Case Reports in Pathology The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Metastatic Carcinoma Occurring in a Gastric Hyperplastic Polyp Mimicking Primary Gastric Cancer: The First Reported Case Wed, 22 Oct 2014 00:00:00 +0000 Hyperplastic polyps of the stomach are regarded as benign. However, in rare cases they may contain incipient primary carcinomas. To our knowledge, breast carcinoma metastatic to a gastric hyperplastic polyp has not yet been reported. We describe the case of a 69-year-old woman to whom a gastric polyp was endoscopically excised. The patient had previously undergone a right mastectomy for mixed, invasive ductal and lobular carcinoma 5 years earlier. Histological sections from the gastric lesion showed typical features of hyperplastic polyp with foci of poorly differentiated adenocarcinoma including signet ring cells infiltrating the lamina propria. The histologic findings were consistent with a primary gastric cancer. However, the carcinoma cells were immunopositive for estrogen and progesterone receptors and GATA3 and negative for CDX2, Hep Par 1, and MUC5AC. E-cadherin showed membranous reactivity in some of the carcinoma cells while in others it was negative. Accordingly, metastatic mixed, lobular and ductal breast carcinoma was diagnosed. We conclude that metastatic adenocarcinoma mimicking primary gastric cancer can be rarely encountered in hyperplastic gastric polyps. Gabriel M. Groisman, Roman Depsames, Baruch Ovadia, and Alona Meir Copyright © 2014 Gabriel M. Groisman et al. All rights reserved. Malignant Trigeminal Nerve Sheath Tumor and Anaplastic Astrocytoma Collision Tumor with High Proliferative Activity and Tumor Suppressor P53 Expression Wed, 15 Oct 2014 11:19:39 +0000 Background. The synchronous development of two primary brain tumors of distinct cell of origin in close proximity or in contact with each other is extremely rare. We present the first case of collision tumor with two histological distinct tumors. Case Presentation. A 54-year-old woman presented with progressive atypical left facial pain and numbness for 8 months. MRI of the brain showed left middle cranial fossa heterogeneous mass extending into the infratemporal fossa. At surgery, a distinct but intermingled intra- and extradural tumor was demonstrated which was completely removed through left orbitozygomatic-temporal craniotomy. Histopathological examination showed that the tumor had two distinct components: malignant nerve sheath tumor of the trigeminal nerve and temporal lobe anaplastic astrocytoma. Proliferative activity and expressed tumor protein 53 (TP53) gene mutations were demonstrated in both tumors. Conclusions. We describe the first case of malignant trigeminal nerve sheath tumor (MTNST) and anaplastic astrocytoma in collision and discuss the possible hypothesis of this rare occurrence. We propose that MTNST, with TP53 mutation, have participated in the formation of anaplastic astrocytoma, or vice versa. Maher Kurdi, Hosam Al-Ardati, and Saleh S. Baeesa Copyright © 2014 Maher Kurdi et al. All rights reserved. A Fatal Case of Primary Basaloid Squamous Cell Carcinoma in the Intrahepatic Bile Ducts Tue, 07 Oct 2014 06:23:41 +0000 Primary squamous cholangiocellular carcinomas are very rare. We describe a case of a 67-year-old man, who underwent chemotherapy and surgery for a right-sided liver tumor with an unusual presentation of metastasis to a lymph node in the left armpit. The patient was asymptomatic at the time of diagnosis but expired 20 months after surgery with epidural, lung, and spine metastasis. In addition to the unusual clinical presentation, the diagnosis of the liver tumor was that of a primary basaloid squamous cell carcinoma of the intrahepatic bile ducts, an entity with only one previous report in the literature. Johan Kirkegaard, Mie Grunnet, and Jane Preuss Hasselby Copyright © 2014 Johan Kirkegaard et al. All rights reserved. Langerhans Cell Histiocytosis of the Thyroid with Multiple Cervical Lymph Node Involvement Accompanying Metastatic Thyroid Papillary Carcinoma Thu, 02 Oct 2014 12:43:53 +0000 A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac arrest with respiratory causes in the early postoperative period. Langerhans cell histiocytosis is a rare primary condition which involves abnormal clonal proliferation of Langerhans cells in various tissues and organs. Thyroid involvement is infrequently seen. Although the etiology is unknown, genetic components may be linked to the disease. It is also associated with a family history of thyroid disease. Papillary thyroid carcinoma is the most common malignant epithelial tumor of the thyroid gland. Langerhans cell histiocytosis presenting with papillary thyroid carcinoma is rare. The privilege of our case is langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying cervical lymph node metastatic thyroid papillary carcinoma. A. Bahar Ceyran, Serkan Şenol, Barış Bayraktar, Şeyma Özkanlı, Z. Leyla Cinel, and Abdullah Aydın Copyright © 2014 A. Bahar Ceyran et al. All rights reserved. Low-Grade Malignant Triton Tumor of the Neck: A Case Report and Review of the Literature Mon, 22 Sep 2014 08:51:35 +0000 Rhabdomyoblastic differentiation in a malignant peripheral nerve sheath tumor (MPNST) is termed malignant triton tumor (MTT), a rare neoplasm that poses a diagnostic dilemma in the differential diagnosis of neck masses and portends poor prognosis. We report a sporadic case of MTT of the neck in a 23-year-old female. We present the pathological findings. Immunohistochemistry confirmed the neurogenic origin with S-100 expression and the rhabdomyoblastic differentiation with desmin and vimentin positivity. Radical surgical excision was done. After 4 years there were no signs of recurrence or distant metastasis. The clinical, microscopic, and long-term follow-up of this case are consistent with those of a low-grade malignancy. Taissir Omar, Hanaa Raslan, Sahar El Sheikh, Moataz Rizq, and Awatef Draz Copyright © 2014 Taissir Omar et al. All rights reserved. HER2-Positive Metaplastic Spindle Cell Carcinoma Associated with Synchronous Bilateral Apocrine Carcinoma of the Breast Thu, 18 Sep 2014 06:48:21 +0000 Apocrine carcinoma, which is strictly defined as over 90% of tumor cells showing apocrine differentiation, is a rare variant of breast cancer. Here we report an uncommon case in which apocrine carcinomas developed concurrently in both breasts; in addition, a sarcomatoid spindle cell lesion was coincident in the right breast. Both apocrine carcinomas were immunohistochemically negative for estrogen receptor (ER) and progesterone receptor (PgR), but diffusely positive for androgen receptor (AR), GCDFP-15, and HER2. The presence of intraductal components in bilateral carcinomas and the absence of lymph node metastasis suggested that they were more likely to be individual primary lesions rather than metastatic disease. The spindle cell lesion showed a relatively well-circumscribed nodule contiguous with the apocrine carcinoma. HER2 oncoprotein overexpression was observed not only in the apocrine carcinoma, but also in the spindle cell lesion. Since the spindle cell component was intimately admixed with apocrine carcinoma and had focal cytokeratin expression, we diagnosed it as metaplastic spindle cell carcinoma, which was originated from the apocrine carcinoma. To our knowledge, this is the first case report of a patient with synchronous bilateral apocrine carcinomas coinciding with metaplastic carcinoma. Katsumi Kito, Toshiharu Maeda, Keiko Ninomiya, Atsuro Sugita, Teiri Sagawa, Kinya Matsuoka, Kousei Kinoshita, Naoki Hyodo, Nagisa Morita, and Keizo Furuya Copyright © 2014 Katsumi Kito et al. All rights reserved. A Clinical and Pathological Variant of Acute Transplant Glomerulopathy Thu, 11 Sep 2014 07:44:22 +0000 Acute transplant glomerulopathy transplant glomerulopathy (TG) is a common cause of late renal allograft loss. We describe a unique case of a renal transplant recipient who developed rapid-onset nephrotic-range proteinuria and acute kidney injury secondary to C4d negative acute TG. Two courses of intravenous Rituximab resulted in significant improvement in proteinuria and allograft function. In the setting of acute nephrotic-range proteinuria postrenal allograft, both renal biopsy with electron microscopy and screening for de novo donor-specific antibody should be performed to distinguish atypical presentations of TG from other diagnoses. Miklos Z. Molnar, G. V. Ramesh Prasad, Darren A. Yuen, Serge Jothy, and Jeffrey S. Zaltzman Copyright © 2014 Miklos Z. Molnar et al. All rights reserved. Primary Squamous Cell Carcinoma of the Thyroid Diagnosed as Anaplastic Carcinoma: Failure in Fine-Needle Aspiration Cytology? Tue, 09 Sep 2014 08:38:28 +0000 A case of primary squamous-cell carcinoma (SCC) of the thyroid which had been initially diagnosed as an anaplastic carcinoma (ATC) is described: female, 73 years old, with a fast-growing cervical nodule on the left side and hoarseness for 3 months. Ultrasonography showed a 4.5 cm solid nodule. FNA was compatible with poorly differentiated carcinoma with immunoreactivity for AE1/AE3, EMA. Thyroidectomy was performed. Histopathological examination showed a nonencapsulated tumor. Immunohistochemistry disclosed positivity for AE1/AE3, p53,p63, and Ki67. The diagnosis was ATC. A second opinion reported tumor consisting of squamous cells, with intense inflammatory infiltrate both in tumor and in the adjacent thyroid, with final diagnosis of SCC, associated with Hashimoto thyroiditis. No other primary focus of