Case Reports in Oncological Medicine The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Familial Lymphoproliferative Malignancies and Tandem Duplication of NF1 Gene Sun, 14 Dec 2014 09:11:15 +0000 Background. Neurofibromatosis type 1 is a genetic disorder caused by loss-of-function mutations in a tumor suppressor gene (NF1) which codifies the protein neurofibromin. The frequent genetic alterations that modify neurofibromin function are deletions and insertions. Duplications are rare and phenotype in patients bearing duplication of NF1 gene is thought to be restricted to developmental abnormalities, with no reference to cancer susceptibility in these patients. We evaluated a patient who presented with few clinical signs of neurofibromatosis type 1 and a conspicuous personal and familiar history of different types of cancer, especially lymphoproliferative malignancies. The coding region of the NF-1 gene was analyzed by real-time polymerase chain reaction and direct sequencing. Multiplex ligation-dependent probe amplification was performed to detect the number of mutant copies. The NF1 gene analysis showed the following alterations: mosaic duplication of NF1, TRAF4, and MYO1D. Fluorescence in situ hybridization using probes (RP5-1002G3 and RP5-92689) flanking NF1 gene in 17q11.2 and CEP17 for 17q11.11.1 was performed. There were three signals (RP5-1002G3conRP5-92689) in the interphases analyzed and two signals (RP5-1002G3conRP5-92689) in 93% of cells. These findings show a tandem duplication of 17q11.2. Conclusion. The case suggests the possibility that NF1 gene duplication may be associated with a phenotype characterized by lymphoproliferative disorders. Gustavo Fernandes, Mirela Souto, Frederico Costa, Edite Oliveira, and Bernardo Garicochea Copyright © 2014 Gustavo Fernandes et al. All rights reserved. Metastasis of Laryngeal Squamous Cell Carcinoma to Bilateral Thigh Muscles Sun, 14 Dec 2014 08:09:10 +0000 Importance. Laryngeal cancer infrequently results in distant metastases, but metastasis to skeletal muscle is extremely uncommon. Observations. A 55-year-old male presenting with progressive dyspnea and hoarseness was found to have Stage IVA T4aN2cM0 laryngeal cancer and eventually underwent total laryngectomy. Before the patient could be started on adjuvant chemoradiation, the patient developed masses on both thighs. Biopsy revealed metastatic squamous cell carcinoma consistent with the primary laryngeal cancer. He was offered palliative chemotherapy; however, he developed new soft tissue masses to the left of his stoma and in the prevertebral area one week later. He also had new cervical and supraclavicular nodes and a pathological compression fracture of L3. Patient died within 4 months of diagnosis. Conclusions. Distant metastasis such as skeletal metastasis portends a poor prognosis. Further studies are required to determine the best course of treatment in these patients. Zarah Lucas, Akash Mukherjee, Stanley Chia, and Irina Veytsman Copyright © 2014 Zarah Lucas et al. All rights reserved. Postoperative Radiation Therapy for Parotid Mucoepidermoid Carcinoma Sun, 14 Dec 2014 00:10:48 +0000 Salivary gland cancers are rare and represent approximately 5% of all head and neck cancers and only 0.3% of all malignancies. The majority (75%) of salivary gland tumors occur in the parotid gland, and while benign lesions are more common, mucoepidermoid carcinoma (MEC) makes up 40–50% of malignant parotid gland tumors. No randomized controlled trials exist regarding the role of adjuvant radiation for patients who undergo surgical resection of low-grade MECs. Herein, we report two cases of successful postoperative radiation therapy in low-grade, pT2N0 MEC of the parotid gland. The role of adjuvant radiation therapy for patients with MEC of the parotid gland is based on data from institution reviews and lacks data from randomized controlled trials. Per our review of the literature, the pathological findings of positive surgical margins and/or perineural invasion in two patients with low-grade MEC of the parotid gland warranted adjuvant radiation for improved local control after partial parotidectomy. Both patients tolerated postoperative radiation therapy with only mild side effects and, at last follow-up, five years after completion of therapy, had no clinical or radiographic evidence of either local recurrence or distant metastasis. Meghan P. Olsen, Allen O. Mitchell, and Edward F. Miles Copyright © 2014 Meghan P. Olsen et al. All rights reserved. Cutaneous Angiosarcoma of the Foot: A Case Report and Review of the Literature Tue, 09 Dec 2014 10:04:33 +0000 Primary Angiosarcoma of the skin of the foot is very rare. Angiosarcoma is typically treated with resection and wide-field postoperative radiation therapy. Chemotherapy