tumor is locally infiltrative, making its complete surgical removal difficult. Aim The aim of the study is to highlight and illustrate the imaging and pathological features of AAM. Materials and Methods After obtaining clearance from the Institutional Ethics Committee, a retrospective study was conducted on patients with histopathological diagnosis of AAM, presenting to the dedicated sarcoma clinic between 2016 and 2019. The demographic, clinical, radiological, pathological, and treatment details were obtained from the institute's database. The magnetic resonance imaging (MRI) and computed tomography images were reviewed to assess different imaging parameters. Follow-up imaging was assessed for residual or recurrent mass in the pelvis or perineum and distant metastasis. Results A total of four patients were included with an age range of 28 to 50 years. The baseline MRI of these four patients shows pelvic mass extending into the perineum, infiltrating along the surrounding fascial planes with the characteristic laminated appearance on T2-weighted image and gradual intense enhancement following contrast administration. All patients had residual disease post-surgery and were put on hormonal therapy. Conclusion AAMs are locally aggressive, rarely metastasizing mesenchymal tumor that has a specific predilection for the perineum and pelvis of females. MRI features like laminated or striated appearance, post-contrast enhancement, and finger-like infiltrating projections should raise the suspicion of the diagnosis on baseline imaging.

Aim: To study the role of check point inhibitors (CPI) in sarcoma and gastrointestinal stromal tumors. Materials & methods: Retrospective data of 15 patients diagnosed with advanced sarcoma or gastrointestinal stromal tumors and treated with CPI. Results: 3/14 patients (21.4%) responded to treatment with a disease control rate of 42.8% (6/14). After a median follow-up of 14 months (range: 2–24 months), 11 (73.3%) patients progressed, the median progression-free survival was 4 months (95% CI: 1.7–6.3) and median overall survival was 14 months (95% CI: 2.6–25.7). Only one patient experienced a grade IV adverse event. Conclusion: Our data represent the first real-world application of CPI in sarcoma from India. We believe that CPI should be further evaluated in clinical trials.

Background There is sparse literature on trabectedin in advanced soft-tissue sarcomas from developing world. It would be interesting to know about use and outcomes of trabectedin in Indian patients. Method In a retrospective study, consecutive patients treated with trabectedin from 2016 to 2019 were analyzed. Patients with L-sarcomas were treated at a dose of 1.5 mg/m2, while those with translocation-related sarcomas were treated at a dose of 1.2 mg/m2 as a 24-hour infusion through peripherally inserted central catheter line. From July 2019, infusions were administered through an ambulatory elastomeric pump, while before that patients were admitted for 24 hours. We used SPSS version 23.0 for statistical calculation. Result A total of 20 patients received trabectedin with a total of 116 infusions. The median age was 46 years (range: 22–73 years). The male (n = 11, 55%) and female patients were almost equal (n = 9, 45%). Thirteen patients (65%) had Eastern Cooperative Oncology Group Performance Status 1. Majority of the patients had leiomyosarcoma (n = 8, 40%); remaining comprised of liposarcoma (3, 15%), translocation-related sarcomas excluding myxoid liposarcoma (n = 8, 40%) and others (n = 1,5%). Most common site was extremity (n = 11, 55%) followed by retroperitoneal (n = 3, 15%), visceral (n = 3, 15%), and others (n = 3,15%). Median number of previous lines received was 2 (range: 0–4). Median number of trabectedin cycles received was 4 (range: 1–17). Best response assessed was stable disease (n = 10, 50%), progressive disease (n = 6, 30%), partial response (n = 1, 5%), and not assessed in 3 patients. After a median follow-up of 19 months, median progression-free survival was 4 months. Conclusion In this heavily treated population (composed of L-sarcomas and translocation-related sarcomas) with many patients with poor performance status, the outcome with trabectedin is in synchrony with literature. However, the need of 24-hour admission might deter quality of life. Elastomeric pump seems to be a reasonable alternative to admission and can be a breakthrough in administering trabectedin, especially in developing countries.