SCC was found. Patient has shown a 48-month survival period. Clinically, primary SCCs of the thyroid and ATCs are similar. The distinction is often difficult particularly when based on the cytological analysis of FNA material. Fernanda Bolfi, Maria A. C. Domingues, Manuel Sobrinho-Simões, Paula Soares, Ricardo Celestino, Emanuel C. Castilho, Guareide Carelli, Norberto S. Paes, and Glaucia M. F. S. Mazeto Copyright © 2014 Fernanda Bolfi et al. All rights reserved. Primary Burkitt Lymphoma of the Fourth Ventricle in an Immunocompetent Young Patient Sun, 31 Aug 2014 07:53:26 +0000 Primary Burkitt lymphoma of the central nervous system (CNS) is rare, with only few cases reported in the literature. An 18 year-old immunocompetent male presented with multiple cranial nerves palsies and was found to have a mass predominantly in the 4th ventricle of the brain. Tumor was surgically removed and showed morphological and immunohistochemical features consistent with Burkitt lymphoma. The patient responded very well to anthracycline based chemotherapy with high dose methotrexate (HD MTX) and intrathecal (IT) chemotherapy delivered by Ommaya reservoir. Primary Burkitt lymphoma of the CNS is a rare entity that poses differential diagnostic challenge with other small round blue cell tumors. Abdulrahman Alabdulsalam, Syed Z. A. Zaidi, Imran Tailor, Yasser Orz, and Sadeq Al-Dandan Copyright © 2014 Abdulrahman Alabdulsalam et al. All rights reserved. Small Cell Carcinoma of the Uterine Cervix in a Pregnant Patient Diagnosed with Liquid Based Cytology and Cell Block Immunocytochemistry Thu, 28 Aug 2014 12:36:26 +0000 Definitive cytomorphologic diagnosis of small cell carcinoma of the uterine cervix is possible but can be challenging in routine cervicovaginal cancer screening specimens. Several small series of reported cases of cervical small cell carcinoma have shown this uncommon malignancy to represent fewer than 2% of all invasive cervical cancers. This tumor type is associated with poor prognosis and rapid disease progression and can develop to an advanced stage in the interval between screening visits. Only rare case reports of small cell carcinoma arising in gravid cervices are known. In the current case a 29-year-old, gravida 6, para 2, pregnant (10-week gestation) female presented with postcoital bleeding. A definitive diagnosis of small cell carcinoma of the cervix was made possible by liquid based Pap testing with ancillary cell block preparation allowing for immunocytochemical characterization of the lesional cell population. Mawuli F. Attipoe and Charles D. Sturgis Copyright © 2014 Mawuli F. Attipoe and Charles D. Sturgis. All rights reserved. Pleuropulmonary Blastoma: A Case Report and Review of the Literature Thu, 07 Aug 2014 10:34:26 +0000 The case of 38-month-old boy is being reported who was brought to the pediatrics clinic with fever, cough, hemoptysis, and breathing difficulty. Imaging studies revealed a right lower chest mass. Lobectomy and histopathological examination revealed it to be predominantly solid pleuropulmonary blastoma type II. It is a rare pediatric pleuropulmonary tumor with aggressive behavior and tendency to spread to the brain. The case is being presented to make the general histopathologist aware of this rare entity and to highlight to the pediatric physicians/surgeons, radiologists, and histopathologists the fact that lung cysts in infants and young children should be evaluated seriously and sampled thoroughly to diagnose cases of type I pleuropulmonary blastoma which will progress over time to type II or type III tumors. Also the siblings and first degree relatives of the patient should be screened for associated pulmonary and extrapulmonary benign and malignant conditions. Amjad Ali Khan, Ahmed Kamal El-Borai, and Mohammad Alnoaiji Copyright © 2014 Amjad Ali Khan et al. All rights reserved. Thyroid-Like Follicular Carcinoma of the Kidney in a Young Patient with History of Pediatric Acute Lymphoblastic Leukemia Tue, 15 Jul 2014 12:42:01 +0000 Thyroid-like follicular carcinoma of the kidney (TLFCK) is a rare histological variant of renal cell carcinoma not currently included in the World Health Organization classification of renal tumors. Only 24 previous cases of TLFCK have been reported to date. We report a case of TLFCK in a 19-year-old woman with history of pediatric acute lymphoblastic leukemia. This patient is the youngest with TLFCK to be reported to date and the first with history of lymphoblastic leukemia. The development of TLFCK in a young patient with history of lymphoblastic leukemia is interesting and suggests that genes involved in leukemogenesis may also be important for TLFCK pathogenesis. Recognition of TLFCK is important to distinguish it from other conditions that show thyroid-like