A Nonpediatric Extraosseous Ewing Sarcoma of the Pancreas: Differential Diagnosis and Therapeutic StrategiesYohannan, Binoy;Feldman, Mark
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/2792750pmid: 32082662
Extraosseous Ewing’s sarcoma is a rare and aggressive malignant tumor with a poor prognosis. The pancreas is an extremely uncommon primary site, with only 27 cases that have been published worldwide. We report a 26-year-old female who presented with 5 days of left upper quadrant pain, nausea, and vomiting. On examination, she was anicteric and had epigastric and left upper quadrant tenderness without guarding, rebound tenderness, or a palpable mass. She had slightly elevated serum aminotransferase and lipase levels. Abdominal computerized tomography revealed a multilobulated tumor arising from the body and tail of the pancreas. A biopsy confirmed a small round cell tumor, and immunohistochemistry was positive for CD99 in approximately 70% of the tumor cells. A fluorescence in situ hybridization (FISH) assay showed a 22q12 rearrangement. She was diagnosed with extraosseous Ewing sarcoma of the pancreas and underwent multiagent neoadjuvant chemotherapy followed by surgical resection, but subsequent imaging revealed evidence of systemic disease progression. She chose to go on hospice care and died a few weeks later.
Sistrunk Procedure on Malignant Thyroglossal Duct CystKartini, Diani;Panigoro, Sonar S.;Harahap, Agnes S.
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/6985746pmid: 32395358
A thyroglossal duct cyst is a lesion that occurs as a result from failure of the thyroglossal duct to obliterate during fetal development. Malignant progression is a rare event that might occur in less than 1% of all cases. Because of its rarity, there are conflicting opinions regarding the management of the case. In the present study, a 46-year-old male presented with a painless neck mass that had increased in size over the last 6 months. There was no difficulty in swallowing and breathing, change in voice, significant weight loss, or any signs of hyperthyroidism. Laboratory workup showed that results were within normal limits. Thyroid gland ultrasonography and cervical contrast CT scan revealed a complex cystic mass that pointed towards a thyroglossal duct cyst. We performed Sistrunk procedure. Postoperative pathology examination revealed microscopic appearance of the thyroglossal duct cyst with a classic follicular variant of papillary thyroid carcinoma. Our latest follow-up showed no signs of tumor recurrence or any complications following surgery on locoregional status. As a fine needle aspiration biopsy cannot ensure a precise result in all of cases, it is essential to perform a solid physical examination and thorough supporting examination in deciding the precise management for the patient.
A Huge Pelvic-Abdominal Malignant GIST Tumour in a Patient with Neurofibromatosis Type 1: Case Report and Literature ReviewOmar, Islam;Alsaati, Hani;Waris, Ejaz
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/6590307pmid: 31984144
Gastrointestinal stromal tumours are rare tumours of the gastrointestinal tract (GIT) accounting for 0.1%–3% of all gastrointestinal tumours. The most common location is the stomach (55%) followed by the small bowel (31.8%), colon (6%), other various locations (5.5%), and the oesophagus (0.7%). They may also occur in extraintestinal locations. The signs and symptoms of GIST depend on the tumour’s location and size. Gastrointestinal bleeding is one of the most common symptoms. Other signs and symptoms include abdominal discomfort, pain or distention; intestinal obstruction, and weight loss. The association between the development of GISTs and