and radiation therapy have also been used. Regardless of the treatment, the risk of local and distant relapse remains high for this disease. We present a case of an elderly patient who developed cutaneous angiosarcoma of the foot. It posed as a diagnostic dilemma at presentation. Chronic lymphedema was a possible predisposing factor. Given his age, preexisting renal dysfunction, refusal of surgery, and preference not to receive chemotherapy, the patient was ultimately treated with definitive radiotherapy. We present this case because of its rare site, unique presentation and delay in diagnosis of the condition, and attainment of an excellent response to radiation at the time of follow-up. We also review the current literature on this topic. Sharang Tenjarla, Lucy Ashley Sheils, Theresa M. Kwiatkowski, and Sheema Chawla Copyright © 2014 Sharang Tenjarla et al. All rights reserved. MYST3/CREBBP Rearranged Acute Myeloid Leukemia after Adjuvant Chemotherapy for Breast Cancer Mon, 08 Dec 2014 00:10:20 +0000 Although rare, clinicians and patients must be aware that therapy related malignancies, specifically acute myeloid leukemia (AML), can occur as a complication of adjuvant chemotherapy for breast cancer. Vigilance for signs and symptoms is appropriate. AML with t (8;16) is a specific translocation leading to formation of a fusion protein (MYST3/CREBBP). The MYST3/CREBBP AML tends to develop within 2 years of adjuvant chemotherapy, especially for breast cancer, without preceding myelodysplasia. It usually presents with disseminated intravascular coagulation and osteolytic lesions and has a poor prognosis despite aggressive resuscitation and therapy. With the increasing use of adjuvant chemotherapy for breast cancer, we are seeing a definite increase in the incidence of therapy related myelodysplastic syndromes and AML. One must keep this complication in mind while counseling and following up breast cancer patients who have received adjuvant chemotherapy. New osteolytic bone lesions in a patient with history of breast cancer do not necessarily mean metastatic disease and should be fully evaluated. Arjun Gupta, Mrinal M. Patnaik, and Harris V. Naina Copyright © 2014 Arjun Gupta et al. All rights reserved. Giant Malignant Phyllodes Tumour of Breast Thu, 04 Dec 2014 00:10:12 +0000 The term phyllodes tumour includes lesions ranging from completely benign tumours to malignant sarcomas. Clinically phyllodes tumours are smooth, rounded, and usually painless multinodular lesions indistinguishable from fibroadenomas. Percentage of phyllodes tumour classified as malignant ranges from 23% to 50%. We report a case of second largest phyllodes tumour in a 35-year-old lady who presented with swelling of right breast since 6 months, initially small in size, that progressed gradually to present size. Examination revealed mass in the right breast measuring  cms with lobulated firm surface and weighing 10 kgs. Fine needle aspiration cytology was reported as borderline phyllodes; however core biopsy examination showed biphasic neoplasm with malignant stromal component. Simple mastectomy was done and specimen was sent for histopathological examination which confirmed the core biopsy report. Postoperatively the patient received chemotherapy and radiotherapy. The patient is on follow-up for a year and has not shown any evidence of metastasis or recurrence. Ramakrishnan Krishnamoorthy, Thejas Savasere, Vinod Kumar Prabhuswamy, Rajashekhara Babu, and Sadashivaiah Shivaswamy Copyright © 2014 Ramakrishnan Krishnamoorthy et al. All rights reserved. A Case of Multiple Myeloma with Metachronous Chronic Myeloid Leukemia Treated Successfully with Bortezomib, Dexamethasone, and Dasatinib Tue, 02 Dec 2014 09:22:26 +0000 The coexistence of multiple myeloma and chronic myeloid leukemia in a single patient is a very rare event that has been reported very infrequently in the literature. We report a case of a patient who developed chronic myeloid leukemia four years after his diagnosis with multiple myeloma. Historically, no link between the two malignancies has been identified. This synchronous existence complicates the treatment plan for these patients, and there is a lack of evidence on the best therapeutic approach. Our patient was successfully treated with a combination of bortezomib, dexamethasone, and dasatinib, which he tolerated well for eleven months until he eventually succumbed to cardiac complications and pulmonary hypertension leading to his death. Samer Alsidawi, Abhimanyu Ghose, Julianne Qualtieri, and Neetu Radhakrishnan Copyright © 2014 Samer Alsidawi et al. All rights reserved. Familial Pancreatic Cancer: The Case for Prophylactic Pancreatectomy in Lieu of Serial Screening and Shared Decision Making Thu, 20 Nov 2014 12:21:35 +0000 At-risk family members with familial pancreatic cancer (FCaP) face uncertainty regarding the individual risk of developing pancreatic cancer (CaP) and whether to choose serial screening or prophylactic pancreatectomy to avoid CaP. We treated 2 at-risk siblings with a history of FCaP, congenital hepatic fibrosis (CHF), and jaundice secondary to a bile duct stricture. In one, a pancreaticoduodenal resection was done and in the second a total pancreatectomy. Malignancy was not present, but extensive pancreatic intraepithelial neoplasia (PanIn) 2 was present throughout both pancreata. The clinical course and literature review are presented along with the previously unreported association of CHF and CaP. Mazen E. Iskandar, Michael G. Wayne, Justin G. Steele, and Avram M. Cooperman Copyright © 2014 Mazen E. Iskandar et al. All rights reserved. Metastatic Basal Cell Carcinoma Accompanying Gorlin Syndrome Wed, 19 Nov 2014 13:34:15 +0000 Gorlin-Goltz syndrome or basal cell nevus syndrome is an autosomal dominant syndrome characterized by skeletal anomalies, numerous cysts observed in the jaw, and multiple basal cell carcinoma of the skin, which may be accompanied by falx cerebri calcification. Basal cell carcinoma is the most commonly skin tumor with slow clinical course and low metastatic potential. Its concomitance with Gorlin syndrome, resulting from a mutation in a tumor suppressor gene, may substantially change morbidity and mortality. A 66-year-old male patient with a history of recurrent basal cell carcinoma was presented with exophthalmus in the left eye and the lesions localized in the left lateral orbita and left zygomatic area. His physical examination revealed hearing loss, gapped teeth, highly arched palate, and frontal prominence. Left orbital mass, cystic masses at frontal and ethmoidal sinuses, and multiple pulmonary nodules were detected at CT scans. Basal cell carcinoma was diagnosed from biopsy of ethmoid sinus. Based on the clinical and typical radiological characteristics (falx cerebri calcification, bifid costa, and odontogenic cysts), the patient was diagnosed with metastatic skin basal cell carcinoma accompanied by Gorlin syndrome. Our case is a basal cell carcinoma with aggressive course accompanying a rarely seen syndrome. Yeliz Bilir, Erkan Gokce, Banu Ozturk, Faik Alev Deresoy, Ruken Yuksekkaya, and Emel Yaman Copyright © 2014 Yeliz Bilir et al. All rights reserved. Male Pelvic Squamous Cell Carcinoma of Unknown Primary Origin Thu, 13 Nov 2014 06:44:25 +0000 Pelvic squamous cell carcinoma of unknown primary origin has been described in several case reports of female patients. However, there have been no published reports describing male patients with pelvic squamous cell cancer of unknown primary origin. Our case describes a 52-year-old man who presented with right buttock pain, rectal urgency, and constipation. His physical examination demonstrated tenderness to palpation around his gluteal folds. Computed tomography scan of his abdomen and pelvis demonstrated a large mass in his retroperitoneum. The mass was determined to be squamous cell carcinoma of unknown primary origin. Additionally, the patient had small nodules in his right lower lung lobe and right hepatic lobe. The patient was treated with concomitant chemoradiation, including cisplatin and intensity-modulated radiation therapy, followed by carboplatin and paclitaxel. The patient achieved partial remission, in which he remained one year after his presentation. Our case is consistent with the literature which suggests that squamous cell carcinoma of unknown primary origin occurring outside of the head and neck region may have a more favorable prognosis than other carcinomas of unknown primary origin. Further studies are necessary to determine the most appropriate work-up, diagnosis, and optimal treatment strategies. Lauren Chiec, Sadhna Verma, Ady Kendler, and Nagla Abdel Karim Copyright © 2014 Lauren Chiec et al. All rights reserved. Recurrence of Pelvic Chondrosarcoma through Fascial Defect into Abdominal Cavity Wed, 12 Nov 2014 11:39:00 +0000 Our patient was a 76-year-old female who has been operated on 2 times in 8 years for pelvic chondrosarcoma. The patient came to our clinic with a large mass in left iliac region which extended into the paravertebral area. Physical examination and preoperative imagining studies revealed a mass at the left iliac area that infiltrated sciatic notch and extended from posterior iliac region towards the anterior side of iliac bone through the sciatic notch and an incisional hernia including descending colon. The mass was also penetrating the abdominal cavity through the hernia. Surgical intervention was planned. Since the tumor infiltrated the sciatic nerve, hemipelvectomy was indicated. Patient