features, as a misdiagnosis can result in adverse patient care. William W. Wu, Julia T. Chu, Ali Nael, Sherif A. Rezk, Stephen G. Romansky, and Lisa Shane Copyright © 2014 William W. Wu et al. All rights reserved. Primary Hairy Cell Leukemia/Lymphoma of the Breast: A Case Report and Review of the Literature Tue, 15 Jul 2014 09:56:06 +0000 Hairy cell leukemia/lymphoma (HCL) is a rare B-cell neoplasm primarily involving spleen, bone marrow, and blood. However, other sites of primary involvement do occur and can present a diagnostic and therapeutic challenge. We present an unusual case of HCL involving predominantly the breast that was diagnosed as an incidental finding during an elective reduction mammoplasty in an otherwise healthy asymptomatic woman. Bone marrow performed for staging revealed limited involvement by HCL. Notably, there was no splenomegaly and/or involvement of other extramedullary sites. The peripheral blood revealed minimal involvement detected by flow cytometry. Extensive immunohistochemical studies supported by positive BRAF V600E mutational status confirmed the diagnosis of HCL. The patient remains asymptomatic without treatment one year following the diagnosis. This is the first case of a well-documented HCL presenting primarily in the breast in an asymptomatic patient. We review the literature on extramedullary, extrasplenic involvement by HCL and discuss the diagnostic challenges as well as the utility of immunohistochemistry and molecular studies in the diagnosis of atypical presentations of HCL. Monika Pilichowska, Ahmad Shariftabrizi, Ian Mukand-Cerro, and Kenneth Miller Copyright © 2014 Monika Pilichowska et al. All rights reserved. Primary Renal Angiosarcoma with Extensive Necrosis: A Difficult Diagnosis Tue, 15 Jul 2014 08:56:41 +0000 Angiosarcoma of the kidney is an exceedingly rare and aggressive neoplasm. Very few cases have been reported in the English literature to date. We report a case of primary renal angiosarcoma with extensive necrosis and discuss its diagnostic difficulties. An 86-year-old male presented with a 12 cm necrotic renal mass and multiple pulmonary and hepatic nodules. A CT guided renal biopsy revealed extensive necrosis and few vascular channels lined by malignant endothelial cells. Diagnosis was given on a morphologic base and proven by an immunohistochemical study. Primary renal angiosarcoma should be included among the differential diagnosis of necrotic renal lesions. Sohail Qayyum, Jignesh G. Parikh, and Nadeem Zafar Copyright © 2014 Sohail Qayyum et al. All rights reserved. Intracranial Gliofibroma: A Case Report and Review of the Literature Sun, 13 Jul 2014 11:56:16 +0000 Gliofibroma is a rare tumor with biphasic morphology, commonly occurring in the first two decades of life. Currently, the tumor is not listed as a distinct entity in the current World Health Organization (WHO) classification of central nervous system tumors. As its biological behavior, histogenesis, and prognostic factors are still debated, the aim of this paper was to describe a case of a gliofibroma and to update the data about these lesions. Hence, we present here clinical symptoms, pathological findings, and evolution observed in a child with gliofibroma. A 10-year-old girl with seizures was referred for study. Neuroimaging showed a hemispheric hyperdense tumor with little peritumoral edema and no mass effect. The tumor was totally removed. Histologically, the tumor consisted of a mixture of glial cells and collagen-rich stroma. Immunohistochemical examination revealed positive staining for GFAP, CD 99, S100, and vimentin. EMA staining showed a paranuclear dot pattern in only few cells in isolated areas. These findings of a glial component with collagenous stroma were consistent with a desmoplastic glioma. Because of the rarity of this entity, we believe it is important to report every case in order to adequately analyze and categorize the tumor in the next WHO classification. Patricia Gargano, Graciela Zuccaro, and Fabiana Lubieniecki Copyright © 2014 Patricia Gargano et al. All rights reserved. Invasive Mucinous Adenocarcinoma Associated with Adjacent Sessile Serrated Lesion of the Appendix Vermiform: A Case Report Wed, 09 Jul 2014 10:25:29 +0000 Although the definition of sessile serrated lesion (SSL) of colon is controversial and the risk of progression to malignancy is also under investigation at present, SSL is generally described as a polyp characterized by a serrated architecture. It is estimated to represent a feature of a new cancerization pathway, coined “serrated neoplasia pathway,” particularly in right-sided colon adenocarcinomas. On the other hand, in appendix, the role of this pathway remains uncertain, probably because very few cases of appendiceal adenocarcinoma associated