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Sample records for rare entity presenting

  1. Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management.

    Science.gov (United States)

    Da Aw, Lin; Zain, Murizah M; Esteves, Sandro C; Humaidan, Peter

    2016-01-01

    A typical male looking adolescent with a legal female gender assignment presented with haematuria. Investigations led to the diagnosis of Persistent Mullerian Duct Syndrome. The condition is indeed a rare entity that needs a multidisciplinar team management. Case hypothesis: A case of Persistent Mullerian Duct Syndrome undiagnosed at birth because karyotyping was defaulted, thus resulting in a significant impact on the legal gender assignment and psychosocial aspects. Promising future implications: The reporting of this case is important to create awareness due to its rarity coupled with the rare presentation with hematuria as a possible masquerade to menstruation. There were not only medical implications, but also psychosocial and legal connotations requiring a holistic multidisciplinary management. Copyright® by the International Brazilian Journal of Urology.

  2. Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management

    Directory of Open Access Journals (Sweden)

    Lin Da Aw

    Full Text Available ABSTRACT Main findings: A typical male looking adolescent with a legal female gender assignment presented with haematuria. Investigations led to the diagnosis of Persistent Mullerian Duct Syndrome. The condition is indeed a rare entity that needs a multidisciplinary team management. Case hypothesis: A case of Persistent Mullerian Duct Syndrome undiagnosed at birth because karyotyping was defaulted, thus resulting in a significant impact on the legal gender assignment and psychosocial aspects. Promising future implications: The reporting of this case is important to create awareness due to its rarity coupled with the rare presentation with hematuria as a possible masquerade to menstruation. There were not only medical implications, but also psychosocial and legal connotations requiring a holistic multidisciplinary management.

  3. Fibrolamellar Hepatocellular Carcinoma Presenting as Obstructive Jaundice: Uncommon Presentation of a Rare Entity

    International Nuclear Information System (INIS)

    Arora, Richa

    2015-01-01

    Fibrolamellar hepatocellular carcinoma is a rare primary malignant liver tumor, significantly different from generic hepatocellular carcinoma with distinct demographics, risk factors, imaging features, histopathology and prognosis. Unlike conventional hepatocellular carcinoma, it presents in young individuals with no preexisting hepatitis or cirrhosis and does not cause elevation of serum alpha feto proteins in most cases. This paper presents a case report of this rare tumor in a young female with an unusual clinical manifestation of obstructive jaundice (which has not been reported so far) along with a review of its imaging and pathological features, with treatment options. Fibrolamellar HCC is a rare variant of classic HCC with different epidemiology, risk factors, clinical manifestations, radiological, pathological and prognostic features. Therefore, it is important to be familiar with the entity for its early diagnosis and management

  4. Vaginal Endosalpingiosis Case Report: A Rare Entity Presenting as Intermenstrual Bleeding

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    Sara Câmara

    2017-01-01

    Full Text Available Endosalpingiosis is a benign and rare entity whose pathophysiology remains unknown. It has been described in pelvic organs, the abdomen, or axillar lymph nodes. Its underrecognition has occasionally led to its misinterpretation for an adenocarcinoma. This case reports the treatment and follow-up of vaginal endosalpingiosis, presenting as a vaginal polyp in a premenopausal women with intermenstrual bleeding. To our knowledge this is the first reported case of vaginal endosalpingiosis and the second mucosal localization after bladder endosalpingiosis.

  5. Toxoplasmosis of spinal cord in acquired immunodeficiency syndrome patient presenting as paraparesis: A rare entity

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    Sachin R Agrawal

    2014-01-01

    Full Text Available Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm 3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.

  6. Anal canal duplication and triplication: a rare entity with different presentations.

    Science.gov (United States)

    Palazon, P; Julia, V; Saura, L; de Haro, I; Bejarano, M; Rovira, C; Tarrado, X

    2017-05-01

    Anal canal duplication (ACD) is the rarest of gastrointestinal duplications. Few cases have been reported. Most cases present as an opening in the midline, posterior to the normal anus. The aim of our revision is to contribute with eight new cases, some of them with unusual presentations: five presented as the typical form, one with a perianal nodule, and two presented as two separate orifices (anal canal triplication). Complete excision was performed in all patients with no complications. ACD is the most distal and the least frequent digestive duplication. Its treatment should be surgical excision, to avoid complications such as abscess, fistulization, or malignization. Anal canal triplication has never been described before.