refused hemipelvectomy. Therefore, palliative debulking surgery was considered. We treated the case with marginal excision and abdominal wall reconstruction employing prolene and vicryl suture materials in order to prevent a postoperative visceral herniation and local invasion. At the latest follow-up appointment in 2 years, the patient still had no signs of tumor recurrence. This case showed us that an incisional hernia can serve as a pathway for the recurrence invasion of the chondrosarcoma. Kemal Gökkuş, Tolgay Akin, Ergin Sagtas, Murat Saylik, and Ahmet Turan Aydın Copyright © 2014 Kemal Gökkuş et al. All rights reserved. Thymic Carcinoma Presenting as Disseminated Intravascular Coagulation Wed, 12 Nov 2014 00:00:00 +0000 Thymomas and thymic carcinomas are rare tumors, which originate from the epithelial cells of the thymus. We present a case of thymic carcinoma, which presented with DIC as an initial manifestation. DIC improved with corticosteroid treatment and thymic carcinoma was amendable to chemoradiation. Mamatha Siricilla, Jawairia Memon, and Robert Avery Copyright © 2014 Mamatha Siricilla et al. All rights reserved. Transcatheter Arterial Chemoembolization and Chemotherapy plus Sorafenib in a Large Hepatocellular Carcinoma with Arterioportal Shunt Thu, 06 Nov 2014 09:40:35 +0000 Introduction. Arterioportal shunts (APS) are sometimes encountered in patients with hepatocellular carcinoma (HCC) and associated with poor prognosis. The management of HCC with APS is a challenge so far. Case Presentations. We report here in detail a 37-year-old man who was diagnosed as an advanced HCC accompanied with severe APS and treated by two sessions of transcatheter arterial chemoembolization (TACE) and three sessions of transcatheter arterial chemotherapy (TAC) plus sorafenib therapy. The tumor shrinks were revealed continuously during 152 days after the diagnosis. Although tumor progress emerged at 209 days after the diagnosis, the patient remarkably achieved 366-day survival. Discussion. TACE plus sorafenib may be a promising treatment for advanced HCC accompanied with APS. Prospective case-control studies should be advocated to evaluate the combination of TACE, TAC, and sorafenib in the management of HCC with APS. Jun Chen, Shixi Chen, Wei Xi, Bei Wu, Hui Yu, and Yang Gao Copyright © 2014 Jun Chen et al. All rights reserved. Acute Cardiogenic Shock Induced by Infusional 5-Fluorouracil Tue, 28 Oct 2014 12:19:05 +0000 A 49-year-old patient with metastatic carcinoma of the bladder and no prior history of heart disease presented with diffuse ST elevation, elevated troponins, and biventricular dysfunction requiring intensive care unit admission and inotropic support after receiving her first course of infusional 5-fluorouracil (5-FU). Over the course of several days, the patient’s cardiac function and clinical status returned to baseline. A follow-up echocardiogram performed 5 days after initial presentation revealed an ejection fraction of 59 percent, with no evidence of wall motion abnormalities. Subsequent 5-FU chemotherapy was discontinued, and the patient went on to receive second-line chemotherapy. Jeffrey Sulpher, Franco Dattilo, Susan Dent, Michele Turek, M. Neil Reaume, and Christopher Johnson Copyright © 2014 Jeffrey Sulpher et al. All rights reserved. Atypical Reversible Leucoencephalopathy Syndrome after Bevacizumab/Folfox Regimen for Metastatic Colon Cancer Tue, 21 Oct 2014 07:42:22 +0000 We are reporting a case of multifocal reversible leucoencephalopathy syndrome induced by chemotherapy based on Folfox-Bevacizumab regimen. A 44-year-old female, with no history of hypertension, received a chemotherapy based on Folfox-Bevacizumab for her metastatic colon cancer (5 FU: 325 mg/m2 d1 by intravenous infusion, Oxaliplatin 80 mg/m2 d1, and Bevacizumab: 7.5 mg/Kg d1). During the fourth cure, she presented delirium, seizures, and visual disturbances. The computed tomography (CT) of the brain showed hypodense lesions of the white matter of frontal, parietal, and occipital lobes, which were bilateral and symmetrical. The clinical table was reversible under symptomatic treatment. Narimane Salmi, Ibrahim Elghissassi, Khadija Bellahammou, Asmaa Lakhdissi, Hind Mrabti, and Hassan Errihani Copyright © 2014 Narimane Salmi et al. All rights reserved. A Krukenberg Tumor from an Occult Intramucosal Gastric Carcinoma Identified during an Autopsy Thu, 16 Oct 2014 08:12:13 +0000 A healthy 45-year-old Japanese female developed right pleural effusion, ascites, and a pelvic mass. Bilateral salpingo-oophorectomy resolved the pleural effusion and ascites. Histopathological examination of the ovaries showed bilateral Krukenberg tumors with signet-ring cell carcinoma (SRCC). Extensive testing including upper and lower gastrointestinal