Case Reports in Oncological Medicine The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. Phyllodes Tumor of the Breast Metastasizing to the Vulva Wed, 15 Apr 2015 12:54:08 +0000 Phyllodes tumors of the breast are rare breast tumors that resemble fibroadenoma. They are composed of two types of tissues: stromal and glandular tissues. Unlike fibroadenoma, they are commonly found in the third decade of life and they tend to grow more rapidly. Depending on the relative components of the cells and mitotic activity, they are classified into benign, borderline, and malignant. They are usually present as a lump in the breast. Phyllodes tumors are usually managed by wide excision. The excision should be wide enough to ensure a tumor-free margin. Recurrence rate is very high and most recurrences are usually local. Metastasis to the vulva has not been reported. Olusegun Kayode Ajenifuja, Nonna Kolomeyevskaya, Fadi Habib, Adekunle Odunsi, and Shashikant Lele Copyright © 2015 Olusegun Kayode Ajenifuja et al. All rights reserved. Diagnostic Challenges in Primary Hepatocellular Carcinoma: Case Reports and Review of the Literature Wed, 01 Apr 2015 07:31:48 +0000 Hepatocellular carcinoma is the fifth most common malignancy and the third leading mortality cause worldwide. It typically develops secondarily to liver cirrhosis, due to hepatitis B or C infection, alcohol abuse, metabolic disease, and so forth. According to the American Association for the Study of Liver Diseases (AASLD) guidelines, which constitute diagnostic standards, the diagnosis of primary hepatocellular carcinoma (HCC) should be based on contrast-enhanced imaging. Lesion hyperenhancement should be observed throughout the arterial phase, followed by the washout during the venous phase. The diagnosis can also be based on the histopathological evaluation of liver biopsy specimen. Although the standards are clear, we often see patients with advanced HCC in clinical practice, who cannot be offered any effective treatment. Patients with chronic liver disease, presenting with inconclusive and changeable test results, constitute a separate problem. In such cases the diagnostic process is typically long-term and delayed. In this paper we present three case reports where the diagnosis could not be made promptly and the patients died as a result of a delayed diagnostic process. Monika Pazgan-Simon, Sylwia Serafinska, Justyna Janocha-Litwin, Krzysztof Simon, and Jolanta Zuwala-Jagiello Copyright © 2015 Monika Pazgan-Simon et al. All rights reserved. Foreign Body Granulomas Induced by Intramuscular Leuprorelin Acetate Injection for Prostate Cancer: Clinical Mimics of Soft Tissue Sarcoma Tue, 31 Mar 2015 13:33:01 +0000 We describe two cases of florid, foreign body granulomatous reaction occurring in the upper arms of males in their eighth decade, who were undergoing treatment with depot injection of leuprorelin acetate for prostatic carcinoma. These patients presented with rapidly enlarging extremity soft tissue masses and were referred to a tertiary sarcoma center with clinical suspicion of a primary soft tissue neoplasm. The occurrence of injection site granulomas secondary to leuprorelin acetate administration is rarely known outside the urological and dermatological communities, and their recognition is important due to the spectrum of clinical differential diagnoses and potential for diagnostic confusion with metastatic prostatic cancer and primary sarcoma and in order to avoid unnecessary stress and clinical intervention for patients. Khin Thway, Dirk C. Strauss, Myles J. Smith, and Cyril Fisher Copyright © 2015 Khin Thway et al. All rights reserved. False Positive Radioiodinated Metaiodobenzylguanidine (123I-MIBG) Uptake in Undifferentiated Adrenal Malignant Tumor Mon, 30 Mar 2015 12:16:17 +0000 123I-Metaiodobenzylguanidine (123I-MIBG) scintigraphy is a widely used functional imaging tool with a high degree of sensitivity and specificity in diagnosis of pheochromocytoma. However, rare cases of false positive reactions have been reported. A 67-year-old male patient was admitted with epigastric pain. Abdominal computed tomography (CT) revealed a heterogeneous left adrenal mass 6 cm in diameter; following hormone testing, 123I-MIBG scintigraphy was performed to determine the presence of pheochromocytoma, which confirmed eccentric uptake by a large left adrenal gland mass. Chest CT and PET-CT confirmed metastatic lymphadenopathy; therefore, endobronchial ultrasound transbronchial needle aspiration was performed. Metastatic carcinoma of unknown origin was suspected from a lymph node biopsy, and surgical resection was performed for definitive diagnosis and correction of excess hormonal secretion. A final diagnosis of undifferentiated adrenal malignant tumor was rendered, instead of histologically malignant pheochromocytoma, despite the uptake of 123I-MIBG demonstrated by scintigraphy. Hee Soo Jung, Seok Jun Moon, Yun Mi Kim, Hye Rim Kang, Seok Mo Lee, Soo Jin Jung, Seok Jin Choi, Tae Kyoon Kim, Min Jeong Kwon, Jeong Hyun Park, and Soon Hee Lee Copyright © 2015 Hee Soo Jung et al. All rights reserved. Late Onset Ipilimumab-Induced Pericarditis and Pericardial Effusion: A Rare but Life Threatening Complication Mon, 30 Mar 2015 12:02:59 +0000 Metastatic cutaneous melanoma has poor prognosis with 2-year survival rate of 10–20%. Melanoma cells express various antigens including gp100, melanoma antigen recognized by T cells 1 (MART-1), and tyrosinase, which can induce immune-mediated anticancer response via T cell activation. Cytotoxic T-lymphocyte associated antigen-4 (CTLA-4) is an immune check point molecule that negatively regulates T cell activation and proliferation. Accordingly, recent phase III clinical trials demonstrated significant survival benefit with ipilimumab, a human monoclonal antibody (IgG1) that blocks the interaction of CTLA-4 with its ligands. Since the efficacy of ipilimumab depends on T cell activation, it is associated with substantial risk of immune mediated adverse reactions such as colitis, hepatitis, thyroiditis, and hypophysitis. We report the first case of late onset pericarditis and cardiac tamponade associated with ipilimumab treatment in patient with metastatic cutaneous melanoma. Seongseok Yun, Nicole D. Vincelette, Iyad Mansour, Dana Hariri, and Sara Motamed Copyright © 2015 Seongseok Yun et al. All rights reserved. Tolosa-Hunt Syndrome in Double-Hit Lymphoma Mon, 30 Mar 2015 11:21:50 +0000 Tolosa-Hunt syndrome (THS) is a painful condition characterized by hemicranial pain, retroorbital pain, loss of vision, oculomotor nerve paralysis, and sensory loss in distribution of ophthalmic and maxillary division of trigeminal nerve. Lymphomas rarely involve cavernous sinus and simulate Tolosa-Hunt syndrome. Here we present a first case of double-hit B cell lymphoma (DHL) relapsing and masquerading as Tolosa-Hunt syndrome. The neurological findings were explained by a lymphomatous infiltration of the right Gasserian ganglion which preceded systemic relapse. As part of this report, the diagnostic criteria for Tolosa-Hunt syndrome and double-hit lymphoma are reviewed and updated treatment recommendations are presented. Prakash Peddi, Kevin M. Gallagher, Chandrikha Chandrasekharan, Qi Wang, Eduardo Gonzalez-Toledo, Binu S. Nair, Reinhold Munker, Glenn M. Mills, and Nebu V. Koshy Copyright © 2015 Prakash Peddi et al. All rights reserved. Metastatic Colonic Adenocarcinoma in Breast: Report of Two Cases and Review of the Literature Wed, 25 Mar 2015 11:34:18 +0000 Metastatic adenocarcinoma to the breast from an extramammary site is extremely rare. In the literature, the most current estimate is that extramammary metastases account for only 0.43% of all breast malignancies and that, of these extramammary sites, colon cancer metastases form a very small subset. Most commonly seen metastasis in breast is from a contralateral breast carcinoma, followed by metastasis from hematopoietic neoplasms, malignant melanoma, sarcoma, lung, prostate, and ovary and gastric neoplasms. Here we present two rare cases, in which colonic adenocarcinomas were found to metastasize to the breast. In both cases, core biopsies were obtained from the suspicious areas identified on mammogram. Histopathology revealed neoplastic proliferation of atypical glandular components within benign breast parenchyma which were morphologically consistent with metastatic adenocarcinoma. By immunohistochemical staining, it was confirmed that the neoplastic components were immunoreactive to colonic markers and nonreactive to breast markers, thus further supporting the morphologic findings. It is extremely important to make this distinction between primary breast cancer and a metastatic process, in order to provide the most effective and appropriate treatment for the patient and to avoid any harmful or unnecessary surgical procedures. Jiten P. Kothadia, Rezina Arju, Monica Kaminski, Arvind Ankireddypalli, Sushil Duddempudi, Jonathan Chow, and Shah Giashuddin Copyright © 2015 Jiten P. Kothadia et al. All rights reserved. Lymphocyte-Predominant Hodgkin’s Disease in Children: A Case Study and Review of the Literature Tue, 24 Mar 2015 13:49:20 +0000 A three-year-old boy presented with an enlarging neck mass. Biopsy demonstrated IgD-positive nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL), which was staged as IIa. The patient received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with rituximab and had excellent results. NLPHL is a relatively rare disease that is biologically distinct from classic Hodgkin lymphoma (cHL). NLPHL is a B-cell malignancy likely of germinal center origin that has an overall good prognosis and favorable response to treatment. Unlike cHL, NLPHL is ubiquitously CD20-positive. Recent evidence supports the efficacy of targeted anti-CD20 therapy in NLPHL, though prospective data is limited. This case demonstrates several unique features of NLPHL and further supports the use of rituximab in front-line therapy. The clinical characteristics among patients at various ages are discussed with a special focus on the IgD-positive subtype. A thorough literature search demonstrates this to be the youngest patient with NLPHL yet described. James R. Stier and Robert J. Vasquez Copyright © 2015 James R. Stier and Robert J. Vasquez. All rights reserved. Neuroendocrine Cell Carcinoma of Unknown Primary Arising in Long Standing History of Multiple Sclerosis Tue, 24 Mar 2015 13:20:58 +0000 Multiple sclerosis (MS) is a chronic autoimmune disease that targets myelinated axons in the central nervous system (CNS). Cancer of unknown primary site (CUP) is a well-recognised clinical disorder, accounting for 3–5% of all malignant epithelial tumors. CUP is clinically characterised as an aggressive disease with early dissemination. Studies of cancer risk in MS patients have shown inconsistent findings. An increased risk of malignancy in patients with MS has been suggested, but recently serious questions have been raised regarding this association. Use of disease-modifying therapies might contribute to an increased cancer risk in selected MS patients. The concurrence of MS and CUP is exceptionally rare. Here we describe the case of a neuroendocrine carcinoma of unknown primary diagnosed in a male patient with a nine-year history of MS. The discussion includes data from all available population-based register studies with estimates of certain malignancies in patients with MS. Stergios Boussios, Vasiliki Kostadima, Anna Batistatou, Ioannis Tourkantonis, George Fotopoulos, Maria I. Argyropoulou, and Nicholas Pavlidis Copyright © 2015 Stergios Boussios et al. All rights reserved. Case Report of a Patient with Left Ventricular Assistance Device Undergoing Chemotherapy for a New Diagnosis of Lung Cancer Sun, 22 Mar 2015 13:29:45 +0000 The optimal management of cancer in patients with severe heart failure is not defined. This issue is particularly challenging when a diagnosis of limited-stage small cell lung cancer (SCLC) is made incidentally in the context of evaluating patient for candidacy for cardiac transplantation. Limited-stage SCLC is typically managed on a curative therapeutic paradigm with combined modality approach involving chemotherapy and radiation. Even with excellent performance status and good organ function, the presence of severe cardiomyopathy poses significant challenges to the delivery of even single modality approach with chemotherapy or radiotherapy, let alone the typical curative combined modality approach. With mechanical left ventricular devices to provide cardiac support, treatment options for cancer in the setting of advanced heart failure may be improved. Here we discuss the therapeutic dilemma involving a patient with severe cardiomyopathy and left ventricular assistant device (LVAD) who was found to have limited-stage SCLC during the evaluation process for cardiac transplantation. Maliha Khan, Anum Wasim, Aibek E. Mirrakhimov, Blaithin A. McMahon, Daniel P. Judge, Linda C. Chu, Ashtami Banavali, and Amer M. Zeidan Copyright © 2015 Maliha Khan et al. All rights reserved. The Case of an Elderly Male Patient with Unknown Primary Mucinous Adenocarcinoma within Presacral Teratoma (Teratoma with Malignant Transformation) Sun, 22 Mar 2015 12:50:24 +0000 Teratomas are rarely seen in adults, and presacral region is an area where they rarely settle in. Similarly, only about 1% of teratomas show malignant transformation. Malignant transformation is often associated with the area where teratoma settles in. Malignant transformation of mediastinal teratomas is more frequent than the ones located in retroperitoneal area and gonad. They most commonly show rhabdomyosarcoma, primitive neuroectodermal tumor, enteric adenocarcinoma, and leukemia transformation. In teratomas showing malignant transformation, the clinical course is aggressive; and survival of patients with metastatic disease is very low. The primary treatment of teratomas with malignant transformations is surgical. Effect of radiotherapy and chemotherapy is not clear in patients, to whom surgical operation cannot be applied, or those who are with residual tumor, even if surgical operation can be applied to them, or those who are at metastatic stage. In this paper, we presented a 76-year-old male patient due to the histologic diagnosis of mucinous adenocarcinoma within teratoma, in whom approximately 7 cm presacral mass was found during the radiographic examination made by the reason of low back pain and pelvic pain. Ozgur Tanriverdi, Ayca Ersen, Suna Cokmert, Emine Koca, Naki Bulut, Suha Gul, and Nevin Yilmaz Copyright © 2015 Ozgur Tanriverdi et al. All rights reserved. Metastasis of Ciliary Body Melanoma to the Contralateral Eye: A Case Report and Review of Uveal Melanoma Literature Sun, 22 Mar 2015 10:45:44 +0000 Many types of cancers metastasize to the eye. However, uveal melanoma metastasizing to the contralateral choroid is very rare. We report the case of a 68-year-old man with history of treated uveal melanoma of the right eye that developed metastasis to the liver and the choroid of the left eye. Ten years earlier, he was diagnosed to have uveal melanoma of the right eye and was initially treated with plaque radiotherapy. Two years later, upon progression of the disease in the right eye he had enucleation of the eye. We describe the patient’s clinical history, the diagnosis of recurrent disease in the contralateral eye, therapy of the left eye, and systemic metastasis. In addition, we reviewed the published medical literature and described the recent advances in the management of uveal melanoma. Nouritza M. Torossian, Roy T. Wallace, Wen-Jen Hwu, and Agop Y. Bedikian Copyright © 2015 Nouritza M. Torossian et al. All rights reserved. A Unique Presentation of Occult Primary Breast Cancer with a Review of the Literature Wed, 18 Mar 2015 11:14:25 +0000 We are reporting a case of a 34-year-old woman with occult primary breast cancer discovered after initially presenting with neurological symptoms. She was successfully treated with neoadjuvant chemotherapy followed by definitive axillary lymph node dissection and ipsilateral whole breast radiotherapy. The case presented is unique due to the rarity of occult primary breast cancer, especially in light of her initial confounding neurological signs and symptoms, which highlights the importance of careful staging. Inaya Ahmed, Kavita Dharmarajan, Amy Tiersten, Ira Bleiweiss, Hank Schmidt, Sheryl Green, and Richard L. Bakst Copyright © 2015 Inaya Ahmed et al. All rights reserved. Safety and Efficacy of Combined Yttrium 90 Resin Radioembolization with Aflibercept and FOLFIRI in a Patient with Metastatic Colorectal Cancer Mon, 16 Mar 2015 12:36:10 +0000 Background. When associated with isolated four or fewer liver foci, metastatic colorectal cancer is amenable to surgical resection. Alternative therapeutic methods for isolated liver metastases include radioembolization with yttrium 90 (Y90) and transarterial chemoembolization (TACE). We present here a case of a patient with two sites of liver metastatic disease from colorectal cancer who underwent Y90 radioembolization combined with aflibercept and FOLFIRI. Case Report. A 56-year-old female with history of bilateral breast cancer and metastatic colon cancer with prior hemicolectomy and 4 previous chemotherapy regimens developed liver metastasis. She was started on aflibercept and FOLFIRI and concurrently underwent two treatments of radioembolization with Y90, initially targeting the largest right lobe tumor, and then a subsequent treatment targeting the smaller left lobe tumor with retreatment of the right lobe tumor. Her liver metastases exhibited partial response on imaging utilizing the modified RECIST criteria. Interestingly, the patient CEA levels decreased after the procedure. Discussion. This is the first reported case of a patient managed with radioembolization with Y90 combined with aflibercept, an anti-VEGF treatment, and FOLFIRI. An ongoing randomized clinical trial aims to define the role of combined targeted therapy and chemotherapy with radioembolization with Y90. Andre De Souza, Kevin Pelham Daly, James Yoo, and Muhammad Wasif Saif Copyright © 2015 Andre De Souza et al. All rights reserved. Maintenance Therapy Containing Metformin and/or Zyflamend for Advanced Prostate Cancer: A Case Series Sun, 15 Mar 2015 13:08:17 +0000 Metformin is derived from galegine, a natural ingredient, and recent studies have suggested that metformin could enhance the antitumor effects of hormone ablative therapy or chemotherapy and reduce prostate cancer-specific mortality. Zyflamend is a combination of herbal extracts that reduces inflammation and comprises turmeric, holy basil, green tea, oregano, ginger, rosemary, Chinese goldthread, hu zhang, barberry, and basil skullcap. We propose a maintenance regimen with metformin and/or Zyflamend that targets cancer stem cells and the tumor microenvironment to keep the cancer dormant and prevent it from activation from dormancy. Herein, we report the clinical course of four patients who experienced a clinical response after treatment with metformin and/or Zyflamend. Mehmet Asim Bilen, Sue-Hwa Lin, Dean G. Tang, Kinjal Parikh, Mong-Hong Lee, Sai-Ching J. Yeung, and Shi-Ming Tu Copyright © 2015 Mehmet Asim Bilen et al. 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.