Case Reports in Pathology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Intraventricular Neurilemmoma (Schwannoma): Shall GFAP Immunostaining Be Regarded as a Histogenetical Tag or as a Mere Histomimetical Trait? Thu, 30 Jun 2016 12:33:16 +0000 http://www.hindawi.com/journals/cripa/2016/2494175/ Neurilemmomas are benign neoplasms presumedly derived from Schwann cells which rarely originate within the central nervous system. Moreover, their intraventricular location has been seldom noticed with less than 30 cases reported worldwide. Here, we add another case study to the record as well as the fifth one in Latin American population. A 16-year-old boy without significant past clinical data debuted with headache and progressive left eye blindness during six months. Neuroimaging scans showed a bulky, multiloculated, intraventricular tumour emerging from the posterior horn of the left lateral ventricle. Microscopically, the lesion put on view the classical schwannian histology: spindle cells arranged in both compact and loosely textured areas. Verocay bodies were not present but vessel hyalinisation, pericellular reticulin, and senescent atypia were observed. The immunoperoxidase reactions were also consistent with neurilemmal differentiation; however, glial fibrillary acidic protein expression was widespread and unexpectedly seen. Traditionally conceived as “nerve sheath tumours” the dual immunophenotype herein demonstrated points to a different histogenetical pathway other than sheer Schwann cell derivation. As previously advised by some authors, neoplastic transformation from a multipotent stem cell may explain the occasional finding of these tumours in unconventional intracranial compartments. Miguel Fdo. Salazar, Martha Lilia Tena Suck, Daniel Rembao Bojórquez, and Citlaltepetl Salinas Lara Copyright © 2016 Miguel Fdo. Salazar et al. All rights reserved. Intrabiliary Hepatic Metastasis of Colorectal Carcinoma Mimicking Primary Cholangiocarcinoma: A Case Report and Review of the Literature Sun, 26 Jun 2016 10:04:59 +0000 http://www.hindawi.com/journals/cripa/2016/4704781/ Intrabiliary metastasis from colorectal carcinoma (CRC) growing within or invading bile ducts is not a very common pattern. However, accurate diagnosis of metastatic lesions is very important for selection of adjuvant therapy and prognosis. We report a case of 71-year-old male who developed painless jaundice due to hepatobiliary obstruction. MRI demonstrated 1.4 cm intraductal mass at hepatic hilum with severe intrahepatic ductal dilation, consistent with cholangiocarcinoma. ERCP (endoscopic retrograde cholangiopancreatography) showed intraductal segmental biliary stricture. Biopsy from the lesion showed adenocarcinoma favoring primary cholangiocarcinoma due to the papillary morphology and location of the mass. His past history was significant for rectosigmoid carcinoma (pT1N0) ten years ago and liver resection for metastatic CRC four years ago. He subsequently underwent central hepatectomy with resection of common bile duct. Grossly, there was a 1.2 cm intraductal mass at the bifurcation of bile ducts with multiple nodules in liver parenchyma. Microscopic examination revealed intraductal carcinoma with papillary architecture colonizing bile duct epithelium with resultant dilation and tortuosity. Occasional liver parenchymal nodules show classical metastatic pattern resembling CRC. Because of two distinct morphologic patterns and patient’s past history, immunostains were performed. CK7 stained uninvolved bile duct epithelium with no staining in intrabiliary metastatic growth. CK20 and CDX2 were positive, thus confirming intrabiliary growth as metastatic growth from CRC. In summary, findings from our case indicate that intrabiliary growth of metastatic CRC can easily be overlooked with major duct involvement. Pathologic evaluation with use of immunohistochemical stains is very important to achieve correct diagnosis. Yimin Dong, Hitendra Patel, and Charmi Patel Copyright © 2016 Yimin Dong et al. All rights reserved. A Biphasic Pleural Tumor with Features of an Epithelioid and Small Cell Mesothelioma: Morphologic and Molecular Findings Wed, 15 Jun 2016 09:32:59 +0000 http://www.hindawi.com/journals/cripa/2016/1532424/ Malignant mesotheliomas are generally classified into epithelioid, sarcomatoid, desmoplastic, and biphasic types with rare reports of a small cell form. These small cell variants display some morphologic overlap with desmoplastic small round cell tumors (DSRCTs) which generally occur within the abdominal cavity of young males and are defined by a characteristic t(11;22)(p13;q12) translocation. However, there are rare reports of DSRCTs lacking this translocation. We present a 78-year-old