endoscopy and whole-body imaging did not detect the primary lesion. Six months after bilateral salpingo-oophorectomy, the patient developed multiple osteoblastic bone lesions in the spine, pelvis, and femurs. A biopsy of the bone marrow showed SRCC. We administered four cycles of S-1 and cisplatin, resulting in the shrinkage of osteoblastic lesions; she remained stable for a year. Then, she developed disseminated intravascular coagulation with disease progression in the bones. Although she was treated with paclitaxel, the disseminated intravascular coagulation progressed, and she died in a month. During the autopsy, microscopic examination revealed four foci of intramucosal gastric SRCC and healthy macroscopic gastric mucosa. Yoshiaki Nakamura, Ayako Hiramatsu, Takafumi Koyama, Yu Oyama, Ayuko Tanaka, and Koichi Honma Copyright © 2014 Yoshiaki Nakamura et al. All rights reserved. Metastatic Uterine Leiomyosarcoma in the Upper Buccal Gingiva Misdiagnosed as an Epulis Wed, 15 Oct 2014 08:52:07 +0000 Uterine leiomyosarcoma (LMS) is a rare tumor constituting 1% of all uterine malignancies. This sarcoma demonstrates an aggressive growth pattern with an high rate of recurrence with hematologic dissemination; the most common sites are lung, liver, and peritoneal cavity, head and neck district being rarely interested. Only other four cases of metastasis in the oral cavity have been previously described. The treatment of choice is surgery and the use of adjuvant chemotherapy and radiation has limited impact on clinical outcome. In case of metastases, surgical excision can be performed considering extent of disease, number and type of distant lesions, disease free interval from the initial diagnosis to the time of metastases, and expected life span. We illustrate a case of uterine LMS metastasis in the upper buccal gingiva that occurred during chemotherapy in a 63-year-old woman that underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy for a diagnosis of LMS staged as pT2bN0 and that developed lung metastases eight months after primary treatment. Surgical excision of the oral mass (previously misdiagnosed as epulis at a dental center) and contemporary reconstruction with pedicled temporalis muscle flap was performed in order to improve quality of life. Even if resection was achieved in free margins, “local” relapse was observed 5 months after surgery. Andrea Cassoni, Valentina Terenzi, Davina Bartoli, Oriana Rajabtork Zadeh, Andrea Battisti, Mario Pagnoni, Davide Conte, Alessandro Lembo, Sandro Bosco, Francesco Alesini, and Valentino Valentini Copyright © 2014 Andrea Cassoni et al. All rights reserved. Neoadjuvant Concurrent Chemoradiation for Curative Treatment of Penile Squamous Cell Carcinoma Tue, 07 Oct 2014 07:04:51 +0000 Introduction. Penile cancer is a rare malignancy often treated with neoadjuvant chemotherapy followed by surgery. However, the utility of neoadjuvant chemoradiation, particularly when the tumor is resistant to chemotherapy alone, has not been established. In this study, we report a case of pT3cN3M0 penile squamous cell carcinoma with progression of nodal disease on chemotherapy, which was cured with use of neoadjuvant concurrent chemoradiation. Case Report. A 65-year-old male presented with a fixed left inguinal lymph node with associated firmness of the penile glans. Biopsies of both sites revealed evidence of squamous cell carcinoma. The patient underwent partial penectomy for the primary lesion and began neoadjuvant chemotherapy to reduce the size of the unresectable left inguinal node. However, he displayed disease progression in the left inguinal node. As such, we attempted concurrent chemoradiation therapy with regression of his nodal disease. The patient was able to undergo left inguinal node dissection and has no evidence of disease 18 months since his initial surgery. Conclusion. The use of neoadjuvant chemoradiation for bulky cN2-3 disease seems appropriate in the setting of progressive disease. Further studies are necessary to assess the utility of concurrent chemoradiation both in the neoadjuvant and salvage setting. Arpit Chhabra, David Schwartz, Andrea Leaf, Nikolaos Karanikolas, Jeffrey P. Weiss, and David Schreiber Copyright © 2014 Arpit Chhabra et al. All rights reserved. Adult Neuroblastoma Complicated by Increased Intracranial Pressure: A Case Report and Review of the Literature Thu, 25 Sep 2014 07:47:31 +0000 Neuroblastoma is the third most commonly occurring malignancy of the pediatric population, although it is extremely rare in the adult population. In adults, neuroblastoma is often metastatic and portends an extremely poor overall survival. Our case report documents metastatic neuroblastoma occurring in a healthy 29-year-old woman whose course was complicated by an