Inflammatory myofibroblastic tumors (IMT) are rare soft tissue tumors of intermediate malignant potential with tendency for local recurrence. Although they can occur at all age groups, occurrence in infants is extremely unusual and their imaging characteristics are not well described. A 3-month-old female infant presented with gradually progressive abdominal distention without any fever or weight loss. She had a large ill-defined homogenous hypodense lesion of size 8.4 × 11.4 × 11.3 cm (APxTraxSag) in the abdomen showing mild delayed post contrast enhancement. She underwent exploratory laparotomy with gross total excision of mesenteric mass, histopathology of which was suggestive of IMT. She had recurrence within 6 months of complete resection with a well-defined heterogeneously enhancing lesion of size 1.8 × 1.8 × 2.3cm (APxTraxSag) in right paravesical region abutting the bladder without invasion with a similar lesion of size 4.4 × 2.1 × 3 cm (APxTraxSag) in left subdiaphragmatic region abutting superior surface of spleen (no invasion). Since, surgery in our patient would have entailed splenectomy and partial cystectomy, systemic therapy with ceritinib (anaplastic lymphoma kinase [ALK] inhibitor) was planned for her with which she had a near complete response after 2 months. A high index of suspicion is required to differentiate IMT from other common causes of mesenteric masses in children and role of radiologist is quintessential in this regard. Local recurrence with abutment but without invasion of surrounding structures points to the intermediate malignant pathology of IMT and may provide a clue to diagnosis. Systemic therapy is effective in patients who are ALK positive and destructive surgery should be avoided.

Background: : The human body is almost 70% water and fluid imbalance has been a classic symptom of severe underlying conditions, i.e., hepatic disorders, kidney disorders, cardiovascular disease and inflammation. Boerhavia diffusa Linn. is a pantropical herb used in traditional medicine systems worldwide for inflammation, liver-kidney disorders, skin problems, etc. Ethnopharmacological reports of B. diffusa correlate well with its traditional uses. The roots and leaves of B. diffusa have demonstrated diverse pharmacological activities in in-vitro and in-vivo studies. It occurs in Asia, Africa, North America and South America, recognized by diffuse branches decorated with green, ovate leaves, sprouting in the rainy season. This beautiful plant is a source of various important phytochemicals, i.e., alkaloids, rotenoids, polyphenols, flavonoids, ecdysteroids, lignan, steroids, etc. Materials & Methods: : The objective of the work was to compile the reports emphasizing the triangular relationship among chemical constituents, pharmacological potential and traditional/ethnopharmacological uses of B.diffusa. A thorough literature search was carried out using various search engines. Literature reports were collected from these databases and print journals available in the library were referred. A combination of keywords: Boerhavia/Boerhaavia, punarnavine/lunamarine, rotenoids, isoflavonoids, boeravinone, diuretics, hepatoprotective, and edema were used. Manually articles were screened based on the title and abstract and their cross-references were studied. Inclusion criteria were original publications in peer-reviewed journals containing information on B. diffusa and its chemical constituents. Results: : There is abundant evidence of B. diffusa being an important remedy in generalized edema and kidney-liver disorder due to its diuretic and hepatoprotective activities. It is a source of more than 30 different rotenoids, having hepatoprotective, cardioprotective, antibacterial, antioxidant, antihypertensive activities. It is also a rich source of flavonoids that exhibit a plethora of activities, especially anti-inflammatory, antiosteoporotic and antioxidant activities. Its traditional properties in Ayurveda are proven in terms of its various mechanisms of action, which corresponds well with its ethnomedicinal knowledge worldwide. It has an exceptionally well nutritional profile even though it is a wasteland plant. Conclusion: : The phytochemical data indicate that the novel structure of compounds from B. diffusa makes them unique candidates for exploratory/combinatorial studies. Recently various computational and docking studies have been reported on these compounds. The pharmacological activities shown by B. diffusa are live proof of the potential of its chemical constituents and their molecular mechanism has been studied. It can be said that B. diffusa is an exceptionally good source of therapeutically active novel phytocompounds which can transform the field of hepatoprotective, nephroprotective and anticancer treatments.

Background: Anorectal melanoma (AM) is a rare subtype of melanoma. Aim: To study the clinic–pathologic features and outcomes in patients with AM. Materials & methods: Clinical, pathologic findings and outcomes of patients with AM were recorded. Results: Twenty-seven patients with AM were identified with median age of 57 years. Most patients presented in stage III (44.4%). Lymph node involvement was seen in 70.4%. The response to chemotherapy and immunotherapy was 16.6 and 25.0%, respectively. At a median follow up of 11 months, median overall survival was 30 months. Ballantine stage 3 and weight loss at presentation were predictors of poor survival. Conclusion: AM presents at an advanced stage with lymph node and distant metastasis.