man with a pleura-based biphasic neoplasm with features of both epithelioid mesothelioma and a small cell blastema-like neoplasm. The epithelioid portion showed IHC reactivity for pan cytokeratin, CK5/6, D2-40, and calretinin and the small cell portion marked with CD99, pan cytokeratin, WT1, FLI1, S100, CD200, MyoD1, and CD15. Fluorescence in situ hybridization testing for the t(11;22)(p13;q12) translocation disclosed loss of the EWSR1 gene in 94% of tumor cell nuclei, but there was no evidence of the classic translocation. Array based-comparative genomic hybridization (a-CGH) confirmed the tumor had numerous chromosome copy number losses, including 11p15.5-p11.12 and 22q12.1-q13.33, with loss of the EWSR1 and WT1 gene regions. Herein, we report novel complex CGH findings in a biphasic tumor and review the molecular genetic alterations in both mesothelioma and DSRCTs. Sarah Hackman, Richard D. Hammer, and Lester Layfield Copyright © 2016 Sarah Hackman et al. All rights reserved. Spinal Intradural, Extramedullary Ependymoma with Astrocytoma Component: A Case Report and Review of the Literature Sun, 29 May 2016 13:39:16 +0000 http://www.hindawi.com/journals/cripa/2016/3534791/ Ependymomas are common spinal lesions, with the vast majority arising in an intramedullary location. Several cases have been described in the literature of ependymomas in an intradural, extramedullary location. The authors present a case of a 56-year-old female who presented with several weeks of lower back pain and weakness. MRI revealed an intradural, extramedullary enhancing mass at L1-L2. The mass was successfully resected surgically. Pathologic evaluation revealed a low grade glioma with components of both ependymoma and pilocytic astrocytoma with MUTYH G382D mutation. Extramedullary ependymomas are very rare tumors. To the authors’ knowledge, this is the first case of ependymoma/astrocytoma collision tumors described in an extramedullary location. Gene M. Weinstein, Knarik Arkun, James Kryzanski, Michael Lanfranchi, Gaurav K. Gupta, and Harprit Bedi Copyright © 2016 Gene M. Weinstein et al. All rights reserved. Cellular Blue Nevus Diagnosed following Excision of Melanoma: A Challenge in Diagnosis Thu, 26 May 2016 07:51:12 +0000 http://www.hindawi.com/journals/cripa/2016/8107671/ A case of a 41-year-old woman with a history of nodular melanoma (NM), associated with an indurated dome-shaped blue-black nodule with a diameter of 1.2 cm in the gluteal region, is presented. Clinical diagnosis of the lesion, present from birth, was blue nevus. Recently, the nodule has been showing a mild enlargement and thus complete resection was performed. Histological analysis revealed a pigmented lesion with an expansive pattern of extension into the dermis and the subcutaneous adipose tissue. The lesion displayed an alveolar pattern as well as a pigmented dendritic cell pattern. The histology was consistent with cellular blue nevus (CBN); however, the history of NM which was excised one year earlier, as well as the clinical information about the slow growing lesion, included a differential diagnosis of CBN, borderline melanocytic tumor, and malignant blue nevus. Additional immunohistochemical (HMB-45, p16, and Ki-67) and molecular (BRAF V600E mutation) analyses were performed on both lesions: the CBN-like and the previously excised NM. Along with lesion history and histological analyses, p16 staining and BRAF were useful diagnostic tools for confirming the benign nature of CBN in this case. Nives Jonjić, Andrea Dekanić, Nedeljka Glavan, Larisa Prpić-Massari, and Blaženka Grahovac Copyright © 2016 Nives Jonjić et al. All rights reserved. Short-Spindled Cell Haemangioblastoma with CD34 Expression: New Histopathological Variant or Just a Stochastic Cytological Singularity? Wed, 25 May 2016 11:11:54 +0000 http://www.hindawi.com/journals/cripa/2016/6749590/ Haemangioblastomas are neoplasms of uncertain histogenesis with cellular and reticular variants advocated in current lore. Herein we describe an intriguing cerebellar specimen with unusual traits including spindle cell morphology and CD34 positivity. A thirty-nine-year old man had an infratentorial tumour discovered incidentally and resected three times. In all the instances, histopathological diagnosis was haemangioblastoma; nonetheless, he had neither physical stigmata nor family history of von Hippel-Lindau disease. By histology, the lesion was composed of areas of conventional stromal cells admixed with territories populated by short-spindled cells packed in lobules, sometimes giving the appearance of gomitoli. Immunoperoxidase-coupled reactions confirmed the expression of inhibin A, neuron-specific enolase (NSE), PS100, and CD57 but also revealed focal