2019-12-01 2019, Volume 6 Issue 1

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  • research-article
    Resham Ramkissoon, Akshay Shetty, Adam Doyle, Oyedele A. Adeyi, Keyur Patel

    Graft versus Host Disease (GVHD) can present with mucocutaneous, gastrointestinal and hepatic manifestations, specifically a cholestatic transaminitis. Rarely, some cases can present with only a hepatocellular transaminitis. Our patient presented with an acute hepatitis on day +90 post-hematopoietic stem cell transplant, without other overt manifestations of GVHD. The initial work up was negative for a viral etiology or causative drug, and the patient’s transaminases continued to rise. On day +96, an erythematous rash appeared with biopsy indicating lymphocytic and eosinophilic infiltrates concerning for cutaneous GVHD. Subsequently, a liver biopsy was obtained, and showed marked ductopenia with cholestasis, consistent with hepatic GVHD.

  • research-article
    Mengyuan Pang, Fang Hua, Yixiao Zhi, Eryun Qin, Yu Tao, Rui Hua

    Eosinophilic gastroenteritis (EG) is a gastrointestinal disease characterized by abnormal infiltration of eosinophilic cells in the gastrointestinal tract, excluding known causes of eosinophilia. Eosinophilic peritonitis (EP) is rare and is considered by most scholars to be a systemic or local allergy to exogenous or endogenous allergens. It is a clinical manifestation of eosinophilic gastroenteritis involving the serosal layer. Hereby, we report a case of EP in a patient with liver cirrhosis. A 32-year-old man was admitted to our hospital for intermittent fatigue, abdominal distension and abdominal pain. On account of clinical feature and pathological results of peritoneal puncture biopsy, excluding other causes of peripheral eosinophilia, the diagnosis of EP with hepatic cirrhosis was established. The possibility of EP should be paid great attention to patients with cirrhosis with peritonitis. Gastrointestinal endoscope biopsy, laparoscopy or peritoneal puncture biopsy are conducive to the diagnosis and differential diagnosis. Therefore, once the disease is suspected, gastrointestinal endoscope biopsy should be performed actively, and multiple pathological samples should be taken to contribute to diagnosis and treatment. Laparoscopy or peritoneal puncture biopsy is of vital significance for definitive diagnosis.

  • research-article
    Alessandro D’Amuri, Federica Floccari, Raffaele Filotico, Marcello Filotico

    Cutaneous Angiosarcoma of the elderly's face is a rare and peculiar neoplasm with all its own clinical and morphological characteristics. The observation of a classic case in the clinical presentation and morphology has offered the opportunity to revisit this topic.

  • research-article
    Xavier Dubernard, Bach-Ngam Pham, Michel Pluot, Yohan Renard, Marc Labrousse

    Background: Neurofibromatosis-1 (NF-1) is the most common genetic disease belonging to the group of neural tissue cell growth disorders, known to be complicated by malignant peripheral nerve sheath tumors or MPNST. On the contrary, angiosarcoma is rarely associated with NF-1. Only few cases reported angiosarcoma arising as a sarcomatous transformation within a preexisting MPNST.
    Methods and results: We report here the remarkable case of a 35-year-old NF-1 woman, with a history of excised MPNST 8 years before a new hospitalization for the development of multi localized angiosarcoma, whose discovery is related to an oropharyngeal involvement, with a dramatic presentation. Review of the literature and differences from previous reports are discussed.
    Conclusion: Our case report supports the hypothesis of an angiosarcoma developing as a malignant proliferation apparently not arising from contiguous neurofibroma or MPNST lesions, but representing a second de novo malignancy.

  • research-article
    Jaciel de Oliveira Clementino, Daniel Gallina Martins Abrahão, Manoel Sebastião da Costa Lima Junior, Herintha Coeto Neitzke Abreu

    Visceral leishmaniasis (VL) is an anthropozoonosis caused by Leishmania infantum in most Brazilian states and is known for its significant lethality resulting from improper diagnosis and treatment. VL is difficult to diagnose because its clinical manifestations and laboratory abnormalities are analogous to several other pathologies. We report a case of a 54-year-old man, negative for Human Immunodeficiency Virus (HIV), with VL who was initially diagnosed with anemia, consumptive syndrome, pneumonia, chronic obstructive pulmonary disease, and septic shock and died due to a delayed diagnosis of VL.

