Case Reports in Oncological Medicine The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Renal Medullary Carcinoma: Case Report of an Aggressive Malignancy with Near-Complete Response to Dose-Dense Methotrexate, Vinblastine, Doxorubicin, and Cisplatin Chemotherapy Tue, 19 Aug 2014 07:12:00 +0000 Renal medullary carcinoma (RMC) is a rare but aggressive malignancy affecting young individuals with sickle cell trait. Renal medullary carcinoma commonly presents with advanced or metastatic disease and is associated with a rapidly progressive clinical course and an extremely short overall survival measured in weeks to few months. Due to the rarity of RMC, there is no proven effective therapy and patients are often treated with platinum-based chemotherapy. We report near-complete radiological and pathological response in a patient treated with dose-dense MVAC (methotrexate, vinblastine, doxorubicin, and cisplatin) chemotherapy. The patient underwent consolidation nephrectomy and retroperitoneal lymph node dissection and had a 16-month progression-free survival, one of the longest reported in patients with RMC. Ali Imran Amjad, Hira Ali, Leonard J. Appleman, Jodi Maranchie, Stephen Jackman, Anil Parwani, Rajiv Dhir, Somak Roy, and Rahul A. Parikh Copyright © 2014 Ali Imran Amjad et al. All rights reserved. Synchronous Gastric Gastrointestinal Stromal Tumor and Colon Adenocarcinoma: A Case Report Sun, 17 Aug 2014 12:34:44 +0000 Gastrointestinal stromal tumors (GISTs) represent the majority of primary mesenchymal tumors of the gastrointestinal tract. They are generally considered to be solitary tumors and therefore the synchronous occurrence with other primary malignancies of gastrointestinal track is considered a rare event. Here we present the case of a 75-year-old man admitted to our hospital with a 10-day history of gastrointestinal bleeding. Colonoscopy revealed an ulcerative mass of 4 cm in diameter in the ascending colon. Gastroscopy revealed a bulge in the gastric body measuring 1 cm in diameter with normal overlying mucosa. Surgical intervention was suggested and ileohemicolectomy with regional lymph node resection along with gastric wedge resection was performed. Pathologic examination of the ascending colon mass showed an invasive moderately differentiated adenocarcinoma stage III B (T3N1M0). Grossly resected wedge of stomach showed a well circumscribed intramural tumor which microscopically was consistent with essentially benign gastrointestinal stromal tumor (according to Miettinen criteria). The patient did not receive additional treatment. Two years later the patient showed no evidence of recurrence or metastasis. Thivi Vasilakaki, Kalliroi Koulia, Aikaterini Tsavari, Elissavet Arkoumani, Efstratios Kouroumpas, Anargiros Pavlis, Georgios Christopoulos, Konstantinos Stamatiou, Kassiani Manoloudaki, and Dimitrios Zisis Copyright © 2014 Thivi Vasilakaki et al. All rights reserved. Tension Pneumocephalus Related to Radiotherapy for Nasopharyngeal Carcinoma Thu, 14 Aug 2014 11:54:20 +0000 Introduction. Tension pneumocephalus (TP) is a very rare complication related to radiotherapy for nasopharyngeal carcinoma (NPC). Case Presentation. A 46-year-old man was admitted to the hospital with an altered mental status and aqueous rhinorrhea for several hours of evolution. The computed tomography (CT) scan showed TP, a defect in the skull base and nasocranial fistula. The patient was receiving a second course of radiotherapy for local relapse. With medical treatment the patient recovered neurological status but died two days later. Discussion. In our knowledge, only 4 cases with similar characteristics have been reported in the literature. This is the first case report of TP during radiotherapy. TP was an abrupt and rapid process with neurological impairment for hours of evolution without suspicious osteoradionecrosis (OR) in previous scan images. The defect in the skull base could be due to a rapid disappearance of the tumor. The appearance of aqueous rhinorrhea and neurological symptoms must be viewed as signs of alarm. Esther Jimenez-Jimenez, Sebastià Martí, and M. Victoria Villas Copyright © 2014 Esther Jimenez-Jimenez et al. All rights reserved. An Incidental Finding of Mucinous Colon Cancer by 18F-Choline PET/CT Determining a Change in Clinical Management of a Patient with Recurrent Prostate Adenocarcinoma Wed, 13 Aug 2014 00:00:00 +0000 A 66-year-old-man underwent a PET/CT scan after a biochemical relapse for a prostate cancer previously treated