  7. Thanatophoric Dysplasia: A Rare Entity

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    N.S. Naveen

    2011-05-01

    Full Text Available Thanatophoric dysplasia is the lethal skeletal dysplasia characterized by marked underdevelopment of the skeleton and short-limb dwarfism. The child will be having a short neck, narrow thoracic cage and protuberant abdomen. Other anatomical features include a relatively enlarged head with frontal bossing, prominent eyes, hypertelorism and the depressed nasal bridge. The diagnosis is usually made with the ultrasonography in the second trimester. In this study we report a case of this rare entity with emphasis on its anatomical features, abnormalities and clinical profile with relevant review of literature.

  8. Maxillary Osteomyelitis: A Rare Entity

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    Ayaaz Habib

    2016-01-01

    Full Text Available Osteomyelitis of the maxilla is now a rare event with the advent of antibiotics. The two predominant causes are odontogenic infections and sinusitis. Immunocompromised states such as diabetes, HIV, and malnutrition increase the risk of osteomyelitis. It is important to recognize this early as it is a difficult entity to treat with potentially serious consequences. We report an unusual case of right sided maxillary osteomyelitis in a lady with poorly controlled diabetes in rural Lincolnshire. Biopsy of the right maxillary bone showed features of acute osteomyelitis. This responded well to a prolonged course of oral antibiotics.

  9. Vitamin K deficiency bleeding presenting as nodular purpura in infancy: A rare and life-threatening entity

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    Pratik Gahalaut

    2013-01-01

    Full Text Available Vitamin K deficiency bleeding (VKDB disorder is an uncommon entity, which occurs due to inadequate activity of vitamin K-dependant coagulation factors. An 8-months-old exclusively breast-fed male infant presented with multiple, purpuric and nodular non-collapsible swellings on trunk of 4 days duration. Investigations revealed raised activated partial thromboplastin time and prothrombintime. Fibrinogen level and platelet counts were normal. Late VKDB usually presents as intra-cranial or mucosal hemorrhages. [1] Though skin and mucosal bleeding may occur in 1/3 rd of infants with VKDB, ′nodular purpura′ is not the common presenting feature. Earlier recognition of VKDB and immediate investigation/treatment helps prevent the potentially fatal outcome of the disease. Very little is mentioned about this entity in dermatology literature.

  10. Cardiofaciocutaneous syndrome: A rare entity

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    S Pavithra

    2012-01-01

    Full Text Available The cardiofaciocutaneous (CFC syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation and characteristic dysmorphic features. We, thus, report a rare case of this syndrome in a 1-year-old child who presented with typical features of CFC syndrome.

  11. Intracranial chondroma: a rare entity.

    Science.gov (United States)

    Maheshwari, Veena; Mehdi, Ghazala; Varshney, Manoranjan; Jain, Anshu; Vashishtha, Sonal; Gaur, Kavita; Srivastava, Vinod Kumar

    2011-05-12

    Intracranial chondroma is a rare benign cartilaginous tumour with an incidence of less than 1% of all primary intracranial tumours. The authors are reporting here a case of intracranial chondroma in a 40-year-old man who presented with 5-month history of headache and gradual diminution of vision. A tentative diagnosis of chondroma was made on imprint cytology which was confirmed on histopathological examination.

  12. Pancreatic pseudopapillary tumour: A rare misdiagnosed entity.

    Science.gov (United States)

    Affirul, C A; Qisti, F N; Zamri, Z; Azlanuddin, A; Hairol, A O; Razman, J

    2014-01-01

    Solid pseudo papillary pancreatic tumour is a rare entity. The atypical presentation causes a delayed or misdiagnosis of these pathology. It commonly affects the female population in the 2nd and 3rd decade of life. The presentation varies from non-specific abdominal pain to incidental findings in asymptomatic patients. It is a low-grade premalignant condition that is curable by excision of the tumour. This paper presents a 17-year-old girl with intra-abdominal mass diagnosed with solid pseudo papillary tumour that underwent Whipple's procedure. We discuss the presentations, diagnosis and pathology findings of this rare pathology. The diagnosis remains an enigma in view of the nature and location of the tumour. Resection is still the best choice remains for this condition. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  13. Xanthogranulomatous Prostatitis, a Rare Prostatic Entity