  • research-article
    Butch M Huston, Victor Froloff, Kelly Mills, Michael McGee

    A 51-year old male with a medical history of chronic ethanol and tobacco use was complaining of difficulty breathing and was driven to the local emergency department where he was noted to be in respiratory distress. The decedent had not seen a physician in over ten years. The decedent was undergoing an ultrasound to evaluate for a lower extremity deep venous thrombosis when he arrested. He was unable to be resuscitated. An autopsy was performed. The external examination revealed numerous petechial type hemorrhages of the skin with prominence of the arms and lower legs. Internal examination revealed cardiomegaly (heart weight 720 grams) with left ventricular hypertrophy and prominent endocarditis of the mitral and aortic valves. Infarcts were noted involving the right kidney and the spleen. A postmortem blood culture grew Streptococcus salivarius in both the aerobic and anaerobic culture bottles.

  • research-article
    S Carta, A Chungh, SR Gowda, E Synodinou, PS Sauve, JR Harvey

    Background: Brown tumour of the cervical spine is very rare and is formed due to focal altered bone remodelling secondary to persistent and uncontrolled primary or secondary hyperparathyroidism. It is considered an extreme form of osteitis fibrosa cystica that occurs in the settings of persistently elevated parathyroid hormone.
    Case Report: This a unique lesion presented in a 48 year old male with recurrent bone pain and known End Stage Renal Disease (ESRD) on maintenance haemodialysis. The main clinical complaints were weak and painful legs and the initial presentation was after the patient collapsed at home and fractured spinal level C2. The initial assessment included blood tests and radiological imaging. CT scanning of the spine revealed a destructive lytic lesion with loss of height and architectural changes of the C2 vertebral body and cord compression. The differentials included an acute fracture, a metastatic lesion and Brown’s tumour. Further imaging with an MRI of the spine and PET-CT were performed which confirmed the above lesion and excluded metastatic disease and bone marrow infiltration. A CT guided bone biopsy followed. The patient continued to receive medical treatment for secondary hyperparathyroidism and the C2 fracture was managed with orthosis through a protective hard collar.
    Discussion: An up to date literature review revealed very few cases of Brown tumour of the cervical spine and there was only one previous case with C2 involvement due to primary hyperparathyroidism and one due to secondary hyperparathyroidism. Clinicians must be aware of the varied presentations of Brown’s tumour to identify and provide the appropriate management.

  • research-article
    Ryan Yu, Brigitte Courteau, Ryan Rebello, Alice Lytwyn, Monalisa Sur, Salem Alowami

    Female adnexal tumor of probable wolffian origin (FATWO) is a tumor of low malignant potential that arises predominantly in the broad ligament, mesosalpinx, and ovarian hilus. The rarity of FATWO increases its susceptibility to misdiagnosis as other tumors, especially when it occurs at an unusual site. We report a 29-year-old woman with a 7.2 cm left paravaginal FATWO that invaded into the vaginal lumen. The initial biopsy demonstrated features suggestive of vaginal spindle cell epithelioma, but with increased Ki67 proliferation index. Pathologists should be aware that the common sieve-like architecture of FATWO may not be apparent on small biopsies, which by sampling error may demonstrate predominantly the less common spindle cell architecture. Awareness of FATWO in the differential diagnosis of paravaginal tumors may help to avoid misinterpretation as vaginal spindle cell epithelioma, a previously unappreciated pitfall in the diagnosis of FATWO.

  • research-article
    Mary K. Dick, Apporva Sharma, Poonam Sharma

    Spiradenomas stem from eccrine and apocrine glands to form benign cutaneous tumors. When arising on the breast, nipple, and areola region, spiradenomas can be difficult to differentiate from more worrisome breast masses. This is particularly true in patients with a prior history of breast cancer. These tumors can be mimicked by a wide range of adnexal growths and play a role in syndromic conditions. We present a case of a 56-year-old female with a 20-year enlarging left lower inner quadrant breast mass reviewed on mammography which was later excised and histopathologically diagnosed as a spiradenoma. Spiradenomas can be challenging to diagnose as they can present with similar findings on inexpensive and non-invasive testing such as ultrasound and mammogram. While a biopsy is a definitive tool for diagnosis, this is contraindicated in some spiradenoma mimickers such as epidermal cysts complicating diagnosis. To our knowledge, there are only six case reports of breast spiradenomas. We have reviewed the histopathology of this case and summarized all cases of breast spiradenomas and their malignant counterpart spiradenocarcinomas available in the literature compiling their presentations and imaging findings.