Abstract Background Primary malignant melanoma of the female urethra is an exceedingly rare tumor. It represents 0.2% of all malignant melanomas. Divergent differentiation towards non-melanocytic lineages has not been reported in urethral melanoma. Case presentation We report a rare case of neuroendocrine differentiation in a large primary small cell malignant melanoma involving the urethra, in a 62-year-old lady, who presented with obstructive urinary symptoms. Clinical and radiological workup revealed a large urethral mass with liver and lymph nodal metastases. A biopsy was performed from the urethral and liver lesions which showed poorly differentiated tumor cells with small cell morphology and presence of melanin pigment. These cells were immunopositive for melanocytic and neuroendocrine markers. Ultrastructural examination showed presence of melanosomes and neurosecretory granules in the tumor cells. Conclusions Although malignant melanoma with neuroendocrine differentiation is exceptionally rare, it needs to be recognized among the other well-known variants of malignant melanoma.

Chemotherapy while revolutionizing cancer management by improving survival and quality of life; is also associated with several adverse effects. Lung is the most common organ affected in chemotherapy-related complications, due to either drug toxicity or more commonly due to infections caused by immunosuppression and less commonly due to immune-mediated injury. Radiology, when used in combination with clinical and lab data, can help reach the specific diagnosis or narrow down the differentials. The common radiological patterns of drug toxicity include pulmonary interstitial and airway infiltrates, diffuse alveolar damage, nonspecific interstitial pneumonia, eosinophilic pneumonia, cryptogenic organizing pneumonia, pulmonary hemorrhage, edema and hypertension. Cancer patients are immunosuppressed due to the underlying malignancy itself or due to therapy and are prone to a gamut of opportunistic infections including viral, bacterial, fungal and mycobacterial pathogens. Immune reconstitution inflammatory syndrome (IRIS), a well-known complication in HIV, is now being increasingly recognized in non-HIV patients with immunosuppression. Engraftment syndrome is specifically seen following hematopoietic stem cell transplant during neutrophil recovery phase. Pulmonary involvement is frequent, causing a radiological picture of noncardiogenic pulmonary edema. Thus, radiology in combination with clinical background and lab parameters helps in detecting and differentiating various causes of pulmonary complications. This approach can help alter potentially toxic treatment and initiate early treatment depending on the diagnosis.

Aim: Dermatofibrosarcoma protuberans (DFSP) accounts for less than 2% of all soft-tissue sarcomas. Patients & methods: We retrospectively reviewed our database for patients with locally advanced or metastatic DFSP who had presented to our clinic between January 2016 and January 2020. Results: We identified a total of 14 patients, of whom ten had sarcomatous transformation. Eleven cases had metastatic disease and three were locally advanced. The initial partial response rate to first-line imatinib was 76.9% and the overall median progression-free survival on imatinib was 15 months. Conclusion: We had a high proportion of patients with sarcomatous transformation, in contrast to their relative rarity in the West. While most patients had initial good responses to imatinib, second-line therapies were not as effective.

Background: SMARCA4 deficient uterine sarcoma (SDUS) is a relatively new entity added to the family of uterine sarcoma characterised by SMARCA4/BRG1 deficiency. Case: A 62 years old lady presented with abdominal pain and vaginal discharge. On evaluation, found to have a pelvic mass with lymph nodal involvement. She underwent hysterectomy with bilateral salpingo-oophorectomy and lymphadenectomy. Preliminary diagnosis made outside was endometrial stromal sarcoma. On further review, had epithelioid and rhabdoid morphology with SMARCA4 loss documented on comprehensive gene profiling. Recurrence within few months of surgery was seen. She was started on gemcitabine and taxol based chemotherapy, showing significant clinical and radiological improvement. Conclusion: Diagnostic dilemma of this infrequent, aggressive subtype of uterine sarcoma adds to the hindrance in early recognition. Identifying histology surmounted with gene profiling is helpful in establishing diagnosis resulting in early treatment and improving outcomes.