unusual presentation of elevated intracranial pressures. The patient was treated with systemic chemotherapy, I131 metaiodobenzylguanidine (MIBG) radiotherapy, and autologous stem cell transplant (SCT). Unfortunately the patient’s response to therapy was limited and she subsequently died. We aim to review neuroblastoma in the context of increased intracranial pressure and the limited data of neuroblastoma occurring in the adult population, along with proposed treatment options. Patrick L. Stevens, Douglas B. Johnson, Mary Ann Thompson, Vicki L. Keedy, Haydar A. Frangoul, and Kristen M. Snyder Copyright © 2014 Patrick L. Stevens et al. All rights reserved. Axillary Metaplastic Breast Carcinoma with Ipsilateral Pectoral Invasive Ductal Carcinoma: An Unusual Presentation Tue, 16 Sep 2014 12:33:55 +0000 We report a case of axillary metaplastic breast carcinoma (MBC) with triple negative (ER−/PR−/Her2−) phenotype, concurrent with multifocal invasive ductal carcinoma (IDC) of ipsilateral pectoral breast (ER+/PR+/Her2−) in a 60-year-old woman. The two tumors demonstrate different morphology, immunophenotype, and opposite response to neoadjuvant chemotherapy of paclitaxol, adriamycin, and cyclophosphamide. Methylation analysis of human androgen receptor (HUMARA) on X-chromosome identified monoclonal pattern of X-chromosome inactivation in MBC and mosaic pattern in the IDC. Stem cell origin of MBC is suggested in this case. Clinicopathological features, imaging findings, biological markers, chemoradiation management, and prognosis of MBC are reviewed in comparison to invasive ductal carcinoma. Our case and literature review suggest that traditional chemotherapy applicable to IDC is less effective towards MBC. However, new chemotherapy protocols targeting stem cell and multimodality management of MBC are promising. Recognition of unusual presentation of MBC will help tailor therapy towards tumor with worse prognosis. Lei Zhang, Sabahattin Comertpay, David Shimizu, Richard M. DeMay, Michele Carbone, Stacey A. Honda, and Jodi M. Matsuura Eaves Copyright © 2014 Lei Zhang et al. All rights reserved. Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1 Tue, 16 Sep 2014 10:51:13 +0000 Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present patients with MPNST treated in our department. Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old) arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them. Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear. Apostolos Pourtsidis, Dimitrios Doganis, Margarita Baka, Despina Bouhoutsou, Maria Varvoutsi, Maria Synodinou, Panagiota Giamarelou, and Helen Kosmidis Copyright © 2014 Apostolos Pourtsidis et al. All rights reserved. A PUBS Case in a Palliative Care Unit Experience Mon, 15 Sep 2014 05:46:59 +0000 Purple urine bag syndrome (PUBS) is a rare condition in which purple discoloration of the collecting bag and its associated tubing occurs. It is considered a benign condition. PUBS is usually associated with urinary tract infection occurring in elderly bedridden women, with chronic urinary catheterization. This syndrome is usually reported to occur in alkaline urine, but here we describe a rare case of PUBS involving acidic urine. M. R. Restuccia and M. Blasi Copyright © 2014 M. R. Restuccia and M. Blasi. All rights reserved. Bilateral Choroidal Metastasis from Non-Small Cell Lung Cancer Sun, 14 Sep 2014 09:38:43 +0000 Breast and lung cancers are the most common primary neoplasms to manifest with choroidal metastases. The incidence of choroidal metastases from metastatic lung cancer was reported to be 2–6.7%. We report a case of bilateral choroidal metastasis from non-small cell lung cancer. A 59-year-old Caucasian female patient, never a smoker, was diagnosed with stage IV lung adenocarcinoma metastatic to the pleura, bones, and the brain. Her initial scan of the chest showed innumerable soft tissue nodules and mediastinal adenopathy compatible with metastatic disease. Her initial brain MRI showed numerous small enhancing lesions consistent with extensive disease. Unfortunately, during her follow-up visits, she presented with bulge on her left eye. Simultaneously, her follow-up chest scan showed increase in the size of the lung nodules. She continued to have a reasonable performance status at that time, except for mild increase in her dyspnea. The choroidal metastases require a multidisciplinary care and should be among the differential patients with malignancy who present with ocular symptoms. Tariq Namad, Jiang Wang, Annemarie Tilton, and Nagla Abdel Karim Copyright © 2014 Tariq Namad et al. All rights reserved. Pathological Complete Response and Long-Term Survival in a Very Elderly Patient after Neoadjuvant Chemotherapy for Locally Advanced, Unresectable Gastric Cancer Sun, 