Case Reports in Oncological Medicine http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. Primitive Neuroectodermal Tumor/Ewing Sarcoma Presenting with Pulmonary Nodular Lesions Thu, 29 Jan 2015 12:59:50 +0000 http://www.hindawi.com/journals/crionm/2015/957239/ Primitive neuroectodermal tumors (PNETs) and Ewing sarcoma (EWS) belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. EWS-PNETs that arise in the lung parenchyma involvement are extremely rare in adults. A case of a 32-year-old male presenting with chest pain and diffuse pulmonary nodules on chest X-ray and diagnosed with Ewing sarcoma-PNETs will be presented here. Selvi Asker, Fuat Sayir, Gulay Bulut, Aysel Sunnetcioglu, Selami Ekin, and Alpaslan Yavuz Copyright © 2015 Selvi Asker et al. All rights reserved. Rhabdomyosarcomatous Transformation of a Gastrointestinal Stromal Tumor following Treatment with Imatinib Wed, 28 Jan 2015 07:49:22 +0000 http://www.hindawi.com/journals/crionm/2015/317493/ Rhabdomyosarcomatous dedifferentiation of GIST following tyrosine kinase inhibitor (TKI) therapy is rare, with only a handful of cases previously reported in the literature. Herein we present a case of metastatic GIST initially treated with imatinib that developed radiographic evidence of progression after 8 months of standard dose therapy with continued progression despite attempts at using dose-escalated imatinib 400 mg bid. Due to the patient’s worsening clinical symptoms and radiographic concerns for colonic thickening, abscess, and extraluminal air, the patient underwent a palliative resection of a large heterogeneous mass arising from the posterior stomach and several metastatic foci. Pathology revealed a dedifferentiated GIST with rhabdomyosarcomatous features. This report will highlight the unique features of this case and review the existing literature. Xiaoyin Jiang, H. Bryan Anderson, Cynthia D. Guy, Paul J. Mosca, Richard F. Riedel, and Diana M. Cardona Copyright © 2015 Xiaoyin Jiang et al. All rights reserved. Metastatic Extramammary Paget’s Disease of Scrotum Responds Completely to Single Agent Trastuzumab in a Hemodialysis Patient: Case Report, Molecular Profiling and Brief Review of the Literature Tue, 27 Jan 2015 12:51:30 +0000 http://www.hindawi.com/journals/crionm/2015/895151/ Extramammary Paget’s disease (EMPD) is a rare cancer. Although EMPD is usually noninvasive and treated with local therapy, once metastatic the prognosis of EMPD is poor and treatment options are limited. We report a case of a complete response to single agent trastuzumab in a hemodialysis patient with metastatic Her2/neu overexpressed EMPD of the scrotum. Molecular profiling of his case as well as 12 other EMPD and 8 mammary Paget disease (MPD) cases was completed and revealed multiple biomarker aberrations. Overexpression of Her2 was frequently noted (30%–40%) in both EMPD and MPD patients and when present can be effectively treated with Her2 targeted agents. Trastuzumab therapy can be safely utilized in a hemodialysis patient. In addition, multiple protein overexpression and loss were seen in EMPD including PD-1, PD-L1, PTEN, and AR as well as PIK3CA mutation. These findings may lead to possible therapeutic interventions targeting these pathways in a disease with few effective treatment options. Peter Barth, Essel Dulaimi Al-Saleem, Kristen W. Edwards, Sherri Z. Millis, Yu-Ning Wong, and Daniel M. Geynisman Copyright © 2015 Peter Barth et al. All rights reserved. Cryptococcus laurentii Diarrhea in a Neoplastic Patient Tue, 27 Jan 2015 11:33:55 +0000 http://www.hindawi.com/journals/crionm/2015/216458/ We present a rare case of diarrhea and neutropenia caused by Cryptococcus laurentii (C. laurentii) infection in old patient with metastatic rectal cancer who underwent FOLFOX plus Cetuximab chemotherapy. C. laurentii is an extremely rare human pathogen. To the best of our knowledge, here, we report the first case of diarrhea and neutropenia caused by C. laurentii in a 74-year-old man with metastatic rectal cancer and hepatic metastases who underwent FOLFOX plus Cetuximab chemotherapy. Franco Calista, Federica Tomei, Pasquale Assalone, Divina Traficante, Gianni Di Pilla, Carla Pepe, and Liberato Di Lullo Copyright © 2015 Franco Calista et al. All rights reserved. Primary Angiosarcoma of Urinary Bladder: 13th Reported Patient Mon, 26 Jan 2015 09:20:11 +0000 http://www.hindawi.com/journals/crionm/2015/652870/ Angiosarcoma of the urinary bladder is an extremely rare and poorly characterized tumor. We are presenting the 13th reported patient who was an 89-year-old man initially presented with massive hematuria. His past medical history