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    Alejandro Noyola

    2017-01-01

    Full Text Available There are several benign prostatic pathologies that can clinically mimic a prostate adenocarcinoma. Xanthogranulomatous prostatitis is a benign inflammatory condition of the prostate and a rare entity. A 47-year old male, with 3 years of lower urinary tract symptoms, with a palpable hypogastric tumor, digital rectal examination: solid prostate, of approximately 60 g. Initial PSA was 0.90 ng/mL. He underwent surgical excision of the lower abdominal nodule and prostatectomy. Histopathology showed xanthogranulomatous prostatitis, without malignancy. Xanthogranulomatous prostatitis is an extremely rare entity that can simulate prostate adenocarcinoma, therefore having a correct histopathological diagnosis is essential.

  14. Hybrid Odontogenic Lesion: A Rare Entity

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    Reza Imani

    2017-03-01

    Full Text Available Hybrid tumors are very rare tumors composed of two different tumor entities, each of which conforms to an exactly defined tumor category. A 14-year-old boy was referred for an intraosseous painless lesion with a histopathological feature of multiple odontogenic lesions including calcifying odontogenic cyst, complex odontoma and ameloblastic fibro-odontoma. The final diagnosis considered to be a hybrid odontogenic lesion.

  15. Ossification of the bilateral Achilles tendon: a rare entity

    International Nuclear Information System (INIS)

    Arora, Abhishek J; Arora, Richa

    2015-01-01

    Ossification of the Achilles tendon is a rare clinical entity comprising of one or more segments of variable sized ossified masses in the fibrocartilaginous substance of the tendon. The etiology of ossification of the Achilles tendon is multifactorial with recurrent trauma and surgery comprising major predisposing factors, with others being metabolic, systemic, and infectious diseases. The possibility of a genetic predisposition towards this entity has also been raised, but has not yet been proven. We present a rare case of ossification of the bilateral Achilles tendons without any history of trauma or surgery in a 48-year-old female patient

  16. Amelogenesis imperfecta and localised aggressive periodontitis: A rare clinical entity

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    Gayatri Gundannavar

    2013-01-01

    Full Text Available This case report presents two female patients whose chief complaint was discoloration of teeth. On careful clinical examination it was found that the patients had features of amelogenesis imperfecta and localised aggressive periodontitis. This article will give an insight of clinical and radiographic features of amelogenesis imperfecta with localised aggressive periodontitis, which is a rare clinical entity.

  17. Malignant struma ovarii: a rare oncologic entity

    International Nuclear Information System (INIS)

    Barbosa, Frederico Paes; Mendonca, Sandro Bichara; Souza, Thiago Ferreira de

    2004-01-01

    Struma ovarii (SO) malignant is a rare ovarian teratoid tumor, consisting predominantly of thyroid tissue. It has low incidence and a few cases described by the literature. Usually appears in the 4th or 5th decade of life. Generally they are diagnosed after surgery, being the diagnostic reserved for the lesions on the ectopic thyroid tissue based on cellular atypia, mitotic activity, capsular invasion and metastases. Due to its rarity,it does not still have a consensus about the best therapeutical management and protocol of accompaniment for patients with this neoplastic entity. This article reports a case of a patient with malignant S O, reviewing the relevant aspects of the literature. (author)

  18. An extremely rare clinical entity: congenitally corrected transposition with situs ınversus and single coronary artery presented with complete atrioventricular block in a young man.

    Science.gov (United States)

    Cirakoglu, Omer Faruk; Bayraktar, Ali; Sayin, Muhammet Rasit

    2018-05-01

    Congenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies. However, situs inversus patients with congenitally corrected transposition, single coronary artery anomaly, and atrioventricular block together have not been reported previously. This combination of abnormalities is presented as a first in the literature.

  19. Urethral Leiomyoma: A Rare Clinical Entity

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    Ng Beng Kwang

    2016-01-01

    Full Text Available Extrauterine leiomyomas are encountered occasionally, which can pose a diagnostic dilemma and challenge to the gynaecologist. We report a rare case of urethral leiomyoma. A 31-year-old woman with history of primary subfertility presented with mass at her urethral meatus and lower urinary tract symptoms. She underwent examination under anaesthesia and excision of the urethral mass. Histopathological examination confirmed leiomyoma. Diagnosis and management of this common growth situated at a rare location were reviewed and discussed.