14 Sep 2014 09:25:03 +0000 We address the pathological complete response and long-term survival of elderly patients after neoadjuvant chemotherapy in locally advanced, unresectable gastric cancer. An 83-year-old man was hospitalized for upper abdominal pain. Gastrointestinal endoscopy showed a large tumor spanning from the gastric angle to the antrum, and extending to the duodenum. Histological analysis of the biopsy specimen revealed a poorly differentiated adenocarcinoma. Computed tomography images showed thickening of the gastric wall and invasion of the body and head of the pancreas, but did not show distant metastases. The patient was diagnosed with unresectable gastric cancer, and was treated with neoadjuvant chemotherapy using S-1 (80 mg/m2) and paclitaxel (60 mg/m2). After the third course of chemotherapy, gastrointestinal endoscopy and abdominal computed tomography revealed a remarkable reduction in tumor size. This reduction allowed distal gastrectomy to be conducted. Histological examination of the specimen revealed no cancer cells in the primary lesion or lymph nodes. The patient was treated with adjuvant chemotherapy of oral tegafur-uracil (300 mg/day) for one year after surgery. He lived for five years after surgery without recurrence. Neoadjuvant chemotherapy using S-1 and paclitaxel is a potent strategy for improving survival in very elderly patients with unresectable gastric cancer. Kunihiko Izuishi, Mitsuyoshi Kobayashi, Takanori Sano, Hirohito Mori, and Kazuo Ebara Copyright © 2014 Kunihiko Izuishi et al. All rights reserved. High Dose Radiotherapy to Automated Implantable Cardioverter-Defibrillator: A Case Report and Review of the Literature Sun, 07 Sep 2014 12:28:44 +0000 We report a case of successful full-dose chemoradiotherapy to stage IIIB nonsmall cell lung cancer (NSCLC) in a 59-year-old man with extensive cardiac history and an automated implantable cardioverter-defibrillator (AICD) located within the radiotherapeutic field. In this case, the AICD was a St. Jude Medical Fortify Assura VR 1257-40Q ICD, and it was implanted prophylactically during bypass grafting. Although we do not recommend routine radiotherapy dose to exceed recommended current guidelines due to the potential risks to the patient, this is a situation where relocation of the device was not possible. Fortunately, our patient was not AICD-dependent; so following much discussion and deliberation, the decision was made to treat the patient with AICD in place. The patient completed definitive chemoradiotherapy with concurrent cisplatin and etoposide and thoracic irradiation to 69.6 Gy. The minimum, maximum, and mean doses to the AICD directly were 13.5 Gy, 52.4 Gy, and 29.3 Gy, respectively. The device withstood full thoracic radiation dose, and the patient denied cardiac symptoms during the time before, during, and after completion of therapy. We sought to offer this case for both teaching and guidance in practice and to contribute to the published literature currently available in this area. Inaya Ahmed, Wei Zou, and Salma K. Jabbour Copyright © 2014 Inaya Ahmed et al. All rights reserved. Coexistence of Chronic Lymphocytic Leukemia and Myeloproliferative Neoplasm Tue, 02 Sep 2014 12:27:39 +0000 Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in the Western world. Host immune surveillance caused mainly by the disease itself is speculated to be responsible for high incidence of secondary neoplasms. However, the simultaneous occurrence of CLL and myeloproliferative disorder in the same patient is extremely rare. In the present report, a case of an 81-year-old man who was diagnosed with chronic lymphocytic leukemia and concomitant essential thrombocythemia is presented. We describe the morphologic, immunophenotypic, cytogenetic, and molecular findings in this patient. We also review the current literature. Patrycja Zielinska, Miroslaw Markiewicz, Monika Dzierzak-Mietla, Anna Koclega, Grzegorz Helbig, and Slawomira Kyrcz-Krzemien Copyright © 2014 Patrycja Zielinska et al. All rights reserved. Diagnostic Dilemma of a Rare, Giant Retroperitoneal Schwannoma: A Case Report and Review of Literature Wed, 27 Aug 2014 11:59:08 +0000 Schwannoma is a benign tumour of peripheral nerve sheath. It usually arises from head, neck, and trunk. Retroperitoneal schwannoma is a rare entity, accounting for only 0.3–3% of total schwannomas. Majority of retroperitoneal schwannomas reported in literature have a diameter of 5 to 15 cm. Preoperative diagnosis is difficult due to low frequency, nonspecific clinical course, and nonspecific imaging features. Histology usually provides definitive diagnosis. Schwannomas are solitary, well-circumscribed, and noninvasive, so complete surgical excision provides good result. We report a case of a 