with a laparoscopic surgical procedure which revealed a focal uptake in the posterior wall of sigmoid colon. The biopsy demonstrated a colon cancer with mucinous differentiation producing a shift in clinical priority. To the best of our knowledge this is the first report in the English literature describing the detection by 18F-choline PET/CT of a colorectal cancer with mucinous differentiation. Carmelo Tuscano, Elvio Grazioso Russi, Said Al Sayyad, and Stefano Pergolizzi Copyright © 2014 Carmelo Tuscano et al. All rights reserved. Malign Recurrence of Primary Chest Wall Hemangiopericytoma in the Lung after Four Years: A Case Report and Review of the Literature Wed, 13 Aug 2014 00:00:00 +0000 Hemangiopericytoma (HPC) may develop in every site where the endothelial tissue exits and primarily develops in the skeletal-muscular system or the skin. Adult cases of HPC generally exhibit a benign course. 20–30% of the cases may show a malign course. The tumors that show more than four mitoses, a focal area of necrosis, and increased cellularity on a magnification ×10 are considered as malign. In our paper, we presented our case who showed a lung metastasis at the end of 4 years and who developed a pathological fracture of the right humerus at the end of approximately 2 years, because hemangiopericytoma is rarely seen in the chest wall as a primary tumor. Tulay Akman, Ahmet Alacacioglu, Devrim Dolek, Tugba Unek, Duygu Gurel, Ahmet Ugur Yilmaz, and Ahmet Onen Copyright © 2014 Tulay Akman et al. All rights reserved. Gradual Recovery from Nonambulatory Quadriparesis Caused by Metastatic Epidural Cervical Cord Compression in an Octogenarian Gallbladder Carcinoma Patient Treated with Image-Guided Three-Dimensional Conformal Radiotherapy Alone Using a Field-in-Field Technique Mon, 11 Aug 2014 11:41:25 +0000 Radiotherapy for acute metastatic epidural spinal cord compression (MESCC) involves conventional techniques and dose fractionation schemes, as it needs to be initiated quickly. However, even with rapid intervention, few paraplegic patients regain ambulation. Here, we describe the case of a mid-octogenarian who presented with severe pain and nonambulatory quadriparesis attributable to MESCC at the fifth cervical vertebra, which developed 10 months after the diagnosis of undifferentiated carcinoma of the gallbladder. Image-guided three-dimensional conformal radiotherapy (IG-3DCRT) was started with 25 Gy in 5 fractions followed by a boost of 12 Gy in 3 fractions, for which a field-in-field (FIF) technique was used to optimize the dose distribution. Despite the fact that steroids were not administered, the patient reported significant pain reduction and showed improved motor function 3 and 4 weeks after the IG-3DCRT, respectively. Over the following 4 months, her neurological function gradually improved, and she was consequently able to eat and change clothes without assistance and to walk slowly for 10–20 m using a walker. She succumbed to progression of abdominal disease 8.5 months after the IG-3DCRT. This case demonstrates that image-guided FIF radiotherapy with a dose-escalated hypofractionated regimen can potentially improve functional outcome and local control. Kazuhiro Ohtakara and Hiroaki Hoshi Copyright © 2014 Kazuhiro Ohtakara and Hiroaki Hoshi. All rights reserved. High Grade Myofibroblastic Sarcoma of Paratesticular Soft Tissues Mon, 11 Aug 2014 00:00:00 +0000 Tumors of the paratesticular region most often arise from the soft tissue surrounding the spermatic cord and the epididymis or from the soft tissue (dartos muscle) of the scrotal wall. Paratesticular tumors, despite their rarity, present a high incidence of malignancy (30%), and the therapeutic approach of choice is surgical resection with negative margin. The grade, the histology type, the presence of metastases during the diagnosis, the size of the tumor, the age of the patients, and the surgical margins are all important prognostic factors. We present a case report of a 86-year-old patient with a high grade paratesticular and scrotum sarcoma of soft tissues which was presented as a hard painful mass of the scrotum. The patient was subjected to high ligation of the spermatic cord and received no further treatment and 6 months after the operation no local or systematic recurrence was observed. Ioannis Anastasiou, Panagiotis K. Levis, Ioannis Katafigiotis, Georgios Karaolanis, Viktoria-Varvara Palla, Evangelos Felekouras, Antonios Athanasiou, Marina Perdiki, Dionysios Mitropoulos, and