Case Reports in Pathology The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Hemangioblastoma in the Lung: Metastatic or Primary Lesions? Sun, 14 Dec 2014 00:10:42 +0000 Hemangioblastoma primarily occurs in the CNS, most commonly in the posterior fossa. Extracranial locations are less common, and metastatic tumor involving the lung is exceedingly rare with only three cases previously reported. Two were autopsy studies in patients who died of complications of the CNS hemangioblastomas in 1943 and 1981, and the third was mentioned in a case report addendum providing follow-up information on hepatic hemangioblastoma in 1991. We report a case of a 48-year-old man who presented with multiple lung nodules treated by surgical excision. Pathological study revealed features classic for hemangioblastoma. The patient had a remote history of hemangioblastomas having been excised from the posterior fossa 7 and 20 years previously. This report details a fourth case of metastatic pulmonary hemangioblastoma. It is the first report on surgically resected hemangioblastomas from the lung of a living patient with histological and immunohistochemical characterization. Li Lu, Peter A. Drew, and Anthony T. Yachnis Copyright © 2014 Li Lu et al. All rights reserved. Spinal Cord Glioneuronal Tumor with Rosetted Neuropil-Like Islands in Pediatric Age Group Wed, 10 Dec 2014 08:17:43 +0000 Glioneuronal neoplasms are rare tumors. Recently, an unusual glioneuronal tumor histologically showing neuropil-like islands has been described. Here, we present such a tumor originating from spinal cord of a 14-year-old girl, who has scoliosis and urinary incontinence. Microscopically, the glial component was chiefly fibrillary astrocytic, punctuated by neuropil-like islands. Immunohistochemically, glial tissue was GFAP positive, and neuropil-like areas and big neurons were synaptophysin reactive. For astrocytic component Ki-67 proliferation index was 1% and p53 was immunonegative. This case is unique in that in the literature it is the second reported case in pediatric age group that is located at spinal cord. Nil Comunoglu, Ozgur Kilickesmez, and Buge Oz Copyright © 2014 Nil Comunoglu et al. All rights reserved. Pleomorphic Invasive Ductal Carcinoma of the Breast in a Patient with Huntington’s Disease Tue, 09 Dec 2014 06:35:58 +0000 A pleomorphic invasive ductal carcinoma developed in a patient with Huntington’s disease. The tumour showed marked nuclear pleomorphism and contained large number of bizarre tumour giant cells and abundant abnormal mitoses. Tumour cells showed nuclear vesicles and inclusions similar to those described in nuclei of neural cells in patients with Huntington’s disease. The case suggests that, in some patients, tumour morphology may reflect specific individual features. Sami Shousha Copyright © 2014 Sami Shousha. All rights reserved. Oral Verruciform Xanthoma: A Case Report and Literature Review Mon, 08 Dec 2014 13:50:32 +0000 Oral verruciform xanthoma represents an uncommon entity, which affects mainly oral mucosa. This paper presents the major clinical and histological features of oral verruciform xanthoma and reports a case on the tongue. The differential diagnosis and a literature review are also provided in light of recent information. Yonara Maria Freire Soares Marques, Cleverton Roberto de Andrade, Suzana Cantanhede Orsini Machado de Sousa, and Cláudia Maria Navarro Copyright © 2014 Yonara Maria Freire Soares Marques et al. All rights reserved. Cervical Cystic Hygroma in an Adult Sun, 07 Dec 2014 09:19:52 +0000 Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features. Serhan Derin, Murat Şahan, Yelda Dere, Neşat Çullu, and Leyla Şahan Copyright © 2014 Serhan Derin et al. All rights reserved. Bilateral Diffuse Tumorous Pseudoangiomatous Stromal Hyperplasia: A Case of Bilateral Mastectomy in a 29-Year-Old Woman Tue, 02 Dec 2014 09:55:05 +0000 Pseudoangiomatous stromal hyperplasia (PASH) is a benign breast lesion commonly encountered as an incidental microscopic finding. However, it can also manifest as a mass-forming lesion (tumorous PASH) capable of recurrence after surgical excision. Most of the previously reported cases of tumorous PASH present as a single dominant mass. Here we reported a rare case of diffuse tumorous PASH involving bilateral breasts clinically mimicking malignancy. A 29-year-old African-American female presented with a one-year history of bilateral breast enlargement and asymmetry. Physical examination revealed multiple palpable nodules in bilateral breasts. Imaging studies demonstrated innumerable homogeneously enhancing masses throughout both breasts, greater on the left, with multiple cysts and edema. Biopsy of the breast nodules demonstrated histopathological changes consistent with PASH. Due to the extent of the lesions and progressive clinical symptoms, decision was made to perform bilateral mastectomy. Macroscopic examination of the bilateral mastectomy specimens revealed markedly enlarged breasts with marked edema and numerous well-defined firm nodules. Microscopic evaluation of the nodules confirmed the diagnosis of PASH. No evidence of malignancy was identified. Recognition of this rare form of PASH is essential for the proper clinical management. Hongyan Dai, Carol Connor, Wei Cui, Jason Gatewood, and Fang Fan Copyright © 2014 Hongyan Dai et al. All rights reserved. WHO Grade 2 Neuroendocrine Tumor in a 15-Year-Old Male: A Case Report and Literature Review Sun, 30 Nov 2014 13:24:45 +0000 Neuroendocrine tumors, distinguished from adenocarcinomas by their neuroendocrine differentiation, are the most common pediatric epithelial malignancy that most often occurs in the appendix. In 2010, the WHO classified neuroendocrine neoplasms into three grades based on morphology, mitotic count, and Ki67 proliferation index. A 15-year-old male with a history of anemia and failure to thrive was diagnosed with a well-differentiated neuroendocrine tumor in the jejunum that invaded into the subserosal soft tissue and metastasized to four lymph nodes. Pediatric neuroendocrine tumors frequently arise within hereditary tumor syndromes with pancreatic neuroendocrine tumors being the most common. Several studies also indicate an elevated risk of small intestinal neuroendocrine tumors in which children born to a parent with a history of neuroendocrine tumors in the small intestine have a significant increased risk of developing one. Eric Johannesen and Van Nguyen Copyright © 2014 Eric Johannesen and Van Nguyen. All rights reserved. Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis Sun, 30 Nov 2014 00:10:16 +0000 A rat model of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides reveals crescentic glomerulonephritis as seen in human renal biopsies and diffuse lung hemorrhage that is not well documented in human lung biopsies. A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody. A renal biopsy showed pauci-immune type of crescentic glomerulonephritis and focal arteritis. The prior lung wedge biopsy was retrospectively reviewed to show diffuse hemorrhage and hemosiderosis with focal giant cells. In addition, small arteries revealed subtle neutrophil aggregation, and margination along vascular endothelium, but no definitive vasculitis. The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination. Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient. Nicholas D. Ward, Diane E. Cosner, Colleen A. Lamb, Wei Li, Jacqueline K. Macknis, Michele T. Rooney, and Ping L. Zhang Copyright © 2014 Nicholas D. Ward et al. 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.