included external-beam radiation for prostate cancer 12 years ago. His PSA was 0.26 ng/dL. His CT-Urography demonstrated a highly vascular mass originating from the bladder base. The mass was partially resected, transurethrally. The pathology was consistent with primary angiosarcoma of the urinary bladder. Bone scan and CT-U showed metastasis to spine. The patient was treated with palliative radiotherapy for back pain due to metastasis, and he refused chemotherapy. The patient died 3 months after his initial diagnosis. Zaher Bahouth, Ismael Masarwa, Sarel Halachmi, and Ofer Nativ Copyright © 2015 Zaher Bahouth et al. All rights reserved. Hemangiopericytoma of the Breast: A Case Report and a Review of the Literature Thu, 22 Jan 2015 14:14:53 +0000 http://www.hindawi.com/journals/crionm/2015/210643/ Introduction. Sarcomas of the breast are rare and hemangiopericytoma (HPC) of the breast is even rarer. Case Report. We report a case of a 43-year-old woman who presented with a 4 cm mass in her right breast. Her family history was positive for breast cancer. A fine needle aspiration indicated a malignant vascular tumor. An excision biopsy and frozen section analysis confirmed the presence of an encapsulated mesenchymal tumor. Its morphology and immunohistochemical marker profile were characteristic for a malignant hemangiopericytoma. Thus, she underwent a tumor excision without an axilla sampling. Approximately one year after the surgery the patient is well without local recurrence or metastasis disease to be observed. We also reviewed the literature and discuss the treatment options, characteristics, and immunophenotype of HPC. Conclusions. The accurate diagnosis of HPC depends on the appropriate histological and immunohistochemical examination. Surgical resection is the treatment of choice and due to scarcity of cases and unpredictable biological behavior of these tumors long term follow-up may be warranted. Georgios Koukourakis, Evagelos Filopoulos, Kasiani Kapatou, and Georgios Zacharias Copyright © 2015 Georgios Koukourakis et al. All rights reserved. Aggressive Extraocular Sebaceous Carcinoma Recurring after Mohs Micrographic Surgery Thu, 22 Jan 2015 06:39:38 +0000 http://www.hindawi.com/journals/crionm/2015/534176/ Sebaceous carcinomas (SC) are rare adnexal tumors with possible aggressive behavior usually arising in the head and neck region of adults in the seventh decade of life. Treatment has traditionally been with surgical excision with 5-6 mm wide margins but Mohs micrographic surgery (MMS) has also been reported as an effective treatment modality. We present a case of a Caucasian female renal transplant patient with a rapidly enlarging nodule on the left preauricular cheek that was excised with MMS with negative margins. The tumor recurred rapidly and metastasized ultimately leading to the death of the patient. There was some disagreement amongst pathologists as to the possible nature of the diagnosis with the original biopsy being labeled as a poorly differentiated carcinoma. We aim to highlight the potential aggressive nature of SC and review the features of the neoplasm including histological features that help in making the diagnosis. Konstantin V. Grigoryan, Laurel Leithauser, and Hugh M. Gloster Jr. Copyright © 2015 Konstantin V. Grigoryan et al. All rights reserved. Peripheral T Cell Non-Hodgkin’s Lymphoma following Treatment of Hodgkin’s Lymphoma Tue, 13 Jan 2015 11:18:36 +0000 http://www.hindawi.com/journals/crionm/2015/438385/ Previous reports have suggested that non-Hodgkin’s lymphoma (NHL) is more likely to develop in patients with Hodgkin lymphoma (HL) compared to the general population. These two can occur synchronously or metachronously. We report here on a case of nodular sclerosis classical HL and T cell NHL that occurred in a patient metachronously. Peripheral T cell lymphoma (PTCL) of the patient was found about 2 years after treatment of classical HL. When the patient was diagnosed with HL, biopsy revealed typical RS cells, presenting positive for CD30 and CD15 and negative for CD79a and CD3 in immunohistochemistry. And PCR analysis showed IgH gene rearrangement; however, T cell receptor gene rearrangement and Epstein-Barr virus (EBV) were not detected on PCR analysis. After 2 years of treatment of HL, colonoscopic biopsy and lymph node biopsy showed CD3 positive atypical cells intermixed with small reactive lymphoid cells and plasma cells, indicating T cell lymphoma. PCR analysis demonstrated T cell receptor gene rearrangement and did not detect EBV. Although it is rare, synchronous or metachronous HL and NHL may occur. Therefore, we may need to