with SSL were reported, and furthermore, immunohistochemical examination was rarely carried out. We herein report an interesting case of invasive appendiceal mucinous adenocarcinoma exhibiting SSL, which was pathologically estimated as a potential precursor lesion, and performed representative immunohistochemistry for both the mucinous adenocarcinoma and SSL in the same specimen. To further elucidate the progression of the appendiceal carcinoma from SSL, both an adequate sectioning of the lesion and systematic immunohistochemical examination of a large number of appendiceal carcinoma cases containing adjacent SSL would be required. Osamu Kinoshita, Yasutoshi Murayama, Yoshiaki Kuriu, Masayoshi Nakanishi, Chohei Sakakura, and Eigo Otsuji Copyright © 2014 Osamu Kinoshita et al. All rights reserved. Axillary Metastasis from an Occult Tubal Serous Carcinoma in a Patient with Ipsilateral Breast Carcinoma: A Potential Diagnostic Pitfall Wed, 09 Jul 2014 00:00:00 +0000 Axillary nodal metastasis from a nonmammary neoplasia is much rarer than diseases associated with a primary breast carcinoma. However, this has to be considered in the differential diagnosis of nodal disease in patients with a history of breast cancer. Here, we report the case of a 73-year-old female with a past medical history of breast cancer, presenting with an ipsilateral axillary metastatic carcinoma. The immunohistochemical profile of the metastatic lesion was consistent with a high grade serous carcinoma. After undergoing a total abdominal hysterectomy and salpingo-oophorectomy, thorough pathological examination revealed two microscopic foci of serous carcinoma in the right fallopian tube, not detectable by preoperative magnetic resonance imaging. In this context, the poorly differentiated appearance of the metastatic tumor and positive staining for estrogen receptor, might lead to a misdiagnosis of metastatic breast carcinoma. As the therapeutic implications differ, it is important for the pathologist to critically assess axillary lymph node metastases, even in patients with a past history of ipsilateral breast carcinoma and no other known primary tumors. Chantal Atallah, Gulbeyaz Altinel, Lili Fu, Jocelyne Arseneau, and Atilla Omeroglu Copyright © 2014 Chantal Atallah et al. All rights reserved. Nodular Lymphocyte Predominant Hodgkin Lymphoma versus T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Diagnostic Challenge Mon, 07 Jul 2014 14:04:42 +0000 Lymphomas with overlapping histological features of two distinct entities cause difficulty in classification. Their classification is of particular significance when the two alternatives require different treatment modalities. We present a diagnostically challenging case of a nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) with features of T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL). Our patient is a 39-year-old woman who presented with painless subclavicular and axillary lymphadenopathy. The biopsied lymph node showed diffuse architectural effacement and scattered large neoplastic cells with large irregular nuclei and prominent nucleoli. These cells were positive for CD20 and Bcl-6 and negative for CD15, CD30, IgD, and Bcl-2. The background cells were predominantly T lymphocytes, whereas B cells were markedly depleted. The lymph node was interpreted as NLPHL, consistent with THRLBCL-like variant. NLPHL, especially THRLBC-like variant, and de novo THRLBCL are characterized by significant morphologic and immunophenotypic overlap. Our case demonstrates a rare predominance of background T-cells in NLPHL and emphasizes the importance of thorough evaluation of multiple morphologic and immunophenotypic features as an essential approach for arriving at the correct diagnosis. Anton V. Rets and Susan R. S. Gottesman Copyright © 2014 Anton V. Rets and Susan R. S. Gottesman. All rights reserved. Polypoid Adenomyoma of Endocervical Type Tue, 01 Jul 2014 11:46:20 +0000 We report herein a 53-year-old Japanese female case of polypoid adenomyoma of endocervical type. A sessile 16 mm sized cervical polyp, hard in consistency, was surgically removed. Histologically, the polypoid lesion was composed of smooth muscle bundles and scattered benign-looking endocervical glands. The mucin was diffusely alcianophilic. Immunohistochemically, some mucous glands were positive for MUC1 (CA15-3) and MUC5AC, and the other small glands were immunoreactive for MUC6. MUC2 and mucin characteristic of gastric gland mucous cells (M-GGMC-1 or HIK1083) were negative. Carcinoembryonic antigen was consistently expressed along the apical surface. Estrogen receptor was positive, while progesterone receptor was negative. Ki-67 labeling index was low. These findings were consistent with the endocervical nature of the mucin-producing