Importance The Cancer Aging Research Group (CARG) toxicity score is used to assess toxicity risk in geriatric patients receiving chemotherapy.Objective The primary aim was to validate the CARG score in geriatric patients treated with curative intent chemotherapy in predicting grade 3–5 toxicities.Design This was a longitudinal prospective observational study.Setting Tata Memorial Hospital, Mumbai, India, a tertiary cancer care referral centre.Participants Patients, aged ≥65 years, with gastrointestinal, breast or gynaecological stage I–III cancers being planned for curative intent chemotherapy. A total of 270 patients were required for accrual in the study.Exposure(s) Total risk score ranged from 0 (lowest toxicity risk) to 19 (highest toxicity risk).Main outcome(s) and measure(s) The primary endpoint of the study was to evaluate whether the CARG risk score predicted for grade 3–5 toxicities.Results The study cohort of 270 patients had a mean age of 69 (65–83) years, with the most common cancers being gastrointestinal (79%). Fifty-two per cent of patients had atleast one grade 3–5 toxicity. The risk of toxicity was increased with an increasing risk score (42% low risk, 51% medium risk and 79% high risk; p

Background: Reaching the correct histo-pathological diagnosis of soft tissue sarcomas (STS) is a great challenge and is cornerstone for treatment planning. Need of expertise for diagnosis is limited due to the lack of dedicated expert sarcoma pathologists and oncologists in India. In this study we highlight the pattern of pathological diagnosis and its accuracy outside specialist centre. Methods: We performed retrospective analysis of all patients referred to us with a clinical or histopathologic diagnosis of STS over the period January 2016 to December 2017. According to the protocol, all patients had a review of histopathology diagnosis from our institute. The tissue blocks if available were reviewed and a fresh biopsy was performed when required. The histopathologic diagnosis was also reviewed in the joint clinic, giving clinico-radiological inputs to sarcoma pathologists. For the patients with outside diagnosis and discordant report, we divided them into major discrepancy (including change of diagnosis of sarcoma to benign or other histological entity that could potentially change the treatment plan) or minor discrepancy (like mild change in grade or histopathological diagnosis not affecting the treatment plan). Statistical analysis was done by SPSS ver 23. Results: There were 149 patients with median age of 36 years (range 14-77 years), and 93 patients (62.4%) were males. About 57% (85 cases) of patients had localized disease. Most common subtypes were synovial sarcoma (16%), liposarcoma (9%), soft tissue ewings sarcoma (9%), MPNST (9%), leiomyosarcoma (8%), and undifferentiated pleomorphic sarcoma (8%). Of 149 patients, 47 (31.5%) had not been worked up outside by immunohistochemistry or other molecular studies and thus comparison was not possible; while 4 patients couldn’t retrieve blocks and repeat biopsy could not be performed. Of 97 patients (biopsy = 84, FNAC = 13) who had diagnosis from outside, 37% had major discrepancy and 24% had minor discrepancy as compared with our diagnosis from the sarcoma specialists. Univariate analysis revealed that the major discrepancy was more in non-extremity than extremity STS (p = 0.003). Conclusion: Histopathologic diagnosis in more than half of patients referred from outside centers was discordant with respect to the diagnosis of our centre with major implications on 37% of cases. We believe this is due to the lack of sarcoma pathology experts, and they are virtually non-existent in the multidisciplinary clinic set up outside the tertiary care centres.

Purpose Osteosarcoma (OS) is a relatively chemosensitive primary bone tumor, with the peak age of onset occurring in late childhood and early adolescence. The treatment paradigm of nonmetastatic OS has typically been multimodality therapy, including neoadjuvant and adjuvant chemotherapy with definitive surgery. Over the years, various permutations and combinations of chemotherapeutic agents have been used. However, the majority of recent trials have still used high-dose methotrexate as the backbone, with cisplatin and doxorubicin (MAP). In the last decade, various strategies targeted to improving outcomes in OS have included the addition of a fourth drug to the three-drug MAP regimen, changing therapy according to histopathologic response and the addition of immunotherapies. Through this review, we sought to underscore a few pertinent issues related to chemotherapy in nonmetastatic OS, with special reference to challenges confronted in Indian settings. Methods We reviewed the literature, focusing on studies comparing high-dose methotrexate and non–high-dose methotrexate–containing regimens. In addition, this review focuses on non–methotrexate-containing triple-drug therapy. Results Although a high-dose methotrexate regimen has become standard of care in developed countries, there are few data to suggest that it is superior to a non–high-dose methotrexate regimen. Conclusion Developing countries with lack of infrastructure and logistics for high-dose methotrexate might resort to non–high-dose methotrexate–containing regimens with a simultaneous focus on early detection, decreasing abandonment, multidisciplinary clinics, improved surgery, and meticulous pathologic evaluations.