  20. Neurothekeoma of petrous apex: A rare entity

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    Zarina Abdul Assis

    2013-01-01

    Full Text Available Intraosseous nerve sheath tumors are very rare tumors accounting for lesser than 0.2% of primary bone tumors. We present an 18-year-old female who presented with left facial paresis for the last 1 year. Magnetic resonance imaging (MRI demonstrated expansile, multiseptated, enhancing bony lesion in the left petrous apex. There was also abnormal enhancement of the 7-8 th nerve complex within the internal auditory canal. Tumor was excised by subtemporal extradural approach. The lesion was diagnosed as intraosseous neurothekeoma on histopathology. This is an extremely rare tumor and its MRI appearance in this location is being described for the first time in literature.

  1. Adenosquamous carcinoma of vesicovaginal fistula: a rare entity.

    Science.gov (United States)

    Tabali, Rudresh; Ramkumar, Aravind

    2014-01-01

    A 56-year-old lady presented with a vesicovaginal fistula (VVF) along with past history of abdominal hysterectomy. Biopsy of the fistulous tract showed squamous cell carcinoma (SCC). Patient underwent radical cystourethrectomy, total vaginectomy, and bilateral pelvic lymph node dissection along with ileal conduit. The final histopathology report of the resected specimen showed adenosquamous carcinoma in VVF. As this is a rare entity, we are reporting this case.

  2. Adenosquamous Carcinoma of Vesicovaginal Fistula: A Rare Entity

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    Rudresh Tabali

    2014-01-01

    Full Text Available A 56-year-old lady presented with a vesicovaginal fistula (VVF along with past history of abdominal hysterectomy. Biopsy of the fistulous tract showed squamous cell carcinoma (SCC. Patient underwent radical cystourethrectomy, total vaginectomy, and bilateral pelvic lymph node dissection along with ileal conduit. The final histopathology report of the resected specimen showed adenosquamous carcinoma in VVF. As this is a rare entity, we are reporting this case.

  3. Bilateral cerebellopontine arachnoid cyst: A rare entity.

    Science.gov (United States)

    Sharma, Anand; Sharma, Achal; Mittal, Radhey S; Gandhi, Ashok

    2015-01-01

    Bilateral cerebellopontine angle (CPA) arachnoid cysts (ACs) are very rare: only one case is reported in literature. Pathogenesis of those cysts is unknown; they are thought to be congenital. The presenting symptoms of CPA AC are frequently nonspecific or otological. The management of ACs of the CPA is controversial. We are reporting two cases of bilateral CPA AC with their pathophysiology and review of literature.

  4. Isolated single coronary artery (RII-B type presenting as an inferior wall myocardial infarction: A rare clinical entity

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    Ankur C. Thummar

    2014-09-01

    Full Text Available Isolated single coronary artery without other congenital cardiac anomalies is very rare among the different variations of anomalous coronary patterns. The prognosis in patients with single coronary varies according to the anatomic distribution and associated coronary atherosclerosis. If the left main coronary artery travels between the aorta and pulmonary arteries, it may be a cause of sudden cardiac death. We present multimodality images of a single coronary artery, in which the whole coronary system originated by a single trunk from the right sinus of Valsalva with inter-arterial course of left main coronary artery. This rare type of single coronary artery was classified as RII-B type according to Lipton's scheme of classification. A significant flow-limiting lesions were found in the right coronary artery that was successfully treated with percutaneous coronary intervention.

  5. Herpes zoster oticus: A rare clinical entity

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    Shailesh Gondivkar

    2010-01-01

    Full Text Available Herpes zoster oticus also known as Ramsay Hunt syndrome is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion causes otalgia, auricular vesicles, and peripheral facial paralysis. Ramsay Hunt syndrome is rare in children and affects both sexes equally. Incidence and clinical severity increases when host immunity is compromised. Because these symptoms do not always present at the onset, this syndrome can be misdiagnosed. Although secondary to Bell′s palsy in terms of the cause of acute atraumatic peripheral facial paralysis, Ramsay Hunt syndrome, with incidence ranged from 0.3 to 18%, has a worse prognosis. Herpes zoster oticus accounts for about 12% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. The most advisable method to treat Ramsay Hunt syndrome is the combination therapy with acyclovir and prednisone but still not promising, and several prerequisites are required for better results. We present a case of 32-year-old man suffering from Ramsay Hunt syndrome with grade V facial palsy treated effectively with rehabilitation program, after the termination of the combination therapy of acyclovir and prednisone.