columnar cells. This is the 18th case of adenomyoma of endocervical type reported in the English literature. Yuka Takeda, Daiju Araki, Toru Arase, and Yutaka Tsutsumi Copyright © 2014 Yuka Takeda et al. All rights reserved. Cellular Angiofibroma of the Prostate: A Rare Tumor in an Unusual Location Tue, 01 Jul 2014 09:17:44 +0000 We report the unusual occurrence of a cellular angiofibroma in prostatic tissue. In this case, a 84-year-old man presented in the emergency room with urinary retention. Ultrasound revealed an enlarged prostate, which was suggestive for benign prostatic hyperplasia. The patient was treated with a Millin retropubic prostatectomy. Macroscopically the prostate contained multiple circumscribed nodules. Microscopic examination of the tumor showed the appearance of cellular angiofibroma, consisting of bland spindle cells and prominent, hyalinized vessels. The diagnosis was supported by FISH, which revealed monoallelic loss of RB1/13q14 region, as seen in spindle cell lipoma, (extra-) mammary myofibroblastoma, and cellular angiofibroma. Cellular angiofibromas are rare, benign soft tissue tumours and were never reported in the prostatic gland. Inez Wyn, Maria Debiec-Rychter, Ben Van Cleynenbreugel, and Raf Sciot Copyright © 2014 Inez Wyn et al. All rights reserved. An Unusual Case of Systemic Inflammatory Myofibroblastic Tumor with Successful Treatment with ALK-Inhibitor Wed, 18 Jun 2014 07:40:05 +0000 Systemic inflammatory myofibroblastic tumor is an exceedingly rare entity. A 45-year-old Hispanic female presented with a 6-month history of left-sided thigh pain, low back pain, and generalized weakness. PET/CT scan revealed abnormal activity in the liver, adrenal gland, and pancreas. MRI of the abdomen demonstrated two 6-7 cm masses in the liver. MRI of the lumbar spine demonstrated lesions in the L2 to L4 spinous processes, paraspinal muscles, and subcutaneous tissues, as well as an 8 mm enhancing intradural lesion at T11, all thought to be metastatic disease. A biopsy of the liver showed portal tract expansion by a spindle cell proliferation rich in inflammation. Tumor cells showed immunoreactivity for smooth muscle actin and anaplastic lymphoma kinase 1 (ALK1). Tissue from the L5 vertebra showed a process histologically identical to that seen in the liver. FISH analysis of these lesions demonstrated an ALK (2p23) gene rearrangement. The patient was successfully treated with an ALK-inhibitor, Crizotinib, and is now in complete remission. We present the first reported case, to our knowledge, of inflammatory myofibroblastic tumor with systemic manifestations and ALK translocation. This case is a prime example of how personalized medicine has vastly improved patient care through the use of molecular-targeted therapy. Sanjivini V. Jacob, John D. Reith, Angerika Y. Kojima, William D. Williams, Chen Liu, and Lizette Vila Duckworth Copyright © 2014 Sanjivini V. Jacob et al. All rights reserved. Small Intestinal Amyloidosis: A Rare Cause of Diverticular Disease Thu, 05 Jun 2014 11:57:12 +0000 Systemic amyloidosis frequently involves the small intestine. However, its association with diverticular disease has been seldom reported to date. To draw attention to this rare but potentially harmful association, we herein present an additional case of small bowel diverticular disease associated with amyloidosis. Gabriel M. Groisman and Hector I. Cohen Copyright © 2014 Gabriel M. Groisman and Hector I. Cohen. All rights reserved. A Case of Unsuspected Peritoneal Mesothelioma Occurring with Colonic Adenocarcinoma Masquerading as Peritoneal Metastases Tue, 20 May 2014 11:45:42 +0000 We report a case of synchronous primary colonic adenocarcinoma and malignant mesothelioma. A 61-year-old male presented with a six-month history of fatigue and weight loss. An abdominal computed tomography (CT) scan showed a 5.8 cm partially obstructing mass in the cecum with ascites and peritoneal thickening. A biopsy of the large mass showed an adenocarcinoma. Because the patient was clinically thought to be a T4 colon carcinoma with peritoneal metastatic lesions (M1), prior to initiating chemotherapy, a debulking right hemicolectomy was performed. Resection of the colon and ileum revealed a T3N0 colonic mucinous adenocarcinoma and concurrent diffuse malignant peritoneal mesothelioma. Presenting synchronous colonic and peritoneal mesothelial primary malignancies are exceedingly rare but must be considered to prevent incorrect clinical staging. Wei Xie, Linda K. Green, Rishi A. Patel, and Syeling Lai Copyright © 2014 Wei Xie et al. All rights reserved. Cytopathologic, Histopathologic, and Immunohistochemical Features of Intrahepatic Clear Cell Bile Duct Adenoma: A Case Report and Review of the Literature Mon, 19 May 2014 00:00:00 +0000 