ensure pathological confirmation, especially in case of lymphoma that did not respond to chemotherapy. Sun Hee Chang and Hye Ran Lee Copyright © 2015 Sun Hee Chang and Hye Ran Lee. All rights reserved. Pancreatic Perivascular Epithelioid Cell Tumour Presenting with Upper Gastrointestinal Bleeding Tue, 06 Jan 2015 09:24:07 +0000 http://www.hindawi.com/journals/crionm/2015/431215/ PEComa is a family of rare mesenchymal tumours which can occur in any part of the human body. Primary PEComas of the pancreas are extremely rare tumours with uncertain malignant potential. A 17-year-old female was admitted to the hospital due to melena. She required several transfusions. CT scan demonstrated a mass at the head of the pancreas measuring 4.2 cm in maximum diameter. An endoscopic ultrasound showed an ulcerating malignant looking mass infiltrating 50% of the wall of the second part of the duodenum in the region of the ampulla. Multiple biopsies taken showed extensive ulceration with granulation tissue formation and underlying large macrophages without being able to establish a definite diagnosis. We proceeded with pylorus-preserving pancreaticoduodenectomy. The postoperative course of the patient was unremarkable, and she was discharged on the 8th postoperative day. Histology examination of the specimen showed a PEComa of pancreas. Eighteen months after resection the patient is disease free. To the best of our knowledge this is the first time we describe a case of a pancreatic PEComa presenting with massive gastrointestinal bleeding. Christos Petrides, Kyriakos Neofytou, and Aamir Z. Khan Copyright © 2015 Christos Petrides et al. All rights reserved. Angiosarcoma Arising in a Patient with a 10-Year-Old Hemangioma Wed, 31 Dec 2014 09:12:36 +0000 http://www.hindawi.com/journals/crionm/2014/185323/ The transformation of a benign hemangioma into a malignant angiosarcoma has been rarely reported, with only 11 cases reported in the literature. There have been no reports of malignant transformation of hemangioma into angiosarcoma in association with epithelioid hemangioendothelioma, to our knowledge. The existence of precursor malignancies in the tumorigenesis of sarcomas is still not clearly defined. We describe the case of a 40-year-old woman with a preceding history of a suspected hemangioma for ten years, who upon resection was found on histology to have evidence of a hemangioma with an associated area of epithelioid hemangioendothelioma as well as areas of overt high grade epithelioid angiosarcoma. These findings raise the possibility of the evolution of hemangioma to epithelioid hemangioendothelioma, and the latter to overt angiosarcoma. The patient was managed as having a high grade sarcoma with wide resection and radiation. She declined systemic adjuvant chemotherapy after a thorough discussion about the risks and benefits of chemotherapy, and she currently remains disease free one year after the surgery. Michael J. Nathenson, Diana Molavi, and Albert Aboulafia Copyright © 2014 Michael J. Nathenson et al. All rights reserved. A Case of Gingival Candidiasis with Bone Destruction on Gastric Cancer Patient Receiving Cytotoxic Chemotherapy Mon, 29 Dec 2014 06:44:34 +0000 http://www.hindawi.com/journals/crionm/2014/145394/ We herein report a case of gingival candidiasis in an advanced gastric cancer patient while receiving palliative cytotoxic chemotherapy. A 46-year-old male patient admitted to our hospital for known advanced gastric cancer with newly developed multiple liver metastases. While receiving 2nd line cytotoxic chemotherapy with 5FU, leucovorin, and paclitxel, he complained of gingival swelling accompanied by pain and whitish plaque. Due to lack of response to the conservative oral care, incisional biopsy of gingiva was done and the pathology confirmed gingival candidiasis. Although the lesion healed apparently after two-week antifungal therapy, pain as well as bony destruction remains. By presenting this case report, we intend to emphasize the immunocompromising effect of cancer while being on systemic chemotherapy. Seungtaek Lim, Tae-jun Kil, Hye Ryun Kim, Seonhui Han, and Sun Young Rha Copyright © 2014 Seungtaek Lim et al. All rights reserved. Metastatic Cerebellar Gastrointestinal Stromal Tumor with Obstructive Hydrocephalus Arising from the Small Intestine: A Case Report and Review of the Literature Sun, 28 Dec 2014 08:06:44 +0000 http://www.hindawi.com/journals/crionm/2014/343178/ Gastrointestinal stromal tumor (GIST) is defined as a c-kit-positive gastrointestinal, mesenteric, or omental mesenchymal tumor that very rarely metastasizes to the brain. Metastasis to the cerebellum