Constantinos A. Constantinides Copyright © 2014 Ioannis Anastasiou et al. All rights reserved. Concomitant Bifocal Urothelial Carcinoma and Breast Tumor: Second Primary Cancer or Metastatic Spread to the Breast? Mon, 04 Aug 2014 12:13:19 +0000 Metastases to the mammary gland are an uncommon event in the natural history of most malignant tumors. We report the case of a 60-year-old woman who presented initially with bifocal urothelial carcinoma with a single breast tumor, raising the issue of a primary cancer or a metastatic spread to the breast. The diagnosis of breast metastasis was aided by identity of pathology, immunochemistry, and molecular biology findings between the primary tumor and the breast lesion, among which are the p.K120M mutation, a very rare TP53 mutation, and HER2 overexpression with underlying polysomy of chromosome 17. Clément Dumont, Hélène Gauthier, Jérôme Vérine, Jacqueline Lehmann-Che, Patricia de Cremoux, Damien Pouessel, and Stéphane Culine Copyright © 2014 Clément Dumont et al. All rights reserved. Massive Periocular Squamous Cell Carcinoma Engulfing the Globe: A Rare Case Report Thu, 24 Jul 2014 07:15:38 +0000 The eyelid tumors are rare neoplasms in head and neck oncological practice. The maximum incidence is after sixty years and most of the tumors are situated in the lower eyelid and medial canthus. A wide range of clinical presentations contribute to a high risk of misdiagnosis. The factors such as very large lesions, incomplete excision, histopathologic features such as poor differentiation, multicentric origin, pagetoid spread, and delayed diagnosis are associated with poor prognosis. Because of different tissues at eyelid level, a variety of tumor types and subtypes can arise, but most of them are carcinomas. A rare case of eyelid carcinoma spreading and engulfing the whole of globe which was treated by orbital exenteration and postoperative radiotherapy is presented with a disease-free follow-up of 10 months which, considering its size, is extremely rare. The early diagnosis and proper treatment of such rare tumors still remain the mainstay to predict favourable prognosis. Rajender Singh Arora, Anirudh Bhattacharya, Dwarkadas Adwani, and Sidak Singh Arora Copyright © 2014 Rajender Singh Arora et al. All rights reserved. Myasthenia Gravis-Like Syndrome Presenting as a Component of the Paraneoplastic Syndrome of Lung Adenocarcinoma in a Nonsmoker Wed, 23 Jul 2014 00:00:00 +0000 Adenocarcinoma of the lung is the most common form of lung cancer in nonsmokers. It is commonly seen in the periphery of the lungs. Myasthenia gravis is generally associated with mediastinal malignancies and rarely associated with adenocarcinoma of the lung. We present a case of a 38-year-old male nonsmoker with rapidly progressive adenocarcinoma of the lung associated with myasthenia gravis, a patient whom expired within 27 days of hospital admission and diagnosis. Sahar Eivaz-Mohammadi, Fernando Gonzalez-Ibarra, Hesam Hekmatjou, Rao Mikkilineni, Amit Patel, and Amer K. Syed Copyright © 2014 Sahar Eivaz-Mohammadi et al. All rights reserved. Maintenance Therapy with Trastuzumab in Her2 Positive Metastatic Parotid Ductal Adenocarcinoma Thu, 17 Jul 2014 12:05:05 +0000 Salivary ductal carcinomas (SDCs) are extremely rare and aggressive malignancies, accounting for approximately 6% of all salivary gland malignancies. One distinct feature is their resemblance to ductal carcinomas of breast. A significant percentage of SDCs overexpress Her2 and the use of targeted therapy with trastuzumab can be considered in these patients. We report a rare case of long term disease control with trastuzumab in Her2 positive metastatic parotid ductal carcinoma. Our case also highlights that isolated brain metastasis should be managed aggressively to allow optimal local control when systemic disease is under remission with trastuzumab. We have also reviewed the published literature on the use of trastuzumab in SDCs. Muhammad Shahid Iqbal, Ghazia Shaikh, Sanjoy Chatterjee, Helen Cocks, and Josef Kovarik Copyright © 2014 Muhammad Shahid Iqbal et al. All rights reserved. Large Cell Neuroendocrine Carcinoma of the Rectum Presenting with Extensive Metastatic Disease Wed, 16 Jul 2014 12:13:50 +0000 Introduction. Rectal large cell neuroendocrine carcinoma (LCNEC) is a poorly differentiated neoplasm that is very rare and belongs within the poorest prognostic subgroup among primary colorectal neoplasms. Here, we describe a case of LCNEC of the rectum, which highlights the