23-year-old male, who presented with progressive abdominal distension and intermittent episodes of intestinal obstruction. CECT was suggestive of huge solid-cystic mass in abdominopelvic region. Image guided percutaneous aspiration revealed around 1 litre of frank pus and FNAC was suggestive of abscess. Exploratory laparotomy revealed a large 32 × 28 × 26 cm mass with solid and cystic components containing 1 litre of pus. Histological features of tumour were suggestive of benign schwannoma and immunohistochemistry for S-100 was positive. Postoperative recovery was uneventful. We report this case of a retroperitoneal schwannoma because of giant size, rare location, unusual presentation, and diagnostic dilemma. Mahendra Singh, Lovekesh Kumar, Rajkumar Chejara, Om Prakash Prasad, Yuvraj Kolhe, and Ashish Saxena Copyright © 2014 Mahendra Singh et al. All rights reserved. Waldenström Macroglobulinemia in Hepatitis C: Case Report and Review of the Current Literature Wed, 27 Aug 2014 07:54:26 +0000 Background. Recent literature has associated hepatitis C virus with the development of non-Hodgkin lymphoma. Hepatitis C virus infection appears to promote lymphoproliferation, providing a plausible mechanism for a causative association; however, despite prior reports of patients with comorbid hepatitis C infection and Waldenström macroglobulinemia, the literature is in disagreement regarding whether there exists an association between these two conditions. Case Presentation. This case report describes a 57-year-old African-American male with chronic hepatitis C infection and cryoglobulinemia who presented with several episodes of transient confusion and paralysis and was found to have symptomatic hyperviscosity. The recognition of his condition was facilitated by characteristic findings on ophthalmologic examination. He was subsequently diagnosed with Waldenström macroglobulinemia on bone marrow biopsy. Conclusions. An up to date, comprehensive review of the literature suggests an association between hepatitis C and Waldenström macroglobulinemia. Data on optimal treatment of patients with comorbid hepatitis C infection and Waldenström macroglobulinemia is limited. We have provided a comprehensive review of previously explored treatment options to guide management of other similar patients. Our patient has since been treated with repeated plasmapheresis with a plan to pursue antiviral therapy. Ryan Nipp, Aaron Mitchell, Allyson Pishko, and Ara Metjian Copyright © 2014 Ryan Nipp et al. All rights reserved. Tackling a Recurrent Pinealoblastoma Mon, 25 Aug 2014 08:00:41 +0000 Pineoblastomas are rare, malignant, pineal region lesions that account for <0.1% of all intracranial tumors and can metastasize along the neuroaxis. Pineoblastomas are more common in children than in adults and adults account for <10% of patients. The management of pinealoblastoma is multimodality approach, surgery followed with radiation and chemotherapy. In view of aggressive nature few centres use high dose chemotherapy with autologus stem cell transplant in newly diagnosed cases but in recurrent setting the literature is very sparse. The present case represents the management of pinealoblastoma in the recurrent setting with reirradiation and adjuvant carmustine chemotherapy wherein the management guidelines are not definitive. Siddanna Palled, Sruthi Kalavagunta, Jaipal Beerappa Gowda, Kavita Umesh, Mahalaxmi Aal, Tanvir pasha Chitraduraga Abdul Razack, Veerabhadre Gowda, and Lokesh Viswanath Copyright © 2014 Siddanna Palled et al. All rights reserved. Renal Medullary Carcinoma: Case Report of an Aggressive Malignancy with Near-Complete Response to Dose-Dense Methotrexate, Vinblastine, Doxorubicin, and Cisplatin Chemotherapy Tue, 19 Aug 2014 07:12:00 +0000 Renal medullary carcinoma (RMC) is a rare but aggressive malignancy affecting young individuals with sickle cell trait. Renal medullary carcinoma commonly presents with advanced or metastatic disease and is associated with a rapidly progressive clinical course and an extremely short overall survival measured in weeks to few months. Due to the rarity of RMC, there is no proven effective therapy and patients are often treated with platinum-based chemotherapy. We report near-complete radiological and pathological response in a patient treated with dose-dense MVAC (methotrexate, vinblastine, doxorubicin, and cisplatin) chemotherapy. The patient underwent consolidation nephrectomy and retroperitoneal lymph node dissection and had a 16-month progression-free survival, one of the longest reported in patients with RMC. Ali Imran Amjad, Hira Ali, Leonard J. Appleman, Jodi Maranchie, Stephen Jackman, Anil Parwani, Rajiv Dhir, Somak Roy, and Rahul A. Parikh Copyright © 2014 Ali Imran Amjad et al. All rights reserved.