Intrahepatic clear cell bile duct adenoma is extremely rare, with only 3 previous cases reported in the literature. The cause of cytoplasmic clearing in clear cell bile duct adenoma has not been previously investigated. Distinguishing clear cell bile duct adenoma from other clear cell tumors, particularly clear cell cholangiocarcinoma, can be challenging. Previous studies have shown loss of CD10 expression and focal CD56 expression in cholangiocarcinoma. Expressions of CD10 and CD56 have not been previously studied in clear cell bile duct adenoma. A 37-year-old morbidly obese woman was diagnosed with a 2.8 cm intrahepatic clear cell bile duct adenoma following segmental hepatic resection. Histochemical analysis of the tumor suggested the cause of cytoplasmic clearing in the neoplastic cells to be mucin and not glycogen or lipid. On immunohistochemical staining, the neoplastic cells demonstrated staining for CK7, CA 19-9, polyclonal CEA, CD10 (apical), CD56 (focal), and vimentin. Ki-67 highlighted less than 2% of tumor cell nuclei. This is the first report to study the etiology of cell clearing in clear cell bile duct adenoma. Expression of CD10 in clear cell bile duct adenoma may help distinguish clear cell bile duct adenoma from clear cell cholangiocarcinoma. William W. Wu, Mai Gu, and Di Lu Copyright © 2014 William W. Wu et al. All rights reserved. Intravascular Large B-Cell Lymphoma Presenting as Interstitial Lung Disease Thu, 15 May 2014 06:50:17 +0000 Intravascular large B-cell lymphoma (IVLBL) is a rare subtype of diffuse large B-cell lymphoma that resides in the lumen of blood vessels. Patients typically present with nonspecific findings, particularly bizarre neurologic symptoms, fever, and skin lesions. A woman presented with shortness of breath and a chest CT scan showed diffuse interstitial thickening and ground glass opacities suggestive of an interstitial lung disease. On physical exam she was noted to have splenomegaly. The patient died and at autopsy was found to have an IVLBL in her lungs as well as nearly all her organs that were sampled. Although rare, IVLBL should be included in the differential diagnosis of interstitial lung disease and this case underscores the importance of the continuation of autopsies. Elham Vali Khojeini and Joo Young Song Copyright © 2014 Elham Vali Khojeini and Joo Young Song. All rights reserved. Oral Floor and Gingival Metastasis of Cholangiocarcinoma: A Case Report and Review of the Literature Wed, 07 May 2014 13:05:18 +0000 The oral cavity is very unusual site of metastases even though wide spread metastatic disease may be present. The most common primary sites that metastasize to the oral cavity are lung, kidney, breast, and hepatocellular carcinoma. We present a rare case of a 77-year-old Caucasian female with metastasis from a cholangiocarcinoma to the oral floor contiguous with lingual gingival mucosa. The patient presented with left sided rib pain. A CT scan of the chest, abdomen, and pelvis showed multiple pulmonary nodules and a single dominant mass in the right lobe of the liver. This tumor was 6.5 cm with multiple satellite lesions surrounding it. The liver biopsy was diagnostic of a moderately to poorly differentiated adenocarcinoma, consistent with a primary cholangiocarcinoma. After undergoing one cycle of gemcitabine chemotherapy, the patient noticed an extremely rapidly growing mass involving her right lower gingiva and the entire right floor of her mouth. The biopsy of that mass also showed a moderately to poorly differentiated adenocarcinoma. The gingival tumor had a similar cytomorphology and immunophenotype as her cholangiocarcinoma. Therefore, an unusual site for metastatic cholangiocarcinoma was confirmed. Yukihiro Nakanishi, Bo Xu, and Charles LeVea Copyright © 2014 Yukihiro Nakanishi et al. All rights reserved. Clinical History and Colliquative Myocytolysis Are Keys to the Diagnosis of Shoshin Beriberi Wed, 07 May 2014 13:04:41 +0000 Cardiovascular beriberi presents as either the fulminant (Shoshin beriberi) or chronic form. Shoshin beriberi is a rare disease that may lead to a fatal outcome if the patient does not receive appropriate treatment. In the present report, we describe the case of a 66-year-old man presenting with leg edema and dyspnea at rest. Clinical presentations were nonalcoholic Shoshin beriberi and lactate accumulation; however, clinical improvement was observed after the administration of thiamine. His pretherapy thiamine level (2.1 μg/dL) was consistent with a diagnosis of beriberi. Based on the findings of the present case, we believe that a diagnosis can be made in patients with a clinical history that is consistent with that of Shoshin beriberi, combined with low thiamine levels, lactate accumulation, and colliquative myocytolysis. Learning