Case Reports in Oncological Medicine The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. Primary Borderline Mucinous Tumors of the Testis: A Case Report and Literature Review Mon, 02 Mar 2015 11:20:09 +0000 Ovarian-type epithelial tumors of the testes and paratestes are very rare. Mucinous subtypes of such tumors are extremely rare; only 25 cases have been reported to date. Ovarian-type epithelial tumors are histologically classified into cystadenomas, borderline tumors, and carcinomas. We herein report a case involving a 60-year-old man with a primary borderline mucinous tumor of the testis. He underwent orchiectomy and has developed no recurrence for 4 years. This is the 26th report of a mucinous tumor of the testis in the literature. We also herein review the literature and discuss the etiology, prognosis, and treatment of mucinous tumors of the testes. Satoshi Funada, Toru Yoshida, Masaaki Ito, Fumihiko Kono, and Takehiko Segawa Copyright © 2015 Satoshi Funada et al. All rights reserved. Rare Occurrence of Lip Spindle Cell Lipoma Sun, 01 Mar 2015 06:54:34 +0000 Spindle cell lipoma (SCL) is a rare distinct variant of lipoma, which presents as a painless, circumscribed, slow-growing, superficial lesion on the lip and can mimic a minor salivary gland tumour. We present a slow growing lower lip lesion and its management. Case Report. A 38-year-old female gave an eight-year history of a slow-growing mass on her lower lip with intermittent change in size. She presented with a submucosal nodule and thin overlying mucosa adjacent to the vermilion border. Surgical excision was carried as the diagnostic and therapeutic approach. Conclusion. Lip SCL is rare, and surgical excision is advocated in order to exclude underlying pathology and minor salivary gland tumours. Sandra Girgis and Leo Cheng Copyright © 2015 Sandra Girgis and Leo Cheng. All rights reserved. Metastatic Extrapulmonary Small Cell Carcinoma to the Cerebellopontine Angle: A Case Report and Review of the Literature Wed, 25 Feb 2015 10:11:25 +0000 Extrapulmonary small cell carcinomas (EPSCC) are rare malignancies with poor patient prognoses. We present the case of a 63-year-old male who underwent surgical resection of a poorly differentiated small cell carcinoma, likely from a small intestinal primary tumor that metastasized to the cerebellopontine angle (CPA). A 63-year-old male presented with mild left facial paralysis, hearing loss, and balance instability. MRI revealed a 15 mm mass in the left CPA involving the internal auditory canal consistent with a vestibular schwannoma. Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm. The patient underwent a suboccipital craniectomy and the mass was grossly different visually and in consistency from a standard vestibular schwannoma. The final pathology revealed a poorly differentiated small cell carcinoma. Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease. The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms. Surgical resection and radiotherapy are potential treatment options to improve survival in patients diagnosed with NET brain metastases. We present the first documented case of skull base metastasis of a poorly differentiated small cell carcinoma involving the CPA. Debebe Theodros, C. Rory Goodwin, Genevieve M. Crane, Jason Liauw, Lawrence Kleinberg, and Michael Lim Copyright © 2015 Debebe Theodros et al. All rights reserved. Superior Vena Cava Syndrome and Colon Carcinoma: A Report of a Multifactorial Association Wed, 25 Feb 2015 06:34:12 +0000 Introduction. Superior vena cava (SVC) syndrome results from the obstruction of blood flow through the SVC, having distinct pathophysiological underlying mechanisms. Cancer is associated with an increased risk of thromboembolism that varies according to patient-, tumor-, and treatment-related factors. An individualized clinical approach is important to pursue the accurate diagnosis of the underlying pathology causing thromboembolism in cancer patients. Case Presentation. The authors present a case of a 58-year-old male with an infrequent presentation of an unknown colon carcinoma, who has never had any symptom until he was hospitalized with the diagnosis of superior vena cava syndrome and pulmonary thromboembolism. The patient had an advanced disease by the time of diagnosis and molecular alterations contributing to abnormal hemostasis. He presented venous and arterial thromboembolism and developed disseminated intravascular coagulopathy after surgery, anticoagulant and transfusion therapy, dying 40 days after the hospitalization. Conclusion. The authors discuss thromboembolic disease and tumor metastasis roles in a cancer patient with SVC syndrome. Thromboembolism in a malignancy context is a challenging clinical entity. A multifactorial perspective of the thrombotic disease is warranted to approach thromboembolism risk and stratify patients suitable to receive adequate anticoagulant prophylaxis and targeted therapies, aiming to improve clinical prognosis. Joana Espírito Santo, Inês Coutinho, Ana Pimentel, Rui Garcia, and Rui Marques dos Santos Copyright © 2015 Joana Espírito Santo et al. All rights reserved. New Primary Malignancy Masquerading as Metastatic Prostate Adenocarcinoma Thu, 19 Feb 2015 09:57:16 +0000 In the management of patients with prostate cancer, the development of new radiographic findings can mimic progression of the disease, thereby triggering changes in treatment. Typically, clinicians evaluate additional parameters, such as symptoms and prostate specific antigen (PSA) levels, for further evidence of disease progression. In the absence of additional findings, for example, elevated PSA, the possibility of an additional malignancy should be considered and evaluated. We present three cases of patients undergoing treatment for prostate adenocarcinoma and discovered on imaging to have findings suggestive of disease progression, but ultimately found to be a new primary malignancy. Our cases suggest that, in patients with prostate cancer, the appearance of new lymphadenopathy or bone lesions cannot be assumed to solely represent progression of the prostate cancer and warrant further investigation, especially in the presence of stable PSA levels. Ellen A. Szwed, Sarunas Sliesoraitis, Thu-Cuc Nguyen, Minh-Nguyet Nguyen, Jan S. Moreb, Robert A. Zlotecki, Paul L. Crispen, Nam H. Dang, and Long H. Dang Copyright © 2015 Ellen A. Szwed et al. All rights reserved. Nonpigmented Metastatic Melanoma in a Two-Year-Old Girl: A Serious Diagnostic Dilemma Wed, 11 Feb 2015 14:15:23 +0000 Although rare, malignant melanoma may occur in children. Childhood melanomas account for only 0.3–3% of all melanomas. In particular the presence of congenital melanocytic nevi is associated with an increased risk of development of melanoma. We herein report a case of malignant melanoma that developed on a giant congenital melanocytic nevus and made a metastasis to the subcutaneous tissue of neck in a two-year-old girl. The patient was hospitalized for differential diagnosis and treatment of cervical mass with a suspicion of hematological malignancy, because the malignant transformation of congenital nevus was not noticed before. In this case, we found out a nonpigmented malignant tumor of pleomorphic cells after the microscopic examination of subcutaneous lesion. Nonpigmented metastatic melanoma was diagnosed by several immunohistochemical and flow cytometric studies. She was offered palliative chemotherapy; however, her parents did not accept treatment. The patient died within 9 months of diagnosis. We emphasized here that the possibility of malignant melanoma in the differential diagnosis of childhood tumors should be kept in mind. Gulden Diniz, Hulya Tosun Yildirim, Selcen Yamaci, and Nur Olgun Copyright © 2015 Gulden Diniz et al. All rights reserved. Maxillary Sinus Inflammatory Myofibroblastic Tumors: A Review and Case Report Wed, 11 Feb 2015 09:44:24 +0000 An inflammatory myofibroblastic tumor (IMT) is an immunohistochemically diverse entity demonstrating neoplastic and nonneoplastic qualities. Although IMTs can arise in any area of the body, lesions arising in certain sites, namely, the nasal cavity, paranasal sinuses, and pterygopalatine fossa, demonstrate a heightened neoplastic and invasive potential. Despite case specific complete tumor regression and disease remission in response to pharmacotherapeutics, a subset of IMTs remain resistant to all forms of therapy. We present such a case, a 34-year-old female patient, with a highly resistant, maxillary sinus IMT. Her refractory, ALK-1 negative IMT has not responded well to novel therapies reported in current literature. This case suggests the role of zonal expressivity within a single lesion as a probable mechanism for its highly resistant nature and should promote determination of each IMT’s cytogenetic profile to provide more effective targeted therapy. Paper includes a literature review of all maxillary sinus IMTs from 1985 to 2014 along with their immunohistochemical staining, treatments, and outcomes. Chase C. Hansen, Colby Eisenbach, Carlos Torres, Suzanne Graham, and Fred Hardwicke Copyright © 2015 Chase C. Hansen et al. All rights reserved. Alternative Therapy for Epstein-Barr Virus Related Hemophagocytic Lymphohistiocytosis Sun, 08 Feb 2015 14:02:00 +0000 Hemophagocytic lymphohistiocytosis (HLH) is a rapidly fatal condition characterized by excessive immune activation. HLH can occur as a familial or sporadic acquired disorder. Acquired HLH is more frequently found in adults and is commonly secondary to infections, malignancies, or autoimmune diseases. Diagnosing HLH is challenging because of the rare occurrence, variable presentation, and nonspecific findings of this disorder. Diagnosis of HLH can be based on the diagnostic criteria which were used in the HLH-2004 trial. Given the rarity of this disease, protocols for its treatment have developed slowly, and obtaining adequate short-term and long-term control of the disease continues to be a challenge. Conventional induction therapy for HLH is dexamethasone and etoposide (VP-16), followed by or with cyclosporine. Intrathecal methotrexate ± hydrocortisone is given to those with central nervous system disease. We are reporting a patient who was diagnosed with Epstein-Barr virus (EBV) related HLH. He achieved complete remission with rituximab alone. To our knowledge, this is the first case of an adult patient with EBV related HLH who went into remission with rituximab therapy alone, without using the conventional chemotherapy. Omar Al Asad, Amir Salam, Siva Mannem, Mary Ninan, Avi Markowitz, and Bagi Jana Copyright © 2015 Omar Al Asad et al. All rights reserved. A Case of Calcified Metastatic Colorectal Adenocarcinoma Mimicking a Benign Lesion: Pitfalls in Diagnosis Thu, 05 Feb 2015 08:00:29 +0000 The radiological finding of a calcified intracranial lesion commonly represents a slow growing benign mass. Brain metastases originating from colorectal cancers are rare, occurring in approximately 2-3% of patients. Therefore the presence of a calcified brain lesion in a patient with a positive oncological history requires a high index of suspicion for brain metastases. Presented herein is a case of a frontoparietal calcified lesion initially overlooked as a benign tumour. Subsequent imaging following a neurological episode revealed a significant increase in size of the lesion with surrounding tissue oedema, prompting further investigation for suspicion of a calcified metastatic colorectal adenocarcinoma. Peter Michail, Iftah Amith, Sanila George, and Mathew K. George Copyright © 2015 Peter Michail et al. All rights reserved. A Suspicious Pancreatic Mass in Chronic Pancreatitis: Pancreatic Actinomycosis Tue, 03 Feb 2015 09:47:55 +0000 Introduction. Pancreatic actinomycosis is a chronic infection of the pancreas caused by the suppurative Gram-positive bacterium Actinomyces. It has mostly been described in patients following repeated main pancreatic duct stenting in the context of chronic pancreatitis or following pancreatic surgery. This type of pancreatitis is often erroneously interpreted as pancreatic malignancy due to the specific invasive characteristics of Actinomyces. Case. A 64-year-old male with a history of chronic pancreatitis and repeated main pancreatic duct stenting presented with weight loss, fever, night sweats, and abdominal pain. CT imaging revealed a mass in the pancreatic tail, invading the surrounding tissue and resulting in splenic vein thrombosis. Resectable pancreatic cancer was suspected, and pancreatic tail resection was performed. Postoperative findings revealed pancreatic actinomycosis instead of neoplasia. Conclusion. Pancreatic actinomycosis is a rare type of infectious pancreatitis that should be included in the differential diagnosis when a pancreatic mass is discovered in a patient with chronic pancreatitis and prior main pancreatic duct stenting. Our case emphasizes the importance of pursuing a histomorphological confirmation. F. de Clerck, P. Laukens, V. De Wilde, L. Vandeputte, M. Cabooter, J. Van Huysse, and H. Orlent Copyright © 2015 F. de Clerck et al. All rights reserved. A Peculiar Case of the Abscopal Effect: Radioactive Iodine Therapy Incidentally Palliating Marginal Zone Lymphoma Mon, 02 Feb 2015 10:42:26 +0000 The abscopal effect is an extremely rare phenomenon occurring when irradiation or treatment of a primary tumor burden not only results in debulking of the targeted site but also reduces tumor size at distant sites from the intended treatment area. We present the abscopal effect occurring in a patient with low-grade marginal zone lymphoma who subsequently received radioactive iodine therapy for papillary thyroid carcinoma. She was 67 years old when a routine complete blood count at her primary care physician’s office yielded a persistent leukocytosis of 14,500/μL with lymphocytosis of 9,870/μL. Immunophenotyping and fluorescence in situ hybridization (FISH) analysis confirmed low-grade marginal zone lymphoma. Over eight years, her peak leukocyte and lymphocyte counts were 24,100/μL and 18,100/μL, respectively. Subsequently, she was diagnosed with papillary thyroid carcinoma after presenting with a new complaint of dysphagia. A total thyroidectomy was performed, followed by 172.1 millicuries of oral I-131 sodium iodine radioactive ablation therapy. Following treatment, her leukocyte and lymphocyte counts were 3,100/μL and 1,100/μL, respectively. Over the next four years, her leukocyte and lymphocyte counts remained within normal limits and she remained symptom free. To our knowledge, there has never been a published report describing the use of radioactive iodine causing abscopal effect benefits for patients with underlying lymphoproliferative diseases. Robert C. Kornas, Sarah-Kim Shields, and Lyle S. Goldman Copyright © 2015 Robert C. Kornas et al. All rights reserved. Metastatic, Her-2 Amplified Lacrimal Gland Carcinoma with Response to Lapatinib Treatment Mon, 02 Feb 2015 09:03:01 +0000 Carcinoma of the lacrimal gland is a rare malignancy, limiting opportunities to develop new therapeutic regimens through clinical trials. There are no standard guidelines on optimal treatment of lacrimal gland carcinoma. In addition, lacrimal gland carcinoma includes several different subtypes with distinct behavior and response to treatment, further complicating treatment. Overexpression of the Her-2/neu protein, a potential target for new therapeutic agents, has previously been described in lacrimal gland carcinoma; however, there are no published reports regarding treatment of lacrimal gland cancer with Her-2 directed medications. This case report describes treatment of a patient with metastatic lacrimal gland carcinoma with lapatinib, an oral agent with activity against Her-2/neu amplified malignancies. In this case, Her-2 overexpression was confirmed by biopsy of a metastatic site. PET imaging obtained 6 months after the initiation of lapatinib showed evidence of a partial response to treatment, although the patient later developed progressive neurologic complications related to her malignancy and ultimately died. Lapatinib and other Her-2 targeted agents may represent an effective therapeutic option for this rare malignancy, in cases of metastatic disease. Trevor Dennie Copyright © 2015 Trevor Dennie. All rights reserved. Primitive Neuroectodermal Tumor/Ewing Sarcoma Presenting with Pulmonary Nodular Lesions Thu, 29 Jan 2015 12:59:50 +0000 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 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 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, Kristin 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 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 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 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 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 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 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 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 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 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 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 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.