neurofibromatosis 1 (NF1) has been established. NF1-associated GISTs tend to have a distinct phenotype, and the absence of KIT/PDGRF<svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" style="vertical-align:-0.2063904pt" id="M1" height="6.1673pt" version="1.1" viewBox="-0.0498162 -5.96091 7.51131 6.1673" width="7.51131pt"><g transform="matrix(.013,0,0,-0.013,0,0)"><path id="g113-223" d="M545 106L524 126C493 85 467 65 455 65C438 65 427 113 405 238C448 295 498 362 543 439L533 448L478 435C453 386 423 331 398 295H395C370 404 347 448 282 448C169 448 23 309 23 153C23 54 65 -12 128 -12C203 -12 283 70 339 155H341C360 29 380 -12 411 -12C444 -12 491 11 545 106ZM333 204C265 95 210 54 169 54C137 54 113 96 113 171C113 302 191 405 252 405C301 405 318 306 333 204Z"/></g></svg> mutations in turn has implications on further management when they do not respond well to imatinib treatment. Here, we present one of the largest GISTs reported in the literature with a total volume of <span class="inline_break"><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="32.221pt" style="vertical-align:-0.3499298pt" id="M2" height="8.69875pt" version="1.1" viewBox="-0.0498162 -8.34882 32.221 8.69875"><g transform="matrix(.013,0,0,-0.013,0,0)"><path id="g113-51" d="M412 140C382 77 369 73 315 73H129L270 222C362 320 402 379 402 466C402 571 322 635 234 635C177 635 130 609 99 576L42 495L64 475C90 514 133 568 201 568C274 568 318 519 318 435C318 349 255 267 193 193C144 135 87 78 32 23V0H405C417 45 427 89 440 131L412 140Z"/></g><g transform="matrix(.013,0,0,-0.013,6.24,0)"><path id="g113-54" d="M153 550H386L412 615L406 623H120L82 318C104 327 142 338 184 338C294 338 347 275 347 187C347 112 305 39 221 39C160 39 119 71 97 89C88 97 80 96 71 90C59 80 50 67 49 57C48 45 52 36 66 23C80 9 123 -12 169 -12C221 -11 288 15 342 59C403 109 431 165 431 225C431 308 366 395 238 395C212 395 165 379 127 364L153 550Z"/></g><g transform="matrix(.013,0,0,-0.013,12.48,0)"><path id="g113-47" d="M113 -12C146 -12 170 11 170 46C170 78 146 103 114 103S58 78 58 46C58 11 82 -12 113 -12Z"/></g><g transform="matrix(.013,0,0,-0.013,15.444,0)"><path id="g113-52" d="M285 378C315 398 338 416 353 432C373 451 384 474 384 503C384 579 325 635 236 635H235C182 635 136 610 108 579L65 516L85 496C110 533 150 575 205 575C258 575 300 543 300 481C300 407 232 369 141 339L147 310C163 315 188 321 211 321C268 321 338 284 338 192C338 94 288 40 217 40C160 40 119 68 93 91C85 98 77 97 69 91C60 84 47 71 46 58C44 46 48 35 62 22C75 10 116 -12 162 -12C234 -12 424 62 424 224C424 297 373 359 285 376V378Z"/></g><g transform="matrix(.013,0,0,-0.013,24.59,0)"><path id="g117-42" d="M528 54L331 254L528 455L492 493L294 291L96 493L60 455L257 254L60 54L96 16L294 217L492 16L528 54Z"/></g></svg><span class="ibiop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="23.017pt" style="vertical-align:-0.3499298pt" height="8.69875pt" version="1.1" viewBox="35.076183799999995 -8.34882 23.017 8.69875"><g transform="matrix(.013,0,0,-0.013,35.126,0)"><use xlink:href="#g113-51"/></g><g transform="matrix(.013,0,0,-0.013,41.366,0)"><path id="g113-49" d="M241 635C89 635 35 457 35 312C35 153 89 -12 240 -12C390 -12 443 166 443 312C443 466 390 635 241 635ZM238 602C329 602 354 454 354 312C354 172 330 22 240 22C152 22 124 173 124 313S148 602 238 602Z"/></g><g transform="matrix(.013,0,0,-0.013,50.512,0)"><use xlink:href="#g117-42"/></g></svg><span class="ibiop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="41.399pt" style="vertical-align:-0.3499298pt" height="8.69875pt" version="1.1" viewBox="60.9981838 -8.34882 41.399 8.69875"><g transform="matrix(.013,0,0,-0.013,61.048,0)"><path id="g113-50" d="M384 0V27C293 34 287 42 287 114V635C232 613 172 594 109 583V559L157 557C201 555 205 