aggressive clinical course and poor prognosis associated with this disease. Case Presentation. We report a case of a 63-year-old male who presented to our hospital with a one-month history of lower abdominal pain, constipation, and weight loss. A computed tomography (CT) scan of the chest, abdomen, and pelvis revealed a rectal mass as well as metastatic disease of the liver and lung. Flexible sigmoidoscopy revealed a fungating, ulcerated and partially obstructing rectal mass located 6 cm from the anal verge. This mass was biopsied and pathological examination of the resected specimen revealed features consistent with a large cell neuroendocrine carcinoma. Conclusion. Rectal large cell neuroendocrine carcinomas are rare and have a significantly worse prognosis than adenocarcinomas. At diagnosis, a higher stage and metastatic disease are likely to be found. It is important to differentiate large cell, poorly differentiated neuroendocrine carcinomas from adenocarcinomas of the colon and rectum pathologically because patients may benefit from alternative cytotoxic chemotherapeutic regimens. Vinay Minocha, Sania Shuja, Robert Ali, and Emely Eid Copyright © 2014 Vinay Minocha et al. All rights reserved. Carcinoma Buccal Mucosa Underlying a Giant Cutaneous Horn: A Case Report and Review of the Literature Tue, 15 Jul 2014 09:12:18 +0000 Cutaneous horn is a conical, dense, and hyperkeratotic protrusion that often appears similar to the horn of an animal. Giant cutaneous horns are rare; no incidence or prevalence has been reported. The significance of cutaneous horns is that they occur in association with, or as a response to, a wide variety of underlying benign, premalignant, and malignant cutaneous diseases. A case of giant cutaneous horn of left oral commissure along with carcinoma left buccal mucosa is reported here as an extremely rare oral/perioral pathology. Sunil Kumar, Priyank Bijalwan, and Sunil K. Saini Copyright © 2014 Sunil Kumar et al. All rights reserved. Malignant Mesenteric Perivascular Epithelioid Cell Neoplasm Presenting as an Intra-Abdominal Fistula in a 49-Year-Old Female Thu, 10 Jul 2014 00:00:00 +0000 Perivascular epithelioid cell tumors are rare mesenchymal tumors arising from histologically and immunohistochemically distinctive perivascular epithelioid cells that express both myogenic and melanocytic markers. These tumors are known to arise from different organs in the body and usually have an unpredictable clinical course. We report a case of a 49-year-old female who presented with diffuse abdominal pain, fever, chills, and nonbilious vomiting for a day. Work-up revealed a mesenteric mass measuring  cm, arising in the mesentery of the hepatic flexure, with adjacent gas suggestive of fistularization into the right colon. An exploratory laparotomy with resection of the mesenteric mass was performed, and the initial histopathology results were compatible with either an adenocarcinoma or a sarcoma; however, because of poor differentiation it was difficult to make a definitive diagnosis. However, final histopathology results revealed a malignant perivascular epithelioid cell tumor (with reservation that a S100 negative metastatic melanoma must be excluded clinically). Following surgery the patient was started on everolimus, an m-TOR inhibitor, and has shown good response to this medication. Sakshi Kapur, Napoleon K. Patel, Miles B. Levin, and Richard Huang Copyright © 2014 Sakshi Kapur et al. All rights reserved. Primary Angiosarcoma of the Spleen: An Oncological Enigma Wed, 02 Jul 2014 10:02:39 +0000 Introduction. Primary splenic angiosarcoma is an extremely unusual neoplasm originating from sinusoidal vascular endothelium. Surgical extirpation is the mainstay of treatment of this highly malignant disease. Case Presentation. An 82-year-old woman was admitted with left pleural effusion and a palpable left upper quadrant abdominal mass, secondary to splenomegaly by two large splenic tumors. Classic open splenectomy was performed and angiosarcoma of the spleen was the final histopathological diagnosis, which was primary since no other disease site was revealed. Discussion. The incidence of the disease is 0.14–0.23 cases per million, with slight male predominance. Etiology is not established and clinical presentation may confuse even experienced physicians. Imaging modalities cannot differentiate the lesion from other vascular splenic neoplasms and the correct diagnosis is mainly set after histopathological examination of the resected