  6. Urachal remnant carcinoma - a rare entity

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    Vanesha Naidu

    2013-06-01

    Full Text Available Primary malignancy of the urachal remnant is a rare neoplasm that accounts for less than 0.01% of all adult cancers, with an estimated annual incidence of 1:5 million. The tumour carries a grave prognosis that attests to its highly aggressive nature. Owing to its extra-peritoneal location, the tumour runs a relatively silent clinical course until late presentation, when most patients display extensive local invasion and metastatic spread. In this report, we highlight a case of primary malignancy of the urachus that on initial clinical evaluation masqueraded as a Sister Mary Joseph’s nodule. Characteristic imaging features, however, proved decisive in establishing the diagnosis of a urachal carcinoma.

  7. Pseudomyxoma peritonei (PMP) – a rare entity

    African Journals Online (AJOL)

    Department of Radiology, University of Pretoria. Corresponding author: Z Ebrahim (zaeem@iafrica.com). Pseudomyxoma peritonei (PMP) is a rare complication of mucinous tumours of appendiceal or ovarian origin that results in peritoneal and omental implants. In addition to the appendix and the ovary, other rare.

  8. Splenic abscess: a rare presentation

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    Mohit Bhatia

    2015-01-01

    Full Text Available Splenic abscess is a rare clinical entity with an incidence of 0.2-0.7% in autopsy-based studies. When untreated, splenic abscess is associated with nearly 100% mortality; in treated patients, the mortality rate is 16.6% during the first 90 days. It mostly occurs in patients with neoplasia, immunodeficiency, trauma, diabetes or splenic infarct. The incidence of splenic abscess is thought to be growing because of the increase in the number of immunocompromised patients who are particularly at risk for this disease and also because of the widespread use of diagnostic modalities. However, the optimal treatment for this remains unclear. We present a case of a 42-year-old man diagnosed with multiloculated splenic abscess and was subjected to splenectomy.

  9. Isolated celiac trunk aneurysm dissection: A rare entity

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    Esteban-Zubero, Eduardo

    2017-04-01

    Full Text Available Spontaneous and isolated celiac artery dissection is a rare clinical disease. It is the fourth cause of abdominal aneurysms behind those that occur in the splenic, hepatic, and superior mesenteric arteries. It is important to suspect the diagnosis of this entity whose clinical symptoms are unspecific. We report the case of a 60 year-old male treated in our hospital because of this illness, and present a review of this pathology, specially focused on the diagnosis and the different treatments available.

  10. Gastric Volvulus: A Rare Entity Case Report and Literature Review

    Science.gov (United States)

    Akhtar, Aisha; Sheikh, Abdul Ahad E; Sheikh, Abu Baker; Perisetti, Abhilash

    2018-01-01

    Gastric volvulus is a rare entity defined as an abnormal rotation of the stomach around itself. It is a diagnosis of exclusion; the clinical index of suspicion is always low and is mostly diagnosed on imaging or on the surgery table. When it occurs, it is an emergency due to the risk of strangulation and consequent gangrene of the stomach. Mesentero-axial (MA) gastric volvuli constitute one-third of all cases. Here, we are present an interesting case of acute MA gastric volvulus diagnosed with imaging and treated subsequently. PMID:29755908