immunolabeling for CD34, CD99, and FXIIIa. This case highlights the potential phenotypical diversity that can be found within these neoplasms. Rather than uncertain histogenesis, it may in fact reflect multiple lines of differentiation—histomimesis—prone to adopt unusual morpho- and immunophenotypes in a subset of haemangioblastomas. Miguel Fdo. Salazar, Paola Andrea Escalante Abril, María Verónica Velasco Vales, Celene Martínez Ruiz, Erick Gómez Apo, and Laura G. Chávez Macías Copyright © 2016 Miguel Fdo. Salazar et al. All rights reserved. A Rare Case of Ovarian Filariasis in Abidjan Mon, 23 May 2016 13:44:38 +0000 http://www.hindawi.com/journals/cripa/2016/4075162/ Ovarian filariasis is an exceptional disease and displays a major diagnostic problem even in endemic areas. We reported the case of a 19-year-old patient who had ovarian cyst which was revealed by chronic pelvic pain. The histological examination of oophorectomy specimen led to the Wuchereria bancrofti filariasis of the ovary. The anatomopathologic examination is required for the diagnosis of this disease. Doukouré Brahima, Abouna Alain Didier, Dou Gonat Serge Pacôme, Aman Nguiessan Alphonse, Koffi Abdoul, and Diomandé Mohenou Isidore Jean-Marie Copyright © 2016 Doukouré Brahima et al. All rights reserved. Diffuse Neuroendocrine Cell Hyperplasia: Report of Two Cases Tue, 17 May 2016 06:44:44 +0000 http://www.hindawi.com/journals/cripa/2016/3419725/ Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder characterised by a proliferation of neuroendocrine cells within the lung. It is believed that a minority of the patients with DIPNECH can develop carcinoid tumors. Here, we report two new cases of DIPNECH with coexisting carcinoid tumors. Cevriye Cansız Ersöz, Ayten Kayı Cangır, and Serpil Dizbay Sak Copyright © 2016 Cevriye Cansız Ersöz et al. All rights reserved. An Unusual Case of Extranodal Diffuse Large B-Cell Lymphoma Infiltrating Skeletal Muscle: A Case Report and Review of the Literature Tue, 10 May 2016 06:15:57 +0000 http://www.hindawi.com/journals/cripa/2016/9104839/ Diffuse large B-cell lymphoma is extranodal in approximately 40% of cases, arising in nearly any organ system. DLBCL involvement of soft tissue and in particular skeletal muscle is extremely rare, comprising less than 1% of all extranodal non-Hodgkin lymphomas (NHL). We report a case of a 79-year-old man that presented with a DLBCL of the left triceps. In particular, we describe an unusual histologic appearance of pseudoglandular structures, resembling adenocarcinoma. We performed a review of lymphoma cases involving skeletal muscle diagnosed at our institution over the past 15 years as well as thorough PubMed review of the literature. We discuss the features of lymphoma involving skeletal muscle as it pertains to clinical characteristics, histologic subtype, tumor localization, diagnostic studies, therapy, and outcome. Finally, we highlight the diagnostic difficulties that can present in these rare and often challenging cases. Joseph Hatem and Agata M. Bogusz Copyright © 2016 Joseph Hatem and Agata M. Bogusz. All rights reserved. Posterior Mediastinal Adenomatoid Tumor: A Case Report and Review of the Literature Sun, 08 May 2016 14:17:54 +0000 http://www.hindawi.com/journals/cripa/2016/6898526/ Adenomatoid tumor is an uncommon benign neoplasm of mesothelial differentiation that distinctively arises in and around the genital organs. In rare instances, it has been described in extragenital locations. There have been only two reports documenting its occurrence in the anterior mediastinum, and no reports documenting its occurrence in the posterior mediastinum. We report the first case of posterior mediastinal adenomatoid tumor. A 37-year-old Caucasian woman presented with symptoms of bronchitis. Imaging studies identified a 2.0 cm posterior mediastinal mass abutting the T9 vertebral body, clinically and radiologically most consistent with schwannoma. Histologic sections revealed a lesion composed of epithelioid cells arranged in cords and luminal profiles embedded in a fibrotic to loose stroma and surrounded by a fibrous pseudocapsule. Lesional cells showed vacuolated eosinophilic cytoplasm and peripherally displaced nuclei with prominent nucleoli. There was focal cytologic atypia but no mitotic figures or necrosis was identified. The lesional cells expressed cytokeratin, calretinin, and nuclear WT1 but were negative for PAX8, TTF1, p53, chromogranin, CD31, and CD34, and Ki67 showed <2% proliferation rate, diagnostic of adenomatoid tumor. Three years after resection, the patient is in good health without tumor recurrence. Thus, our encounter effectively expands the differential diagnosis of posterior mediastinal neoplastic entities. Vishwas