spleen. As with other sarcomas, surgery is the only curative approach, while chemo- and radiotherapy have poor results. Prognosis remains dismal. Myoteri Despoina, Dellaportas Dionysios, Ayiomamitis Georgios, Strigklis Konstantinos, Kouroumpas Efstratios, and Zizi-Sermpetzoglou Adamantia Copyright © 2014 Myoteri Despoina et al. All rights reserved. Symptomatic Histologically Proven Necrosis of Brain following Stereotactic Radiation and Ipilimumab in Six Lesions in Four Melanoma Patients Tue, 01 Jul 2014 08:54:10 +0000 Four cases previously treated with ipilimumab with a total of six histologically confirmed symptomatic lesions of RNB without any sign of active tumour following stereotactic irradiation of MBM are reported. These lesions were all originally thought to be disease recurrence. In two cases, ipilimumab was given prior to SRT; in the other two ipilimumab was given after SRT. The average time from first ipilimumab to RNB was 15 months. The average time from SRT to RNB was 11 months. The average time from first diagnosis of MBM to last follow-up was 20 months at which time three patients were still alive, one with no evidence of disease. These cases represent approximately three percent of the total cases of melanoma and ten percent of those cases treated with ipilimumab irradiated in our respective centres collectively. We report this to highlight this new problem so that others may have a high index of suspicion, allowing, if clinically warranted, aggressive surgical salvage, possibly resulting in increased survival. Further studies prospectively collecting data to understand the denominator of this problem are needed to determine whether this problem is just the result of longer survival or whether there is some synergy between these two modalities that are increasingly being used together. Stephanie Du Four, Angela Hong, Matthew Chan, Michail Charakidis, Johnny Duerinck, Sofie Wilgenhof, Wei Wang, Linda Feng, Alex Michotte, Meena Okera, Brindha Shivalingam, Gerald Fogarty, Richard Kefford, and Bart Neyns Copyright © 2014 Stephanie Du Four et al. All rights reserved. Her-2 Positive Gastric Cancer Presented with Thrombocytopenia and Skin Involvement: A Case Report Mon, 23 Jun 2014 00:00:00 +0000 Gastric cancer is the 5th most frequent cancer around the world and the 3rd most frequent reason of deaths due to cancer. Every year, about 1 million new cases are taking place, with varying geographical distribution. Gastric cancer is often metastatic to liver, lungs, and bones in hematogenous way, to peripheral lymph nodes in lymphogenous way, and to peripheral tissues in adjacency way, yet bone marrow (BM) and cutaneous metastasis are quite seldom. Pancytopenia is a more frequent finding identified in BM metastasis of solid organ cancers, and isolated thrombocytopenia is less often. The human epidermal growth factor 2 (HER-2) is positive in gastric cancer at a rate of 7–34%. Here, we have presented our HER-2 positive gastric cancer incident which presented with BM and cutaneous metastasis, and has no 18F-fluoro-2-deoxi-D-glucose (FDG) involvement except bone metastases. Deniz Arslan, Mukremin Uysal, Ali Murat Tatlı, Seyda Gunduz, Sema Sezgin Goksu, Cumhur İbrahim Başsorgun, Hasan Senol Coskun, Hakan Bozcuk, and Burhan Savaş Copyright © 2014 Deniz Arslan et al. All rights reserved. Mixed Germ Cell Testicular Cancer with Left Ventricular Metastasis Presenting with Embolic Stroke and Small Bowel Tumor Seeding Wed, 18 Jun 2014 09:13:48 +0000 Testicular germ cell tumors (GCTs) metastasize in a very predictable fashion involving the retroperitoneal nodes first followed by hematogenous spread to distant organs like lungs, liver, and brain. Metastasis to heart is an extremely rare entity for GCT and fewer than 20 cases have been reported in the literature so far. We have summarized here a unique case of nonseminomatous germ cell tumor (NSGCT) with intracardiac metastasis resulting in systemic macroembolization to liver, spleen, brain, bowel and musculoskeletal tissues. This led to multiple adverse sequelae including ischemic stroke and bowel perforation. Srinath Sundararajan, Beth Braunhut, Frederick Ahmann, and Amit Agarwal Copyright © 2014 Srinath Sundararajan et al. All rights reserved. Isolated Liver Metastasis of Sacrococcygeal Chordoma: Case Report and Review of the Literature Wed, 11 Jun 2014 11:44:22 +0000 Chordomas are rare neoplasms arising from notochordal remnants and may develop anywhere in the body while the most common anatomic site is the