  11. Concomitant hypo-hyperdontia: A rare entity

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    Yin-Lin Wang

    2018-03-01

    Full Text Available Background/purpose: Concomitant hypo-hyperdontia (CHH is a rare numeric dental anomaly characterized by congenital missing teeth and supernumerary teeth occurring in the same individual. Due to its rarity and sporadicity, the causes of CHH have been completely unknown. Detailed characterization and presentation of more CHH cases not only strengthen clinical diagnosis and treatment for the patients but facilitate the search for etiological factors of the disorder. Materials and methods: From a pedodontic patient population, 21 CHH subjects, with a mean age of 6 years 10 months, were identified and characterized. Dental records and radiographs were scrutinized and analyzed for the distribution and frequencies of involved teeth and concurrent dental anomalies. Through further literature review, 59 CHH cases with supernumeraries in the premaxillary region were retrieved for comparative analyses. Results: The boys were affected twice as often as the girls. While most cases were unrelated and sporadic, two sisters and a pair of identical twins from two unrelated families were presented. Of all cases, only one was of syndromic CHH carrying Duchenne muscular dystrophy. Bimaxillay CHH, with anomalies involving two jaws, occurred more than 4 times as often as maxillary CHH. While all supernumeraries were found in premaxillary region, hypodontia frequently involved lateral incisors and premolars of both jaws. Conclusion: As genetic contribution to CHH is strongly suggested by its familial occurrence and syndromic cases, environmental factors seem to play certain roles in modifying disease phenotypes. Judicious use of radiographs during early mixed dentition stage enhances clinical diagnosis and treatment of CHH. Keywords: Tooth agenesis, Supernumerary, Numeric anomaly, Premaxillary

  12. A Rare But Important Entity: Epistaxis in Infants.

    Science.gov (United States)

    DeLaroche, Amy M; Tigchelaar, Helene; Kannikeswaran, Nirupama

    2017-01-01

    Epistaxis is a common emergency department (ED) complaint; however, this entity is rare among children younger than 2 years of age. In this age group, epistaxis may be a presenting sign of a bleeding disorder or nonaccidental trauma. We present a case of a 2-month-old infant who was evaluated in the pediatric ED for epistaxis and discharged home. The infant returned 2 days later with facial swelling and irritability, and was found to have significant head trauma. In this article we review the epidemiology and differential diagnoses for epistaxis among infants, as well as the initial approach to the evaluation of this uncommon clinical entity in this age group. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Potential etiologies for epistaxis among infants and young children include severe systemic disease and nonaccidental trauma. Given its rarity and possible clinical significance, the approach to epistaxis differs in this age group. Emergency physicians should screen for and exclude a bleeding disorder while also having a very low threshold for a nonaccidental trauma evaluation. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. A Rare Entity: A Case Report

    African Journals Online (AJOL)

    abp

    2014-07-26

    Jul 26, 2014 ... Key words: Melorheostosis, sclerotic bone dysplasias, CT, MRI ... imperfectly known as clinical presentations are highly variable, and the etiological diagnosis is not fully elucidated. ... Scintigraphy can detect subclinical lesions ... standard radiographs every three months, and an annual bone scan.

  14. DPG-plus syndrome: new report of a rare entity.

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    Azurara, Laura; Marçal, Mónica; Vieira, Filipa; Tuna, Madalena Lopo

    2015-11-12

    Pituitary gland duplication is a particularly rare finding. Different theories have been proposed to explain its pathogenesis, however, this phenomenon is not yet totally understood. Recently, duplication of the pituitary gland (DPG)-plus syndrome has been described, associating DPG with other blastogenic defects. We present the clinical and imaging findings of a newborn girl with DPG, associated with multiple other midline anomalies, including a nasopharyngeal teratoma, palate cleft deformity, bifid nasal bridge, tongue and uvula, hypoplasia of the basis pontis and corpus callosum, duplication of the basilar artery and hypothalamic hamartoma. We describe our patient's multidisciplinary team approach and emphasise the importance of reporting upcoming cases, in order to give more insight into the understanding of this complex entity. 2015 BMJ Publishing Group Ltd.

  15. Retroperitoneal Cellular Angiofibroma: A Rare Gynecological Entity

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    Ana Brandão

    2017-12-01

    Full Text Available Cellular angiofibroma is a mesenchymal tumor, described in 1997, without gender preference, that usually appears at age 40. The vulvovaginal area is the most common site in women, mimicking vulvar benign tumors, like Bartholin gland cyst. However, there are a few described cases of a deep or extra-pelvic angiofibroma. Excision is the treatment of choice and the recurrence rate appears to be low. We present the case of a woman with a heterogeneous tumor in the right adnexial region. At the surgery, a retroperitoneal tumor was excised and the histopathological tissue analysis revealed a cellular angiofibroma.