Objective. Shoshin beriberi is often misdiagnosed because of its rarity; a detailed clinical history and characteristic myocardial histopathology changes may be useful for making a definite diagnosis. Toshiki Kuno, Hiroshi Nakamura, Yutaka Endo, Kohei Saito, Hiroyuki Yamazaki, Hiroyuki Motoda, Yohei Numasawa, Kazuhiko Shimizu, and Toshiyuki Takahashi Copyright © 2014 Toshiki Kuno et al. All rights reserved. A Pitfall in Transrectal Prostate Biopsy: Malakoplakia Evaluation of Two Cases Based on the Literature Review Tue, 29 Apr 2014 07:24:31 +0000 Malakoplakia is a rarely seen inflammatory condition that is considered to develop secondary to a chronic Escherichia coli infection. Although malakoplakia usually affects the genitourinary tract, it may also be observed in the colon, stomach, lungs, liver, bones, uterus, and skin. Malakoplakia of the genitourinary system usually involves the bladder, whereas it may also affect the prostate along with the bladder. Malakoplakia of the prostate is very rare, and it may be clinically mistaken for prostatic malignancies. Definitive diagnosis is only possible through histopathological examination. This study elaborates on two patients who presented to our hospital in 2013 with high PSA levels. The primary clinical consideration was prostate carcinoma. However, these two cases were diagnosed as malakoplakia based on the results of histopathological analysis of the transrectal prostate biopsy specimen. Dudu Solakoglu Kahraman, Sevil Sayhan, Gulden Diniz, Duygu Ayaz, Tugba Karadeniz, and Ertan Can Copyright © 2014 Dudu Solakoglu Kahraman et al. All rights reserved. A Rare Collision Tumor Composed of Follicular Lymphoma and Adenocarcinoma in the Ampulla of Vater: A Case Report Mon, 28 Apr 2014 06:44:10 +0000 The duodenum is infrequently affected by malignant lymphoma, and follicular lymphomas of the duodenum are rare histological subtypes. There are no reported cases of collision of follicular lymphoma and other tumors in the ampulla of Vater. A 57-year-old Japanese man presented with jaundice, and abdominal computed tomography revealed a tumor of the ampulla of Vater invading the pancreatic head with biliary dilatation and a thickened duodenal wall. The patient underwent subtotal stomach-preserving pancreaticoduodenectomy. Histopathology of the resected specimen revealed lymphoid follicular formations with small-to-medium-sized centrocyte-like cells and some centroblast-like cells. The atypical lymphoid cells were immunohistochemically positive for CD10, CD20, and CD79a but negative for CD5 and cyclin D1. BCL2 protein was highly expressed in the follicle centers. The diagnosis was duodenal follicular lymphoma, Grade 1. The follicular lymphoma, 40 mm in diameter, involved duodenal submucosa and regional lymph nodes without distant metastasis. This duodenal follicular lymphoma was partially overlapped by adenocarcinoma of the ampulla of Vater, measuring  mm, which involved the lower common bile duct, pancreas, and duodenum. We report the first case of a surgically treated collision tumor composed of a rare mass-forming follicular lymphoma and adenocarcinoma of the ampulla of Vater. Shioto Suzuki, Fumihiko Tanioka, Keisuke Inaba, Shingo Takatori, Hideto Ochiai, and Shohachi Suzuki Copyright © 2014 Shioto Suzuki et al. All rights reserved. Extranodal Marginal Zone Lymphoma Presenting within the Meckel Diverticulum as Diverticulitis: A Case Report Mon, 28 Apr 2014 06:43:17 +0000 Meckel diverticulum is the most common congenital defect of the gastrointestinal tract. It can be asymptomatic or mimic appendicitis and may be complicated by bleeding, diverticulitis, obstruction, and, rarely, neoplasia. We report the first case of extranodal marginal zone lymphoma occupying a Meckel diverticulum. A 44-year-old man with history of colonic diverticulitis presented to the emergency department for evaluation of acute abdominal pain. Radiography showed enteric obstruction, prompting diagnostic laparoscopy. Above the level of mid-ileum an intact Meckel diverticulum was identified. Microscopy showed extensive infiltration of sheets of small lymphocytes with abundant cytoplasm (monocytoid B-cells) prominently in submucosa and focally transmural involving serosal adipose tissue with multiple reactive germinal centers. The immunostains showed positivity for CD20, BCL-2, and CD43 (weak) and negativity for CD3, CD5, BCL-1, CD10, and BCL-6 in monocytoid B-cells. Fluorescence in situ hybridization studies revealed API2-MALT1 fusion signals consistent with t(11;18)(q21;q21), which confirmed the diagnosis of extranodal marginal zone lymphoma, also known as mucosa associated lymphoid tissue lymphoma. A. Nael, M. L. Wu, P. Nagesh Rao, S. Rezk, and X. Zhao Copyright © 2014 A. Nael et al. All rights reserved.