sacrococcygeal area. The most effective treatment of chordoma is surgery. Chordomas rarely metastasize to lung, bone, soft tissue, liver, lymph nodes, and skin. However, there is currently no standard systemic treatment for advanced stage chordoma. Here, we reported a rare presentation of chordoma patient with liver only metastases and poor prognosis. Murat Akyol, Umut Varol, Ibrahim Yildiz, Ibrahim Vedat Bayoglu, Yasar Yildiz, Lutfiye Demir, Ahmet Dirican, Alper Can, Suna Cokmert, Mine Tunakan Oztop, Ahmet Alacacioglu, Yuksel Kucukzeybek, and Mustafa Oktay Tarhan Copyright © 2014 Murat Akyol et al. All rights reserved. Guillain-Barré Syndrome following Treatment with Sunitinib Malate Wed, 11 Jun 2014 08:22:05 +0000 Sunitinib malate (Sutent, SU011248) is an oral multitargeted tyrosine kinase inhibitor (TKI) used for the treatment of metastatic renal cell carcinoma and imatinib (Gleevec)—resistant gastrointestinal stromal tumor (GIST) with few reported side effects including asthenia, myelosuppression, diarrhea, and mucositis. Scarce literature exists regarding the rare but often serious toxicities of sunitinib. Autoimmune and neurological side effects have been linked to sunitinib’s inhibition of VEGF receptors with a corresponding increase in VEGF levels, which is associated with development of different neuropathies. We hereby report an interesting case of Guillain-Barré syndrome in a middle-aged patient with metastatic renal cell carcinoma following sunitinib treatment. Ziad Kanaan, Zain Kulairi, Mirela Titianu, Sandip Saha, and Sarwan Kumar Copyright © 2014 Ziad Kanaan et al. All rights reserved. A Case of Primary Subglottic Malignant Melanoma with a Successful Surgical Treatment Sun, 08 Jun 2014 00:00:00 +0000 Primary subglottic malignant melanoma is a very rare and underdiagnosed neoplasm. We are reporting a case of primary malignant melanoma of subglottic mucosa in a 78-year-old woman who presented to our hospital with shortness of breath and hoarseness of voice. Laryngoscopy and excisional biopsy along with immunoreactivity to S-100 and human melanoma black-45 (HMB-45) confirmed the diagnosis. The patient was treated with laryngectomy followed by radiotherapy. Five years following surgical treatment, she continues to be asymptomatic. To our knowledge, there is only one reported case of primary malignant melanoma of subglottic mucosa in the medical literatures. Shahzad Ahmad, Mahmoud Abdelghany, Curtis Goldblatt, Owen Stark, and Nicholas Masciotra Copyright © 2014 Shahzad Ahmad et al. All rights reserved. Rare Extraperitoneal Involvement with Fatal Outcome in a Case of Bilateral Luteinized Thecoma of the Ovaries with Sclerosing Peritonitis Thu, 05 Jun 2014 10:56:35 +0000 We report the case of a woman diagnosed with bilateral luteinized thecoma of the ovaries with sclerosing peritonitis, multiple intraperitoneal cystic lesions, and extraperitoneal lesions of the liver, inferior to the spleen, and high suspicion of bone marrow involvement. The patient developed profound pancytopenia with rapid clinical deterioration and a fatal outcome. Mohamed A. Medhat, Mohamed A. Y. Abdel Malek, Saad Zaki, Ahmed Helmy, and James J. Driscoll Copyright © 2014 Mohamed A. Medhat et al. All rights reserved. Jejunal Perforation: A Rare Presentation of Burkitt’s Lymphoma—Successful Management Wed, 04 Jun 2014 11:20:04 +0000 Malignant tumors of the small bowel presenting as acute abdomen are a rare occurrence. Burkitt’s lymphoma presenting as a surgical emergency needing emergency laparotomy is an uncommon presentation of this tumor. We present an interesting case of jejunal perforation as a first manifestation of Burkitt’s lymphoma which was successfully managed with surgical resection, high dose chemotherapy, and good supportive care. Samir Ranjan Nayak, Ganni Bhaskara Rao, Subramanya Sarma Yerraguntla, and Sisir Bodepudi Copyright © 2014 Samir Ranjan Nayak et al. All rights reserved. Squamous Cell Carcinoma of the Pancreas in a Patient with Germline BRCA2 Mutation-Response to Neoadjuvant Radiochemotherapy Mon, 19 May 2014 00:00:00 +0000 Primary squamous cell carcinoma of the pancreas is a rare malignant neoplasia, accounting for approximately 0.5–2% of all malignant pancreatic tumors. These lesions are characterized by poor prognosis. Here we report on a case of a 57-year-old female patient with known BRCA2 germline mutation presenting with primary squamous cell carcinoma of the pancreas as the only malignancy. The tumor was locally advanced at the first