is particularly rare. An 80-year-old man presented with nausea and vomiting with disturbance of consciousness. Magnetic resonance imaging (MRI) revealed tumor in the cerebellar vermis causing obstructive hydrocephalus. The patient subsequently underwent midline suboccipital craniotomy, and the tumor was totally removed. Immunohistochemical analysis showed tumor cells positive for c-kit and CD34, and cerebellar metastasis of GIST was diagnosed. Postoperative radiotherapy was administered. Following surgery and radiotherapy, the patient developed ileus caused by tumor in the small intestine and underwent laparotomy for tumor removal. Following abdominal surgery, left hemiparesis and consciousness disturbance were noted. Computed tomography showed recurrent large tumor with perifocal edema in the right frontal lobe of the brain. The patient died 3 months after initial craniotomy. Intracranial metastasis of GIST is extremely rare. In cases such as the present, where the condition of the patient rapidly deteriorates and features such as rising intracranial pressure and ileus prevent the use of oral agents, molecular-targeted agents administered by intravenous infusion should be utilized. Kunitomo Sato, Toshihide Tanaka, Naoki Kato, Takuya Ishii, Toru Terao, and Yuichi Murayama Copyright © 2014 Kunitomo Sato et al. All rights reserved. Adult Alveolar Soft Part Sarcoma of the Head and Neck: A Report of Two Cases and Literature Review Tue, 23 Dec 2014 10:38:37 +0000 http://www.hindawi.com/journals/crionm/2014/597291/ Background. Alveolar soft part sarcomas (ASPS) of the head and neck are rare, aggressive soft-tissue malignancies. This study describes the clinical course and management of two patients presenting with ASPS in very rare head and neck locations, the larynx and parotid gland. Methods. We identified two patients presenting with ASPS of the head and neck and treated at the University of North Carolina. We compared our results to the literature from 1987 to 2013. Results. Patient ages at diagnosis were 27 and 39 with presenting symptoms of hoarseness and parotid swelling, respectively. Mean follow-up was 87 months. All patients received surgical resection and adjuvant radiotherapy. There were no recurrences or evidence of distant metastatic spread during the series. Disease-free survival time for the patients was 4 months and 168 months, respectively. Conclusions. Our study suggests that a combined-modality approach is important in the treatment of ASPS of the head and neck even in these rare locations. Continued research into new therapies is necessary to improve historically poor outcomes. Brandon T. Mullins and Trevor Hackman Copyright © 2014 Brandon T. Mullins and Trevor Hackman. All rights reserved. Familial Lymphoproliferative Malignancies and Tandem Duplication of NF1 Gene Sun, 14 Dec 2014 09:11:15 +0000 http://www.hindawi.com/journals/crionm/2014/685857/ 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 http://www.hindawi.com/journals/crionm/2014/424568/ 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 http://www.hindawi.com/journals/crionm/2014/345128/ 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 http://www.hindawi.com/journals/crionm/2014/657876/ 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 http://www.hindawi.com/journals/crionm/2014/361748/ 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 http://www.hindawi.com/journals/crionm/2014/956856/ 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 http://www.hindawi.com/journals/crionm/2014/962526/ 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 http://www.hindawi.com/journals/crionm/2014/737183/ 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 http://www.hindawi.com/journals/crionm/2014/362932/ 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 http://www.hindawi.com/journals/crionm/2014/953698/ 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 http://www.hindawi.com/journals/crionm/2014/674369/ 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 http://www.hindawi.com/journals/crionm/2014/202943/ 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 http://www.hindawi.com/journals/crionm/2014/392403/ 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 http://www.hindawi.com/journals/crionm/2014/819396/ 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 http://www.hindawi.com/journals/crionm/2014/391256/ 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 http://www.hindawi.com/journals/crionm/2014/797429/ 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 http://www.hindawi.com/journals/crionm/2014/402342/ 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.