550 205 499V114C205 42 199 34 109 27V0H384Z"/></g><g transform="matrix(.013,0,0,-0.013,67.288,0)"><path id="g113-53" d="M456 178V225H360V632H320C217 496 115 347 20 206V178H280V106C280 40 276 34 189 27V0H445V27C364 34 360 39 360 106V178H456ZM280 225H82C149 335 214 431 278 520H280V225Z"/></g><g transform="matrix(.013,0,0,-0.013,75.704,0)"><path id="g190-100" d="M390 111C344 68 312 56 269 56C212 56 118 102 118 241C118 346 175 401 241 401C277 401 312 388 342 360C350 352 355 349 361 349C372 349 394 371 394 392C394 403 391 411 378 422C362 436 329 449 288 449H287C250 449 190 432 138 392C71 341 37 274 37 197C37 90 112 -12 238 -12C297 -12 363 32 407 90L390 111Z"/></g><g transform="matrix(.013,0,0,-0.013,81.203,0)"><path id="g190-110" d="M797 0V26C739 32 732 36 732 103V296C732 394 682 449 605 449C576 449 550 437 529 423C504 407 475 389 446 366C425 418 382 449 334 449C303 449 279 437 253 421C222 403 201 385 180 371V452C135 432 85 419 41 411V388C99 379 102 374 102 310V103C102 38 93 32 27 26V0H238V26C189 32 180 38 180 103V338C210 363 250 390 289 390C351 390 377 348 377 275V103C377 37 368 32 306 26V0H520V26C465 32 456 38 456 101V296C456 314 455 326 453 338C491 369 529 390 565 390C628 390 653 345 653 274V107C653 36 642 32 583 26V0H797Z"/></g><g transform="matrix(.013,0,0,-0.013,94.816,0)"><path id="g117-36" d="M535 230V280H323V490H265V280H52V230H265V-3H323V230H535Z"/></g></svg><span class="ibiop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="32.223pt" style="vertical-align:-0.3499298pt" height="8.69875pt" version="1.1" viewBox="105.3031838 -8.34882 32.223 8.69875"><g transform="matrix(.013,0,0,-0.013,105.353,0)"><use xlink:href="#g113-51"/></g><g transform="matrix(.013,0,0,-0.013,111.593,0)"><path id="g113-56" d="M447 623H65C61 580 56 530 47 475H76C100 541 106 550 172 550H388C308 376 196 170 91 -1L98 -12L172 -2C268 204 360 408 455 611L447 623Z"/></g><g transform="matrix(.013,0,0,-0.013,117.835,0)"><use xlink:href="#g113-47"/></g><g transform="matrix(.013,0,0,-0.013,120.799,0)"><path id="g113-58" d="M244 635C114 635 38 519 38 422C38 317 111 240 217 240C236 240 255 244 277 256L345 292C311 140 203 39 59 15L64 -15C89 -15 150 -5 204 17C339 72 440 202 440 386C440 521 368 635 244 635ZM228 602C326 602 352 479 352 390C352 370 351 347 348 324C327 308 293 296 258 296C174 296 124 369 124 458C124 517 152 602 228 602Z"/></g><g transform="matrix(.013,0,0,-0.013,129.945,0)"><use xlink:href="#g117-42"/></g></svg><span class="ibiop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="23.017pt" style="vertical-align:-0.3499298pt" height="8.69875pt" version="1.1" viewBox="140.4321838 -8.34882 23.017 8.69875"><g transform="matrix(.013,0,0,-0.013,140.482,0)"><use xlink:href="#g113-51"/></g><g transform="matrix(.013,0,0,-0.013,146.722,0)"><use xlink:href="#g113-52"/></g><g transform="matrix(.013,0,0,-0.013,155.868,0)"><use xlink:href="#g117-42"/></g></svg><span class="ibiop"/><svg xmlns:xlink="http://www.w3.org/1999/xlink" xmlns="http://www.w3.org/2000/svg" width="25.187pt" style="vertical-align:-0.3499298pt" height="8.69875pt" version="1.1" viewBox="166.3541838 -8.34882 25.187 8.69875"><g transform="matrix(.013,0,0,-0.013,166.404,0)"><path id="g113-57" d="M249 635C141 635 70 555 70 471C70 401 114 353 179 316C143 294 106 267 90 252C68 231 45 202 45 157C45 50 130 -12 237 -12C322 -12 435 52 435 169C435 256 372 304 303 343C349 374 375 398 383 407C401 429 411 458 411 487C411 569 344 635 249 635ZM238 603C285 603 337 567 337 482C337 422 310 385 276 358C205 393 145 426 145 500C145 552 179 603 238 603ZM248 20C183 20 125 70 125 163C125 218 158 268 206 300C284 261 355 217 355 143C355 66 308 20 248 20Z"/></g><g transform="matrix(.013,0,0,-0.013,174.819,0)"><use xlink:href="#g190-100"/></g><g