presentation but responded almost completely to neoadjuvant radio-chemotherapy. Our case highlights the facts (i) that pancreatic carcinomas belong to the tumor spectrum of patients with the BRCA2-associated hereditary breast and ovarian cancer syndrome (HBOC) and (ii) that tumors of the pancreas can represent the first or even the only manifestation of HBOC. Furthermore, this case of a nonkeratinizing squamous cell carcinoma indicates that HBOC-associated carcinomas of the pancreas might be characterized by a broader morphological spectrum than was previously thought. Since BRCA mutations cause deficiency of DNA double-strand breakage repair in tumors, neoadjuvant treatment regimens might become a reasonable option in HBOC-associated pancreatic carcinomas. To our knowledge, this is the first reported case of a primary pancreatic squamous cell carcinoma in a patient with this particular genetic background of BRCA2-associated HBOC. Anne M. Schultheis, Gia Phuong Nguyen, Monika Ortmann, Wolfgang Kruis, Reinhard Büttner, Hans-Ulrich Schildhaus, and Birgid Markiefka Copyright © 2014 Anne M. Schultheis et al. All rights reserved. Small Cell Lung Cancer Presenting as Severe Thrombocytopenia and Refractory Hypokalemia Thu, 15 May 2014 07:25:36 +0000 A 70-year-old female with a history of mild cirrhosis was referred by her primary care provider for a platelet count of 36,000/μL which had dropped from 47,000/μL in a week along with mild pain in extremities. Serum potassium was low (2.9 mEq/L) in spite of the patient being recently started on potassium supplement on outpatient for hypokalemia. Initially thrombocytopenia was attributed to cirrhosis. However, platelet counts continued to drop to a nadir of 9000/μL in spite of several platelet transfusions. Hypokalemia was refractory to potassium supplements. Subsequent bone marrow biopsy revealed extensive marrow necrosis with a focus of small cell tumor cells of pulmonary origin. CT scan of the chest showed a spiculated left lung mass. The ACTH level was high, with normal rennin and aldosterone levels. The patient likely had ectopic ACTH syndrome from small cell lung cancer. She died within few days of diagnosis. Severe thrombocytopenia and refractory hypokalemia can rarely be initial presentations of small cell lung cancer. Thrombocytopenia should prompt an evaluation for bone marrow metastases and a search for undiagnosed systemic malignancy. In severe cases of metastases, bone marrow necrosis can be present. Refractory hypokalemia can be the sole presentation of ectopic ACTH production. Rohan Mandaliya, Lesley Hughes, Herbert Auerbach, and Felice LePar Copyright © 2014 Rohan Mandaliya et al. All rights reserved. Letrozole Induced Hypercalcemia in a Patient with Breast Cancer Thu, 15 May 2014 06:42:48 +0000 Hypersecretion of PTHrP is a relatively common cause of malignancy-related hypercalcemia. However, there is only one case report of letrozole induced hypercalcemia. A 52-year-old female patient was referred to our clinic because of the recent discovery of hypercalcemia (11.0 mg/dL). The patient had a history of left breast carcinoma. She had started a course of letrozole (aromatase inhibitor; 2.5 mg dose/day) ten months earlier. Patient’s parathyroid hormone-related protein levels were normal and a bone scintigram revealed no evidence of skeletal metastasis. Other potential causes of high calcium levels were ruled out. We recognized that, when letrozole was taken at one dose daily (2.5 mg), she had recurrent hypercalcemia. Our experience suggests that letrozole may precipitate hypercalcemia in a patient with breast cancer. Suleyman Hilmi Ipekci, Suleyman Baldane, Ercument Ozturk, Murat Araz, Huseyin Korkmaz, Fatih Colkesen, and Levent Kebapcilar Copyright © 2014 Suleyman Hilmi Ipekci et al. All rights reserved. Reversible Posterior Encephalopathy Syndrome Secondary to Sunitinib Tue, 13 May 2014 00:00:00 +0000 Reversible posterior leukoencephalopathy syndrome (RPLS) is clinical radiologic condition associated with neurological symptoms and cerebral white matter edema. It has been associated with uncontrolled hypertension, eclampsia, immunosuppressants, and more recently the use of antiangiogenic drugs. Sunitinib is an inhibitor of the vascular endothelial growth factor receptor widely used in the treatment of metastatic renal cell carcinoma (RCC). We report a rare case of RPLS occurring on therapy with sunitinib in a patient with RCC. Our aim is to highlight the importance of considering RPLS as a