Parekh, Thomas Winokur, Robert J. Cerfolio, and Todd M. Stevens Copyright © 2016 Vishwas Parekh et al. All rights reserved. Paragonimus kellicotti: A Lung Infection in Our Own Backyard Sun, 24 Apr 2016 11:23:08 +0000 http://www.hindawi.com/journals/cripa/2016/2107372/ Paragonimiasis is an infection caused by the lung fluke of the genus Paragonimus. Within the United States, paragonimiasis has been commonly diagnosed in Southeast Asian immigrants infected with the Asian lung fluke Paragonimus westermani. Infections from the North American lung fluke, Paragonimus kellicotti, have been rare, although more infections have been seen in people in the Midwestern United States. A 29-year-old male with a history of pleomorphic xanthoastrocytoma presented with hemoptysis. A CT scan showed a mass in the left upper lung lobe. A biopsy showed eosinophils and parasite eggs, some with a recognizable operculum. Further investigation revealed that he takes canoe trips on rivers within Missouri and would eat crayfish caught from these rivers. A blood sample was confirmed positive for Paragonimiasis serologically at the Center for Disease Control. Paragonimus kellicotti is found in rivers within the Mississippi basin. Infection occurs by consuming uncooked or undercooked crawfish. Microscopic identification of parasite eggs has been the gold standard. Serologic tests have been developed to aid in the diagnosis. Patients typically present with fever and hemoptysis. Common CT findings include pleural effusion, a mass, and lymphadenopathy. Awareness of P. kellicotti is important to guide appropriate diagnostic testing and ensuring proper treatment. Eric Johannesen and Van Nguyen Copyright © 2016 Eric Johannesen and Van Nguyen. All rights reserved. From the Gut to the Liver: Another Organ to Watch in FAP Patients Thu, 24 Mar 2016 11:13:07 +0000 http://www.hindawi.com/journals/cripa/2016/1738696/ We report a rare association of hepatocellular carcinoma with familial adenomatous polyposis in a young patient and its clinical significance. A 28-year-old female with a past medical history of familial adenomatous polyposis (FAP) and subsequent colonic adenocarcinoma underwent total colectomy. She later presented with intermittent right upper quadrant pain and nausea of four months’ duration. MRI of the abdomen revealed multiple liver lesions, the largest 8.5 cm in diameter, with radiologic features suggestive of hepatocellular carcinoma. A CT-guided liver biopsy demonstrated well-differentiated HCC which was confirmed by immunohistochemistry. In patients with a history of FAP, a heightened awareness of the possibility of concurrent or subsequent HCC is warranted. Spencer Paulson, Charmi Patel, and Hitendra Patel Copyright © 2016 Spencer Paulson et al. All rights reserved. Endosalpingiosis of Axillary Lymph Nodes: A Rare Histopathologic Pitfall with Clinical Relevance for Breast Cancer Staging Mon, 21 Mar 2016 14:35:30 +0000 http://www.hindawi.com/journals/cripa/2016/2856358/ Establishment of accurate axillary lymph node status is of essential importance in determining both prognosis and the potential need for adjuvant therapy in patients with invasive breast cancer. Axillary lymph node heterotopias can in some cases result in overdiagnosis of metastatic disease. Nodal endosalpingiosis is perhaps the least commonly reported type of axially lymph node heterotopia. We herein illustrate a case in which second opinion pathologic interpretation combined with ancillary immunohistochemical studies allowed for a specific diagnosis of axillary nodal müllerian-type inclusions, confirming ypN0 staging and resulting in appropriate disease management and prognostication. Laila Nomani, Benjamin C. Calhoun, Charles V. Biscotti, Stephen R. Grobmyer, and Charles D. Sturgis Copyright © 2016 Laila Nomani et al. All rights reserved. Localized Castleman’s Disease in the Breast in a Young Woman Thu, 17 Mar 2016 07:25:21 +0000 http://www.hindawi.com/journals/cripa/2016/8413987/ Castleman’s disease (CD) is a rare lymphoproliferative disorder of unknown etiology. It typically occurs in adulthood but it may also develop in childhood. Clinically, this disease may be classified as localized (unicentric) or systemic (multicentric). Six cases of breast CD have been described in the literature, and all have been reported in adults. Herein we describe the case of a 15-year-old female who presented with a slow-growing tumor in the right breast. The tumor was excised and histopathological examination demonstrated hyaline vascular variant CD. After two years of follow-up, the patient was asymptomatic without evidence of cervical or axillary lymphadenopathy. Rafael Parra-Medina, José Ismael Guio, and Patricia López-Correa Copyright © 2016 Rafael Parra-Medina et al. All rights reserved. Diagnostic Difficulties in Pathological Laboratories in Developing Countries: A Case Report of Differentiated Squamous Cell Carcinoma in a Young Togolese Woman Tue, 15 Mar 2016 09:40:08 +0000 http://www.hindawi.com/journals/cripa/2016/3727484/ We report a case of poorly differentiated squamous cell carcinoma of the vulva induced by human papillomavirus in a 23-year-old woman, in whom we experienced diagnostic difficulties. Tchin Darré, Lantam Sonhaye, Mazamaesso Tchaou, Abdoul-Samadou Aboubakari, Bingo K. M’Bortche, Kofi Amégbor, and Gado Napo-Koura Copyright © 2016 Tchin Darré et al. All rights reserved. Urinary Bladder Adenocarcinoma Metastatic to the Abdominal Wall: Report of a Case with Cytohistologic Correlation Tue, 23 Feb 2016 12:54:07 +0000 http://www.hindawi.com/journals/cripa/2016/8608412/ We report a case of adenocarcinoma metastatic to the abdominal wall in a 71-year-old man with a history of primary bladder adenocarcinoma. CT-guided core biopsy was performed; imprints and histologic sections showed malignant glands lined by tumor cells with hyperchromatic nuclei and prominent nucleoli, infiltrating through skeletal muscle. Immunohistochemistry revealed positivity for CK7, membranous/cytoplasmic β-catenin, caudal-type homeobox transcription factor 2 (CDX2), and α-methylacyl coenzyme A racemase and negativity for CK20, p63, prostate-specific antigen (PSA), and prostate-specific acid phosphatase (PSAP). These findings were interpreted as metastatic adenocarcinoma, consistent with bladder primary. Primary bladder adenocarcinoma is a rare malignancy arising within glandular metaplasia and is associated with cystitis cystica and cystitis glandularis. Predisposing factors include bladder exstrophy, schistosomiasis, and other causes of chronic bladder irritation. This tumor is divided into intestinal, clear cell, and signet ring cell subtypes. Treatment involves radical cystectomy with pelvic lymph node dissection, and prognosis is unfavorable. Primary bladder adenocarcinoma should be differentiated from urachal adenocarcinoma, which arises from urachal remnants near the bladder dome, and secondary adenocarcinoma, or vesical involvement by adenocarcinoma from a different primary. CK7, CK20, CDX2, thrombomodulin, and β-catenin can help distinguish primary bladder adenocarcinoma from colonic adenocarcinoma; PSA and PSAP can help distinguish primary bladder adenocarcinoma from prostate adenocarcinoma. Vikas Nath and Mithra Baliga Copyright © 2016 Vikas Nath and Mithra Baliga. All rights reserved. Foregut Duplication Cyst of the Stomach: A Case Report and Review of the Literature Sun, 21 Feb 2016 11:37:17 +0000 http://www.hindawi.com/journals/cripa/2016/7318256/ Duplication cyst of the stomach is a rare congenital malformation, typically diagnosed in the first year of life. In most adult cases the cyst remains asymptomatic, but patients may present with abdominal symptoms including epigastric discomfort or pain. We present a case of a 65-year-old male with an asymptomatic gastric tumor diagnosed incidentally during initial workup of his esophageal adenocarcinoma. Computed tomography revealed a low density soft tissue tumor near the gastroesophageal junction. Endoscopic ultrasonography demonstrated a cystic lesion as a hypoechoic round mass with well-defined borders. Following complete laparoscopic resection, microscopic review revealed a cyst lined with respiratory pseudostratified ciliated columnar epithelium and layers of smooth muscle with an outermost thin fibrous capsule consistent with a foregut duplication cyst. Y. Tjendra, K. Lyapichev, J. Henderson, and C. P. Rojas Copyright © 2016 Y. Tjendra et al. All rights reserved. Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature Tue, 26 Jan 2016 11:11:13 +0000 http://www.hindawi.com/journals/cripa/2016/1936421/ The Perivascular Epithelioid Cell tumours (PEComas) are rare mesenchymal neoplasms recognized as entity by the World Health Organization. The tumour cells have an uncertain origin and are characterized by distinctive histological and immunohistochemical features. We report a case of PEComa occurring as intraorbital lesion in a 47-year-old man. We found only two other cases described in the literature and we considered all three cases together in order of histology, immunohistochemistry, and clinical outcome. We found a strict histological overlapping and quite similar immunohistological results. All three cases showed a favourable clinical course probably related to small size of tumours (<5 cm), low mitotic rate (<2 mitoses in 50 HPF), and absence of necrosis. Idania Lubo, Ileana Fermín, Olindo Massarelli, Roberta Gobbi, and Paolo Cossu Rocca Copyright © 