Epithelioid sarcoma (ES) is a rare soft tissue sarcoma with an incidence of 0.05 per 100,000 population in the USA. It is characterized by multiple local recurrences and regional lymph nodes form the commonest site of metastases. The function of Integrase Inhibitor 1 (INI1) protein is lost in more than 90% of cases, which was the basis for the introduction of tazemetostat into the therapeutic armamentarium for management of advanced ES. The efficacy and manageable toxicity profile of tazemetostat have been demonstrated recently, leading to its accelerated approval for treatment of advanced ES. We report one of the first real-world cases of relapsed, metastatic ES treated with tazemetostat. The patient attained partial response with the therapy and is tolerating the drug well without serious toxicities.

The therapeutic landscape in advanced gastrointestinal stromal tumor has evolved. Avapritinib and ripretinib have now been approved by the US FDA for platelet-derived growth factor alpha D842V-mutant and refractory gastrointestinal stromal tumor patients, respectively. Here we report five patients who have been on avapritinib under an expanded access program. Response assessment was available for four patients – a partial response in two patients and stable disease in one, while one patient had progressive disease. Though preliminary results of the VOYAGER trial have shown less activity of avapritinib and no significant difference in progression-free survival when compared with regorafenib, avapritinib may show some clinical benefit in a subset of patients refractory to approved therapies. We share our experience of five cases, with clinical benefit in three. We believe avapritinib should be further evaluated in clinical trials.

Inflammatory myofibroblastic tumors (IMT) are rare soft tissue tumors of intermediate malignant potential occurring usually in children and adolescents. Treatment options for advanced diseases are limited. A 35-year-old lady presented to us with fever, cough and decreased appetite. On evaluation, she was diagnosed with left lung IMT. She underwent surgery and developed recurrence with pleural nodules after two years. Immunohistochemistry showed positivity for ALK (diffuse). Since recent evidence suggested that crizotinib is effective in advanced IMT with 50% response rates, she was treated with crizotinib 250mg BD with which she had a complete radiological response at three months. She has completed one year of treatment thus far and continues to be in complete remission. Treatment with ALK inhibitors like crizotinib has brought about a paradigm shift in the management of advanced ALK-positive IMT’s with excellent clinical responses which are durable in a majority of cases.

Leiomyosarcoma of the stomach is an extremely rare malignancy for which treatment in advanced disease is hardly reported. Here, we report a case of a 48-year-old man with metastatic gastric Leiomyosarcoma who had previously received a combination of gemcitabine and docetaxel followed by pazopanib after detection of metastasis. The patient was started on trabectedin as per protocol and had disease control continuing for 17 cycles of trabectedin. His quality of life and absence of significant toxicities highlight the noncumulative nature of trabectedin and potential benefit in responding cases.

Purpose: Advanced dermatofibrosarcoma protuberans (DFSP) is an exceptionally uncommon disease with scarce literature, especially from developing countries. Molecular testing is unfortunately not available in India, and expert diagnosis by a sarcoma pathologist is available only in tertiary care centers. Materials and Methods: We retrospectively analyzed consecutive patients with inoperable DFSP (on the basis of expert histopathology only) who presented to our sarcoma medical oncology clinic from January 2016 to July 2017. Results: There were a total of seven patients, with median age of 35 years, predominantly males (85.7%). Fibrosarcomatous variant and metastatic disease were present in six (85.7%) patients. Partial response rates were 71.4%, and overall disease control was 85.7%. Median progression-free survival was 14 months. Conclusion: DFSP diagnosis on the basis of expert histopathology in the absence of translocation can help out in targeted therapy–based treatment until translocation testing becomes available. The fibrosarcomatous variant has poor outcome, and further research is needed to help this group of patients.

Background: Head-and-neck soft-tissue sarcomas (HNSTS) are extremely rare and lack definite guidelines. Methods: We retrospectively analyzed consecutive adult patients with HNSTS who presented to our sarcoma medical oncology clinic from January 2016 to October 2017. Results: There were a total of 30 patients. Unresectable localized disease was seen in 13 (43%) patients, metastatic disease 10 (34%) patients, while resectable disease in 7 (23%) patients only. Among unresectable localized disease, 3 (25%) patients could be converted to resectable disease after neoadjuvant therapy. Median follow period was 11 months. Progression-free survival was 19 months in patients with resectable disease and 6 months in patients with the unresectable/metastatic disease. Median OS was not reached. Conclusion: Unresectable HNSTS has a poor outcome. Neoadjuvant therapy can be tried in selected cases for achieving respectability or for vital organ preservation until robust data are available. A multidisciplinary approach for local control is crucial in managing unresectable HNSTS.

Sarcomas are extremely complex and heterogeneous group of malignancies. However, exact categorization of the type of sarcoma is essential for the individualized approach for a given patient. It is mandatory that sarcomas should be treated in tertiary care centers with good pathology support and expertise. Here, we present an apt example of a young girl with large abdominal mass which was diagnosed as ovarian rhabdomyosarcoma (RMS). Besides, her excellent response to RMS regimen further reinforces the findings.

Introduction: The palatal rugae are anatomical folds or wrinkles located on the anterior third of the palate behind the incisive papilla on each side of the palatal raphe. The rugae patterns have been studied for various purposes mainly in the field of anthropology, comparative anatomy, genetics, prosthodontics, orthodontics and forensic odontology. This study is aimed to determine the difference in number and pattern of palatal rugae in males and females. Methods: This is a prospective study in which maxillary impressions of 50 volunteers, 25 males and 25 females were taken and the casts prepared. The boundaries of the rugae were marked with a HB pencil and were observed with a magnifying glass following the classification used by Kapali, et al, (1997). Association between rugae number and sex and rugae shape and sex were tested using two sample unpaired t test. Results: The study revealed significant difference in the pattern of converge rugae which were found to be higher in females than males, however, no statistical significant difference was found in the number of rugae between the two sexes. Conclusions: The rugae pattern can be an additional method of differentiation between the males and females in conjunction with the other methods used in forensic sciences. Keywords: forensic science; human identification; palatal rugae.

Introduction: Oral health reflects overall well-being for the elderly population. Compromised oral health may be a risk factor for systemic diseases commonly occurring in old age. Oral health evaluation should be an integral part of the physical examination, and dentistry is essential to qualify geriatric patient care. Aim: To determine the prevalence of oral soft tissue lesions and systemic diseases in institutionalized geriatric population in North India. Materials and Methods: Geriatric patients were clinically evaluated using a standard questionnaire and assessed for known medical illnesses and prevalence of oral soft tissue lesions. Four hundred patients (71% males and 29% females) with age ranging from 60 to 100 years were considered in the study group. Twenty-two (33.8%) patients were edentulous and seven patients (10.8%) were denture wearers. Forty-four (67.69%) patients reported with tobacco habits. Results: Most prevalent medical illness reported was diminished vision (15.5%), followed by hypertension (10%) and diabetes mellitus (6.25%). Several oral soft tissue lesions were reported among the study population. The most prevalent lesions were leukoplakia (12%), smoker′s melanosis (10%), smoker′s palate (9%), pigmentation on tongue (6%), frictional keratosis (5%), lichen planus (3%), denture stomatitis (2.5%), aphthous ulcers (2%), angular chelitis (1.5%), oral submucous fibrosis (1.5%), melanotic macule (1.5%), candidiasis (1.5%), irritation fibroma (1%), geographic tongue (1%), median rhomboid glossitis (1%), and traumatic ulcer (1%). Conclusion: The findings observed in this population are important and can have a determinant effect on the overall quality of life in this population. This information is a crucial prerequisite for health awareness programs involving the community health workers, oral physicians, and medical professionals.

Cysticercosis cellulosae, caused by the larval stage of Taenia solium is a common parasitic infection in Indian subcontinent. Although cysticercosis is common in other parts of the human body, its involvement with temporalis muscle is an extremely rare entity and demands documentation. This paper reports a case of cysticercosis cellulosae in a 35-year-old male patient within the temporalis muscle mimicking temporal space infection; due to the presence of concomitant dental infection, which was diagnosed with the help of high resolution ultrasonography and magnetic resonance imaging and managed conservatively using oral antiparasitic medication. Here, in this case report, we are emphasizing the importance of imaging modalities in diagnosing space infection and cysticercosis.