transform="matrix(.013,0,0,-0.013,180.318,0)"><use xlink:href="#g190-110"/></g></svg></span> and an overall weight of 7.3 kg, which developed in a 43-year-old female patient with NF1 and was resected on an emergency basis due to the rapid deterioration and development of abdominal compartment syndrome. Pathology assessment showed a malignant GIST composed of spindle cells with elongated nuclei with necrosis, marked pleomorphism and numerous giant cell. The mitotic count was >15/50 HPF, Ki 67 was 80%, and the lymphovascular invasion was clear. Immunohistochemistry investigations showed that Vimentin, CD117, and DOG1 were positive, while BCL-2 and CD99 were focal positives. Pan-CK, S-100, CD34, Desmin, SMA, and HMB-45 were negatives.
Undifferentiated Nasopharyngeal Carcinoma Presenting with Shoulder MassIrawan, Cosphiadi;Cahyanur, Rahmat;Yunus, Reyhan Eddy
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/2586248pmid: 32047682
Nasopharyngeal cancer (NPC) is the most common cancer among head and neck cancer that usually presented with unilateral neck mass. Unusual symptoms of NPC can lead us to diagnosis misleading and delayed definitive treatment. We present a case of NPC with bone metastasis in the shoulder. A 33-year-old female presented with right shoulder mass caused by undifferentiated carcinoma of unknown primary, based on biopsy of shoulder mass. After four months, she was complaining painless neck swelling, headache, and hearing impairment in the left ear. Bone MRI showed malignant bone tumour in the right humerus. Neck CT scan showed mass in the nasopharyngeal and bilateral lymphadenopathy. Biopsy in nasopharyngeal revealed undifferentiated carcinoma of nasopharyngeal cancer (WHO-3 type A). The patient was diagnosed as NPC stage IVb and thus was treated with palliative chemotherapy. After three cycles of cisplatin docetaxel, patient condition improved.
A 5-Fluorouracil-Induced Hyperammonemic Encephalopathy Challenged with CapecitabineChahin, Michael;Krishnan, Nithya;Chhatrala, Hardik;Shaikh, Marwan
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/4216752pmid: 32082663
Cancer patients presenting with altered mental status demand a broad differential with early recognition of the etiology. Failure to do so is associated with increased morbidity and mortality. Causes that must be considered include organ involvement of the cancer, electrolytes abnormalities, and even chemotherapeutic agents. A 32-year-old female patient had been recently started on FOLFOX for metastatic colon cancer. Her initial treatments were uneventful, but she later developed encephalopathy during day three of cycle five. During her evaluation, she was found to have hyperammonemia (84 mcmol/L), without hepatic failure, that resolved with stopping chemotherapy and supportive care. After a trial of home infusion fluorouracil, she developed hyperammonemic encephalopathy again. During both admissions, her symptoms resolved with IV hydration and cessation of chemotherapy. She was then successfully challenged with capecitabine (1000 mg/m<sup>2</sup> daily), and additional hydration, and continued chemotherapy without recurrence of symptoms. Hyperammonemia is associated with fluorouracil though the mechanism is unclear. Suspected etiologies include either elevated levels of the drug due to slower metabolism or accumulation of certain metabolites. Additionally, risk factors such urease-producing bacterial infections, dehydration, and increased catabolism are thought to increase the risk for hyperammonemia. This case demonstrates the need for greater awareness of fluorouracil as a cause of hyperammonemic encephalopathy. Knowledge of this may allow for earlier recognition and reduced unnecessary testing.
A Rare Case of Intracranial Nongerminomatous Germ Cell Tumor in a 21-Year-Old Romanian MaleNandi, Minesh;Anil, Rahul;Hamaty, Edward;Adams, William;Stidd, David;Garde, Krizelle;Kandukuri, Hari
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/3787250pmid: 31984143
Extragonadal germ cell tumors are a rare entity that is more prevalent in infants and young children, with preference to midline structures. The category of intracranial germ cell tumors is divided into pure germ cell tumors (GCTs) versus nongerminomatous germ cell tumors (NGGCTs). They are usually present in the second decade of life with a male preponderance. We present here a rare case of intracranial NGGCT in a 21-year-old Romanian male, who presented with complaints of emesis, ataxic gait, and diplopia. A computed tomography scan of the head in the emergency department revealed a pineal/suprapineal mass along with obstructive hydrocephalus and dilated lateral and third ventricles without any bleeding. MRI of the cervical, thoracic, and lumbar spine showed no evidence of leptomeningeal metastasis. The patient had elevated serum markers of beta-hCG and AFP, which pointed towards a diagnosis of nongerm cell tumor, as in pure GCTs, these markers are normal. To relieve the obstruction from the mass effect, the patient had an endoscopic third ventriculostomy (EVT). However, after the procedure, he developed central diabetes insipidus as a complication with a triphasic response. Biopsy of the mass revealed atypical cells with granular architecture and atypical glands with positive immune histological markers for NGGCT. These findings supported the diagnosis of mixed germ cell tumor with yolk sac carcinoma and seminoma components. Patient’s transient central diabetes resolved with normalization in his urine output. He was eventually stabilized and returned to Romania for further management. In summary, intracranial germ cell tumors are rare brain tumors that should be distinguished based on histology and tumor markers as they will help in the guidance of therapy. An initial evaluation with neuroimaging, tumor markers, cytology from CSF, and biopsy is a must to distinguish further treatment and prognosis.
Combined Cerebellar and Spinal Cord Deficits Caused by an Underlying Gynecologic MalignancyOthman, Tamer;Hendizadeh, Moshe-Samuel;Vankina, Ritika;Park, Susan;Kim, Phyllis
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/9021843pmid: 31970005
Paraneoplastic cerebellar degeneration (PCD) is an uncommon autoimmune disorder targeting antigens within the nervous system and is usually associated with an underlying malignancy. Neurologic symptoms frequently precede the cancer diagnosis, which is most often seen in women with breast or gynecologic tumors. Anti-Yo-related PCD is the most common PCD syndrome, and one of the best understood. Although cerebellar signs are characteristic of anti-Yo PCD, myelopathy is an unusual presentation of anti-Yo PCD based on published case series and reports. Unfortunately, the prognosis for anti-Yo PCD is often poor, and most patients become bedridden. We report a case highlighting a severe presentation of cerebellar degeneration along with an unusual finding of myelopathy in a patient with a newly diagnosed gynecologic cancer.
Disseminated Intravascular Coagulation and Malignancy: A Case Report and Literature ReviewSohal, Sumit;Thakur, Akhilesh;Zia, Aleena;Sous, Mina;Trelles, Daniela
2020 Case Reports in Oncological Medicine
doi: 10.1155/2020/9147105pmid: 31976103
Disseminated Intravascular Coagulation (DIC) is a disorder of coagulation which is commonly seen as a complication of infections, traumas, obstetric diseases, and cancers especially hematological and rarely solid cancers. DIC may rarely be the presenting feature of an undiagnosed malignancy. It may present in the form of different phenotypes which makes its diagnosis difficult and leads to high mortality. The treatment comprises supportive, symptomatic treatment and removal of the underlying source. Here, we present a patient with history of being on warfarin for atrial fibrillation and other comorbidities who presented with elevated INR of 6.3 and increasing dyspnea on exertion. Over the course of her stay, her platelet counts started dropping with a concurrent decrease in fibrinogen levels. She eventually developed pulmonary embolism, followed by stroke and limb ischemia, which was indicative of the thrombotic phenotype of DIC. Her pleural fluid analysis showed huge burden of malignant cells in glandular pattern suggestive of adenocarcinoma and was started on heparin drip. However, the patient had cardiac arrest and expired on the same day of diagnosis.