  16. Pediatric Type Follicular Lymphoma: A Rare Entity with Excellent Prognosis

    Science.gov (United States)

    2018-01-19

    YYYY) 12. REPORT TYPE 19/01/2018 Poster 4. TITLE AND SUBTITLE Pediatric -Type Follicular Lymphoma: A Rare Entity with Excellent Prognosis 6. AUTHOR(S...lymphoma is common in older adults but rare in pediatric and young adult patients. Pediatric follicular lymphoma comprises a only 6.5% of childhood... Pediatric follicular lymphoma is defined by a localized high grade appearing lymphoma that lacks these gene rearrangements. Other diagnoses to rule out

  17. Sirenomelia: A Rare Presentation

    Science.gov (United States)

    Srinivas, S; Kumar, Shiva; Reddy, Surweshwar; Prasad, Hari; Irfan, G M

    2012-01-01

    We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed. PMID:26023366

  18. Sirenomelia: a rare presentation.

    Science.gov (United States)

    Reddy, K Ramesh; Srinivas, S; Kumar, Shiva; Reddy, Surweshwar; Prasad, Hari; Irfan, G M

    2012-01-01

    We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed.

  19. Primary malignancy of seminal vesicle: A rare entity

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    Rajaraman Ramamurthy

    2011-01-01

    Full Text Available We report a rare case of seminal vesicle malignancy (primitive neuro ectodermal tumor in a 40-year-old male patient. He was treated with enbloc resection of the tumor and ureteric reimplantation. In view of the rarity of this entity, management of these tumors should be individualized.

  20. Lichen planus hypertrophicus of the vulva - a rare entity.

    Science.gov (United States)

    Job, Anupa Mary; Kaimal, Sowmya

    2017-09-01

    Vulvovaginal lichen planus is a type of chronic inflammatory dermatosis of the vulva, usually seen as a part of widespread lichen planus. The common clinical types of vulval lichen planus include papulosquamous, erosive and pigmented types. Hypertrophic lichen planus of the vulva is a rare entity. We report a case of hypertrophic lichen planus of the vulva which clinically simulated genital warts.

  1. [Isolated splenic metastases from cervical cancer: a rare entity].

    Science.gov (United States)

    Villalón-López, José Sebastián; Souto-del Bosque, Rosalía; Montañez-Lugo, Juan Ignacio; Chávez-González, Bruno

    2014-01-01

    Splenic metastases from solid tumors are a rare event with an incidence of only 2.9% to 9%. Splenic metastases from cervical cancer are a rare entity. Only a few cases have been reported of isolated spleen metastases from cervical cancer. We present the case of a 76-year-old woman with moderately differentiated endocervical adenocarcinoma stromal and endocervical invasion. Clinical stage was Ib1 and Ca-125 values of 150 U. She was managed with hysterectomy and pelvic lymphadenectomy. She received pelvic radiotherapy (45 Gy) followed 24 Gy of brachytherapy. Two years later she presented with abdominal pain. Abdominal computed tomography showed two splenic parenchymal lesions without disease in the remainder of the abdominal cavity and chest with a Ca-125 of 2,733 U. The patient is submitted to splenectomy. Histopathology demonstrates splenic metastases of well-differentiated adenocarcinoma from the endocervix. Immunohistochemical stain showed positivity from carcinoembryonic antigen; estrogen and progesterone receptors are negative. Ca-125 level 8 weeks after surgery was 16 U/ml. The patient received six cycles of adjuvant chemotherapy with paclitaxel and cisplatin. At 12 months follow-up the patient is alive and without evidence of tumor activity. The spleen is an uncommon site of metastasis. Splenectomy is considered the appropriate treatment in order to avoid complications such as splenic rupture and splenic vein thrombosis as well as to improve pain control from splenomegaly. Twelve months after surgery our patient is alive and without evidence of tumor activity.