2016 Idania Lubo et al. All rights reserved. Pulmonary Empty Spaces: Silicone Embolism—A Decade of Increased Incidence and Its Histological Diagnosis Thu, 21 Jan 2016 14:28:33 +0000 http://www.hindawi.com/journals/cripa/2016/3741291/ Pulmonary embolism (PE) is a critical complication related to multiple disorders and different medical or cosmetic procedures. This case report presents two patients who were admitted for respiratory symptoms in the setting of previously receiving silicone injections for cosmetic purposes and were diagnosed with silicone pulmonary embolism. The relevance of including questions about all cosmetic procedures as a part of a medical history is highlighted, in particular about silicone injections. The diagnosis is confirmed by histological means. Additionally, our review showed the change of most common sites of silicone injections and a significant increase in cosmetic procedures causing silicone embolism during the past twelve years. Kirill Lyapichev, Felix Manuel Chinea, Julio Poveda, Jeniffer Pereda, Pablo A. Bejarano, and Monica T. Garcia-Buitrago Copyright © 2016 Kirill Lyapichev et al. All rights reserved. Fibrolipoma of the Buccal Mucosa: A Case Report and Review of the Literature Sun, 17 Jan 2016 17:32:51 +0000 http://www.hindawi.com/journals/cripa/2016/5060964/ Lipomas are common benign soft tissue neoplasms derived from mature adipose tissue. However, they rarely arise in the oral cavity. Fibrolipoma is a histological variant of lipoma that mainly affects the buccal mucosa and causes functional and cosmetic issues. This article describes the case of a 71-year-old male with a fibrolipoma of the left buccal mucosa and a review of previous articles about fibrolipoma. Masayasu Iwase, Naotaka Saida, and Yoko Tanaka Copyright © 2016 Masayasu Iwase et al. All rights reserved. Biliary Adenofibroma with Invasive Carcinoma: Case Report and Review of the Literature Wed, 13 Jan 2016 11:32:44 +0000 http://www.hindawi.com/journals/cripa/2016/8068513/ We report a case of biliary adenofibroma with an invasive carcinoma in a 71-year-old female who presented with bilateral upper abdominal pain. Imaging revealed a 6.3 cm heterogeneously enhancing mass in the left lateral segment of the liver. Histologically, the adenofibroma showed the characteristic components as previously described of biliary adenofibromata, namely, cystic and tubular structures lined by cuboidal to low columnar biliary type epithelium and a dense fibrous stroma composed of spindled cells. Intimately admixed with the adenofibroma was a distinct tumor composed of malignant clear cells which demonstrated stromal and vascular invasion. Although mitotic figures were inconspicuous, Ki67 was brisk and p53 demonstrated 25–50 positivity. Sections also showed a von Meyenberg complex located adjacent to the tumor. This case expands the understanding of this rare tumor and proves two important assertions from previous case reports. First, the presence of an associated von Meyenberg complex with similar morphology and immunohistochemical staining pattern suggests that biliary adenofibromata and von Meyenberg complexes may share related histogenesis. Second, biliary adenofibromata harbor malignant potential and may show malignant transformation. Furthermore, this case highlights the need for these rare tumors to be followed aggressively, as their biological behavior is poorly understood. Anjali Godambe, Elizabeth M. Brunt, Keith H. Fulling, and Taher Reza Kermanshahi Copyright © 2016 Anjali Godambe et al. All rights reserved. Trichohepatoenteric Syndrome or Syndromic Diarrhea—Report of Three Members in a Family, First Report from Iran Wed, 06 Jan 2016 08:34:44 +0000 http://www.hindawi.com/journals/cripa/2016/9684910/ Introduction. Intractable diarrhea of infancy (IDI) includes several types of early onset diarrhea; one of the rare etiologies is trichohepatoenteric (THE) syndrome, also known as syndromic diarrhea (SD) which was primarily described by Stankler et al. Hereby we report a family with several affected members which to our knowledge is the first case report from Iran. Report of Cases. A three-year-old boy referred with short stature, poor weight gain, and intermittent steatotic diarrhea to our center. He was born to healthy, relative parents (cousins). He did not gain any weight after four months of age and began having intermittent steatotic diarrhea, abdominal distension, and fever. He was hospitalized several times. Two other children in the family also showed somewhat similar symptoms. Two sweat tests were negative for cystic fibrosis. Workup for Celiac disease was performed several times which was negative; however, gluten-free diet was tried several times which was not effective. Workup for Hirschsprung’s disease was performed but colon was ganglionic. Evidence of liver involvement was approved by elevated liver enzymes and coarse echo of liver on sonography. Discussion. Trichoenterohepatic syndrome should be put in mind in cases of intractable diarrhea presenting in a family with several affected members. Early diagnosis would save patients from unnecessary workups. F. E. Mahjoub, F. Imanzadeh, S. Mahdavi Izadi, and A. Nahali Moghaddam Copyright © 2016 F. E. Mahjoub et al. All rights reserved. Metastatic Chordoma: A Diagnostic Challenge on Fine Needle Aspiration Sun, 03 Jan 2016 14:21:06 +0000 http://www.hindawi.com/journals/cripa/2016/2187290/ Chordomas are primary low grade malignant tumors of bone that usually arise within both ends of axial skeleton. The Notochord is a midline, ectoderm-derived structure that defines the phylum of chordates. Chordomas may pose difficult diagnostic challenges when encountered in secondary locations, such as lungs or other parenchymatous organs. We report the cytologic findings of a metastatic chordoma sampled through CT-scan guided fine needle aspiration (FNA) of lower lobe lung nodule in a 54-year-old man diagnosed with recurrent chordoma involving the lumber spine and paraspinal region. Ghassan Tranesh and Aziza Nassar Copyright © 2016 Ghassan Tranesh and Aziza Nassar. All rights reserved. Comment on “Retained Placenta Accreta Mimicking Choriocarcinoma” Sun, 03 Jan 2016 14:20:39 +0000 http://www.hindawi.com/journals/cripa/2016/9819584/ Mehmet Aral Atalay and Bilge Cetinkaya Demir Copyright © 2016 Mehmet Aral Atalay and Bilge Cetinkaya Demir. All rights reserved. Metachronous Occurrence of Granular Cell Tumor in Breast Skin and Scalp: Diagnostic Challenging Differentiating Benign from Malignant and a Literature Review Sun, 03 Jan 2016 12:34:54 +0000 http://www.hindawi.com/journals/cripa/2016/8043183/ Granular cell tumor (GCT) is a Schwann cell related benign neoplasm of soft tissue. GCT is an uncommon entity that occurs in a wide variety of body sites, but it is generally presented in the skin, oral cavity, superficial soft tissue, and respiratory and digestive tracts. Most of the GCTs are benign but clinically and radiologically these may mimic malignancy. Histopathological diagnosis is gold standard for establishing the true nature of the lesion. GCT is most commonly solitary but in about 10% of cases can be multifocal, usually involving various skin and soft tissue sites versus involving various internal sites. Therefore, these can involve skin and soft tissue or submucosa and viscera. GCT is usually benign; however, local recurrence is common due to incomplete removal. Malignant cases are rarely reported in 1-2% of cases. In this study, we report clinical and histopathological findings of a 36-year-old woman with metachronous GCT in breast and scalp. The clinical features raise the question of whether these are metachronous benign GCTs or whether this is establishment of malignant behavior. The aim of this report is to present the histopathological and clinical features of GCT and the diagnostic challenge of differentiating benign from malignant GCT. Hampar Akkaya, Havva Serap Toru, Ebru Sebnem Ayva, Zulfikar Karabulut, and Cicek Durusoy Copyright © 2016 Hampar Akkaya et al. All rights reserved. Unexpected Malignant Diagnosis in Colonic Biopsies: Malignant Transformation of Ovarian Mature Teratomas—Two Case Reports and Review of the Literature Thu, 31 Dec 2015 08:24:05 +0000 http://www.hindawi.com/journals/cripa/2015/905462/ Colorectal adenocarcinoma is the second cause of cancer-related deaths in the United States. The occurrence of squamous cell carcinoma in the colorectum is extremely unusual. Malignant transformation from mature cystic teratoma of the ovary is a rare event. The most common transformation is squamous cell carcinoma, followed by adenocarcinoma. It occurs more often in elderly patients, who usually present with advance disease. We report two unusual cases of postmenopausal women diagnosed with squamous cell carcinoma in colon biopsies. After surgical resections, the carcinoma was proven to be the result of malignant transformation of ovarian mature cystic teratomas. Since squamous cell carcinoma of the colorectum is extremely rare, the presence of squamous cell carcinoma in a colonic biopsy in a female patient should alert the clinicians to other possible primary sites, as seen in these cases. Claudia P. Rojas, Parvin Ganjei-Azar, and Monica T. Garcia-Buitrago Copyright © 2015 Claudia P. Rojas et al. All rights reserved. Isolated Ocular Manifestation of Relapsed Chronic Myelogenous Leukemia Presenting as Myeloid Blast Crisis in a Patient on Imatinib Therapy: A Case Report and Review of the Literature Thu, 24 Dec 2015 13:28:06 +0000 http://www.hindawi.com/journals/cripa/2015/380451/ Blast phase in chronic myelogenous leukemia (CML) has rarely been reported to involve extramedullary sites like skin, lymph nodes, and central nervous system. Clinical history, characteristic hematologic findings (elevated leukocyte counts, myelocytic predominance, and basophilia), and Philadelphia chromosome are of high diagnostic significance especially in isolated extramedullary presentations. We describe a unique case of CML relapse with blast phase involving the eye. A 66-year-old man with a known diagnosis of CML on imatinib and in molecular remission for 3 years presented with a painful blind eye. Histologic examination revealed diffuse involvement of choroid, iris, vitreous humor, and the optic nerve by blast cells. The blasts expressed CD34, aberrant TdT, and a myeloid phenotype (CD13, CD33, and CD117). Fluorescence in situ hybridization (FISH) of vitreous fluid detected BCR-ABL1 gene rearrangement. Additionally, trisomy 8 and gains of 9 and 22 were seen which were not present in the initial diagnostic marrow study 3 years ago. At relapse, the bone marrow, peripheral blood, and the cerebrospinal fluid were not involved by CML. Patient received induction chemotherapy and single dose prophylactic intrathecal methotrexate and was maintained on antityrosine kinase therapy and eventually underwent allogenic stem cell transplantation. Rohit Gulati, Yaser Alkhatib, Vijayalakshmi Donthireddy, Michelle Madden Felicella, Madhu P. Menon, and Kedar V. Inamdar Copyright © 2015 Rohit Gulati et al. All rights reserved. Primary Invasive Squamous Cell Carcinoma of the Nipple Mon, 21 Dec 2015 11:37:40 +0000 http://www.hindawi.com/journals/cripa/2015/327487/ Squamous cell carcinoma is one of the most common cutaneous cancers; however, primary squamous cell carcinoma of the nipple is extremely rare. Among the few reported cases, the majority have occurred in older women with rare cases seen in younger women and male patients. Our patient presented with an exophytic mass of the right nipple while pregnant. A superficial biopsy was reviewed at an outside institution and then at our institution and diagnosed as squamous papilloma and then as hyperkeratosis of the nipple, respectively. The subsequent excisional biopsy revealed multiple nests of tumor cells extending into the dermis with associated chronic inflammatory infiltrate, and the lesion was diagnosed as a primary invasive squamous cell carcinoma of the nipple. Following that, a wide local excision of the excision site and sampling of the regional lymph nodes were negative for carcinoma. Due to the rarity of this diagnosis, it is not known whether prognosis and response to therapy differ from cutaneous squamous cell carcinoma at other sites. Therefore, risk stratification and therapy have been based on those for cutaneous squamous cell carcinoma. Avani A. Pendse and Siobhan M. O’Connor Copyright © 2015 Avani A. Pendse and Siobhan M. O’Connor. All rights reserved. The Potential Malignancy of a Solitary Fibrous Tumour of the Lung Thu, 17 Dec 2015 07:21:32 +0000 http://www.hindawi.com/journals/cripa/2015/209490/ Solitary fibrous tumours (SFTs) are rare neoplasms that in the majority of cases are benign. We present the case of a 52-year-old male, with a 23-year history of a slow growing pleural mass, presenting to our department with worsening dyspnoea and localised chest discomfort. The purpose of this case report is to highlight the potential malignancy of a solitary fibrous tumour of the lung along with the key features in diagnosis and management. Rajeev Shukla, Davide Patrini, Elaine Borg, David Lawrence, Martin Hayward, and Nikolaos Panagiotopoulos Copyright © 2015 Rajeev Shukla et al. All rights reserved. Adipocyte-Like Differentiation in a Posttreatment Embryonal Rhabdomyosarcoma Thu, 10 Dec 2015 06:46:30 +0000 http://www.hindawi.com/journals/cripa/2015/406739/ We describe a 16-year-old boy with rhabdomyosarcoma, consistent with embryonal subtype, of the lower extremity who received systemic neoadjuvant chemotherapy and subsequent excision. Microscopic sections of the postchemotherapy excision demonstrated diffuse, prominent, and immature adipocyte-like differentiation, in addition to skeletal muscle differentiation. Adipocyte-like differentiation was confirmed by a combination of positive Oil Red O and adipophilin immunohistochemical staining. To our knowledge, this represents the first report of an unusual phenomenon of differentiation of a soft tissue rhabdomyosarcoma into adipocyte-like cells after chemotherapy. Dana Balitzer, Timothy H. McCalmont, and Andrew E. Horvai Copyright © 2015 Dana Balitzer et al. All rights reserved.