diagnostic possibility and to hold sunitinib for RCC patients presenting with neurologic symptoms. Ricardo Costa, Rubens Costa, Renata Costa, Gilberto Moura de Brito Junior, Henrique Queiroz Cartaxo, and Alex Caetano de Barros Copyright © 2014 Ricardo Costa et al. All rights reserved. Primary Hepatic Lymphoma: A Challenging Diagnosis Sun, 11 May 2014 09:52:02 +0000 Introduction. Primary hepatic lymphoma is an unusual malignancy and is very difficult to diagnose promptly. An intrigue case presenting with cholestatic jaundice is reviewed and main disease characteristics are further discussed. Case Report. A 70-year-old male presented with dull right upper quadrant abdominal pain and mild cholestatic jaundice. Initial evaluation revealed mildly elevated liver function tests and normal tumor markers, while imaging with an abdominal CT-scan showed multiple hypodense nodules in both liver lobes. First impression of metastatic deposits from gastrointestinal origin was not confirmed by endoscopic means. After CT-guided biopsy, primary diffuse large B-cells non-Hodgkin lymphoma was revealed. Appropriate chemotherapy improved patient’s condition markedly. Discussion. Primary hepatic lymphoma is a rare form of extranodal lymphomas, accounting for less than 1% of all extranodal lymphomas in general. In order to define the condition as PHL, liver has to be the only site of lymphoma occurrence or to be involved in a major degree with minimal nonliver disease. Most PHLs are of B-cell origin with large cells as the main cell type. D. Myoteri, D. Dellaportas, E. Arkoumani, A. Marinis, and A. Zizi-Sermpetzoglou Copyright © 2014 D. Myoteri et al. All rights reserved. Blinding Bilateral Hyperviscosity Retinopathy in a 43-Year-Old Nigerian Male with Lymphoplasmacytic Lymphoma: A Case Report and Management Challenges Mon, 05 May 2014 09:21:22 +0000 Lymphoplasmacytic lymphomas are rare and may present with uncommon and devastating symptoms. We report a case of a 43-year-old male who presented with bleeding gums and sudden onset of bilateral blindness but was not on anticoagulants and had no family history of bleeding disorder. He had bilateral hyperpigmented infraorbital skin lesions, visual acuities (VA) of hand motion in both eyes (blindness), round and sluggish pupils, and bilateral diffuse and extensive retinal haemorrhages obliterating the retinal details with central visual field defects. The optical coherence tomography revealed retinal haemorrhage, oedema, detachment, and diffuse photoreceptors damage. Investigations revealed elevated ESR and microglobulin, monoclonal peak on serum protein electrophoresis, high IG with lambda restriction on serum, and urine immunofixation with increased lymphocytes and plasma cells in the bone marrow. A diagnosis of lymphoplasmacytic lymphoma complicated by blinding hyperviscosity retinopathy was made. In the absence of an aphaeresis machine, he received four cycles of manual exchange blood transfusion (EBT) and commenced with chlorambucil/prednisolone due to difficulty in obtaining blood for continued EBT. His general condition and VA has improved and he is stable for more than six months into treatment. Abdulkabir A. Ayanniyi, Uchenna Godswill Ejikeme, Yohanna Tanko, Rilwan C. Muhammad, and Obiageli E. Nnodu Copyright © 2014 Abdulkabir A. Ayanniyi et al. All rights reserved. Oral Metastasis of Metaplastic Breast Carcinoma in a Patient with Neurofibromatosis 1 Wed, 30 Apr 2014 07:15:04 +0000 Neurofibromatosis type 1 (NF1) has been associated with an increased risk for development of malignancy, especially malignant peripheral nerve sheath tumors. In addition, recently, literature has demonstrated an increased risk of breast cancer in women with NF1. The present paper shows a 53-year-old woman with NF1 who presented with metaplastic breast carcinoma and developed multiple metastases, including mandible. Furthermore, we reviewed the English literature, found 63 cases showing the association between NF1 and breast cancer, and added one more case. The present study demonstrated an important association between NF1 and breast cancer. Until the present time, there has been only one case of metaplastic breast carcinoma associated with NF1. Curiously, in our case the oral metastasis corresponded to sarcomatous component of metaplastic breast carcinoma. Ana Paula Molina Vivas, Luana Eschholz Bomfin, Clovis Antonio Lopes Pinto, Ulisses Ribaldo Nicolau, and Fabio Abreu Alves Copyright © 2014 Ana Paula Molina Vivas et al. All rights reserved.