  2. Reverse (posterior) cloaca with congenital rectal stenosis and pseudohermaphrodism: a rare entity with rare association

    Science.gov (United States)

    Ahmed, Intezar; Kureel, Shiv Narain; Chandra, Naveen

    2010-01-01

    Reverse (posterior) cloaca is itself a rare entity and association of this defect with other congenital syndromes or defects is extremely rare to find. To diagnose this unique anomaly one should be aware about this very infrequently encountered clinical entity. Reverse cloaca is a very rare variant of anorectal malformation in which urethra and vagina fused to form a urogenital sinus that diverts posteriorly and opens in the anterior rectal wall at the anus or immediately anterior to it. We report a neonate with the rare clinical entity of reverse cloaca type C, pseudophallus with rudimentary urethra as a component of female pseudohermaphrodism and congenital rectal stenosis. Surprisingly, there was no associated urological abnormality on ultrasonography and laparotomy. PMID:22797480

  3. A Rare Presentation of Odontogenic Keratocyst Mimicking an Antral Polyp

    OpenAIRE

    Sudhakar, S; Geethika, V; Ramaswamy, P; Kumar, PK; Sreenivasulu, P

    2014-01-01

    Maxillary sinus harbours many pathological lesions. Many of those presents as a sinonasal mass and are rarely symptomatic. These masses are usually an antral polyp, mucoceles or mucous retention cysts. Odontogenic keratocyst, a benign odontogenic lesion presenting within the maxillary sinus is a rare entity. We present a case of odontogenic keratocyst of the maxillary sinus in a 35 years old female.

  4. Autosomal recessive anhidrotic ectodermal dysplasia: A rare entity

    Directory of Open Access Journals (Sweden)

    Sangita Ghosh

    2014-01-01

    Full Text Available We describe a case of anhidrotic ectodermal dysplasia (AED with an autosomal recessive mode of inheritance, a very rare entity, in a 2-year-old female child of two asymptomatic, consanguineous parents. Their previous child also had a similar condition. Autosomal recessive AED (AR-AED can have its full expression both in males and females and it is clinically indistinguishable from the x-linked recessive AED (XL-AED, which is the most common type of ectodermal dysplasia. Unlike the partially symptomatic carriers of XL-AED, the heterozygotes of AR-AED are phenotypically asymptomatic.

  5. Solitary plasmacytoma of the mandible - a rare entity.

    Science.gov (United States)

    Baad, Rajendra; Kapse, Sonam C; Rathod, Nanita; Sonawane, Kishor; Thete, Sanjay Gangadhar; Kumar, M Naveen

    2013-06-01

    Plasma cell dyscrasias (multiple myeloma, solitary plasmocytoma of bone and extra medullary plasmocytoma) are cha¬racterized by a monoclonal neoplastic proliferation of plasma cells of which Solitary plasmocytoma of bone (SPB) is a localized form. SPB is most frequently seen in vertebrae and secondarily in long bones. Its presence in jaws is extremely rare. The malignant plasma cells express monotypic cytoplasmic immunoglobulins and plasma cell-associated antigens, with an absence of immature B-cell antigens. Here we report a unique case of plasmacytoma in the right side of mandible, a chronology for diagnosis of the lesion is also reviewed along with clinical, radiographic, histopathological and immunohistochemical evidence. How to cite this article: Baad R, Kapse S C, Rathod N, Sonawane K, Thete S G, Naveen M K. Solitary Plasmacytoma of the Mandible - A Rare Entity. J Int Oral Health 2013; 5(3):97-101.

  6. Neonatal hemophilia: a rare presentation

    Directory of Open Access Journals (Sweden)

    Nuno Ferreira

    2015-12-01

    Full Text Available Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.

  7. Carcinoma caecum - a rare presentation

    International Nuclear Information System (INIS)

    Ali, T.; Mudasser, S.; Mudsser, S.; Sarwar, M.

    2010-01-01

    Caecal and right sided colonic cancers mostly present with fatigue, weakness and iron deficiency anemia. Such tumours rarely perforate posteriorly and involve the retroperitoneum. We report a case of an old Omani lady who came with insidious sign and symptoms of perforated caecum leading to retroperitoneal collection and necrotizing fasciitis of abdominal wall due to carcinoma of caecum. She underwent surgery but despite active intervention, she died because of septicemic shock. (author)

  8. Lingual fibrolipoma – a rare clinicopathological entity | Mungul ...

    African Journals Online (AJOL)

    Lipoma is a relatively common benign tumour occurring at sites of the body which are rich in adipose tissue. Due to sparsity of adipose tissue within the oral cavity, lipoma rarely presents in this region. Fibrolipoma of the tongue is a rare lesion. There are only 14 cases reported in the literature. An 85-year-old patient ...

  9. Tubercular myositis of infraspinatus: a rare clinical entity

    OpenAIRE

    Vikas Verma; Yogesh Kumar Yadav; Anuj Rastogi; