Indian experience with immunotherapy in sarcoma and gastrointestinal stromal tumors: a retrospective study.

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.

Malignant Melanoma of Glans Penis: Findings on 18F FDG PET/CT.

ABSTRACT: Melanomas usually arise in the sun exposed parts of the skin. Mucosal melanoma is a rare condition. Melanoma of glans penis is even a rarer phenomenon with less than 250 cases reported in literature so far. Here we present a case of a 55-year-old man diagnosed with melanoma of glans penis with findings on 18F FDG PET/CT scan done for metastatic workup. 18F-FDG PET/CT showed primary in the glans penis with metastases in the right supraclavicular, mediastinal, and abdominal lymph nodes.

Renal Cell Carcinoma Mimicking with Peritoneal Carcinomatosis and Krukenberg Tumor: Diagnosis Seen on Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography.

Krukenberg tumor (KT) is a rare clinical entity with a mysterious origin. It originates most commonly from adenocarcinoma of the stomach. We present an interestingly rare case of this entity in renal cell carcinoma, revealed by 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (PET/CT) scan. Ovarian cancers with diffuse peritoneal metastasis were considered the differential diagnosis of the disease, based on PET/CT. The potential efficacy of this functional imaging for KT is still in the exploratory phase, but its applications in diagnosis, disease prognostication, therapeutic response monitoring, and follow-up recurrence detection are superior than other imaging modalities.

Solitary Subcutaneous Tissue Metastasis as Recurrence in a Case of Primary Angiosarcoma of Breast: Findings on 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography.

Primary angiosarcomas of the breast are rare tumors, with a fatal outcome. We present a rare case of an operated primary angiosarcoma of the right breast in a 20-year-old female who showed disease recurrence in the right posterior arm subcutaneous tissue on 18F-fluorodeoxyglucose positron emission tomography-computed tomography after 1 year of surgery without any other visceral metastasis.

Unfolding the rarity of SMARCA4 deficient uterine sarcoma (SDUS): A case report.

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.

Paraneoplastic Cutaneous Mucinosis as Initial Presentation of Recurrence in an Operated Case of Carcinoma Ovary: True Extent of Recurrent Metastatic Disease Unveiled on 18F Fluoro-D-Glucose Positron Emission Tomography-Computed Tomography.

Ovarian carcinoma is associated with many cutaneous and paraneoplastic manifestations. We present a case of 52-year-old female who underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy for epithelial origin ovarian carcinoma. She presented with skin rashes over the face and neck region after 2 years. 18F-fluoro-D-glucose positron emission tomography-computed tomography scan revealed recurrent metastatic retroperitoneal lymph nodes.

Extensive Extranodal Cutaneous Lymphomatous Involvement in a Case of Adult T-Cell Lymphoma - Advantage of 18F Fluorodeoxyglucose Positron-emission Tomography-Computed Tomography over Computed Tomography.

Diffuse extranodal cutaneous lymphomatous involvement is a unique presentation in a case of adult T-cell lymphoma. We present the case of a 26-year-old female who presented with erythematous rashes with subsequent evaluation with 18F fluorodeoxyglucose positron-emission tomography-computed tomography showing enlarged inguinal and axillary lymph nodes, and biopsy findings from the inguinal lymph nodes were suggestive of adult T-cell lymphoma.

Avapritinib in advanced gastrointestinal stromal tumor: case series and review of the literature from a tertiary care center in India.

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.

Isolated Pancreatic Metastasis From Choroidal Melanoma After 10 Years of Enucleation Mimicking Neuroendocine Tumor on 68Ga-DOTANOC PET/CT.

Isolated pancreatic metastasis from choroidal melanoma is a rare phenomenon. We describe a case of a 38-year-old woman who underwent enucleation surgery for right choroidal melanoma and subsequently developed lesions in the pancreas 10 years after enucleation, which were depicted on Ga-DOTANOC PET/CT and confirmed to be metastasis from choroidal melanoma on surgical histopathology.

Recurrent Cavitary Pulmonary Metastasis from Osteosarcoma: Findings on 18F-Fluorodeoxyglucose Positron Emission Tomography.

Spontaneous cavitation in primary lung malignancies although common, but is rare in secondary metastatic lung tumors. We present a case of 20-year-old male who presented with cavitary left lung lesion 1-year postexcision of the left tibia osteosarcoma on 18F-fluorodeoxyglucose positron emission tomography-computed tomography which on histopathology confirmed as metastatic lesion secondary to the sarcomatous primary.

3P's of Wermer/MEN1 Syndrome on 68Ga-DOTANOC PET/CT Scan.

Wermer/MEN1 syndrome is composed of the triad of pancreatic, pituitary, and parathyroid (3P's) tumors. We describe the Ga-DOTANOC PET/CT findings of a 43-year-old man, which revealed the classic 3P's of MEN1 syndrome with somatostatin receptor expression. The patient further underwent surgery for parathyroid lesions, which were suggestive of adenomas.

Scrotal Sac Metastasis in Adenocarcinoma Prostate: Findings on 68Ga-PSMA PET/CT.

Metastasis to the scrotal sac is a very rare phenomenon. We present a case of a 45-year-old man diagnosed with locally advanced acinar adenocarcinoma prostate, post bilateral orchidectomy and radiotherapy treatment, who showed a rising trend of serum PSA levels with discovery of scrotal sac metastasis on serial Ga-prostate-specific membrane antigen PET/CT scans.

Isolated Lymph Node Amyloidosis: Response Assessment to Chemotherapy on Serial 18F-FDG PET/CT Scans.

Amyloidosis is a disorder resulting from the deposition of fibrillary protein in the extracellular tissue and can be classified into primary, secondary, familial, and senile types. Isolated lymph node amyloidosis without any other organ involvement is very rarely seen in clinical parlance, and diagnosis remains very challenging owing to nonspecific imaging findings. We present a case of 50-year-old man with lymphadenopathy, which was later confirmed to be amyloidosis on biopsy and serum-free light chain assay with efficacious use of F-FDG PET/CT for response assessment to bortezomib, cyclophosphamide, and dexamethasone.

A Rare Case of Sinonasal Malignant Melanoma - Local, Regional, and Distant Spread Accurately Detected by 18F Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.

Melanomas are usually cutaneous in origin but rarely can also arise from the mucosal linings in the body. Sinonasal melanomas arise from the mucosa of the nasal cavity and paranasal sinuses, which account for approximately 50% of mucosal melanomas in the body. 18F fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT) has proven its role in malignant melanoma in staging especially in stage III and IV disease, restaging, assessing response to therapy and had affected in treatment change in significant number of cases. We present a case of sinonasal melanoma who underwent FDG PET/CT for staging and showed cervical lymph node and marrow metastases.

Inflammatory Myofibroblastic Tumor: Findings on 68Ga-DOTA-NOC Positron-emission Tomography-Computed Tomography.

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumors are a rare group of tumors usually affecting children and young adults. They occur in various anatomic locations, with most common being the lung accounting for almost 95% of the cases. We present a case of a 4-year-old girl who presented with fever and dull abdominal pain for 6 months with a clinically palpable left hypochondrium mass. On suspicion of mesenteric/gastric neuroendocrine tumor, 68Ga-DOTA-NOC positron-emission tomography-computed tomography was done; however, the biopsy revealed IMT.

A Case of Metachronous Triple Carcinoma with Synchronous Double Primary Carcinoma on 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography.

Multiple primary malignancies in a single patient are exceedingly rare, but their prevalence has increased in recent decades due to prolonged survival rates supported by the advent of newer and better generation of chemotherapeutic agents as well as advances in cancer detectability facilitated by sophisticated modalities such as positron emission tomography-computed tomography. Here, we discuss a case of a 66-year-old male who recovered completely from lung carcinoma but subsequently developed synchronous gall bladder and gastric carcinoma after 1 year.

Low-Grade Thymoma with Osseous and Pulmonary Metastases: Role of 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography in Initial Staging.

Thymomas are rare anterior mediastinal tumors that originate in the epithelial cells of the thymus and have a rare propensity to metastasize to extrathoracic locations unless it is a histologic high-grade neoplasm (type B and above). We describe a case of 50-year-old woman diagnosed with type AB thymoma and the role of 18F-fluorodeoxyglucose positron emission tomography-computed tomography in accurate delineation of extrathoracic metastases during initial staging.

Right Ventricle Metastasis from Carcinoma Rectum: Findings on 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography.

Rectal adenocarcinoma metastasizes most commonly to the lungs and liver. Metastasis to heart, although described in literature, is a very rare phenomenon. We describe the 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) findings of a 45-year-old male who was a biopsy-proven case of adenocarcinoma rectum. Apart from metastatic disease involving liver, lungs, bone marrow, and lymph nodes, metastasis to right ventricle was also seen on PET-CT scan.

Adrenocortical Carcinoma with Inferior Vena Cava Thrombus on 18F-FDG-PET-Computed Tomography.

Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignant neoplasm which can produce intravascular extension into the inferior vena cava (IVC) and can rarely extend into the right atrium. We describe the 18F Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography findings of a 57-year-old man diagnosed with ACC with IVC thrombus extending up to the right atrium.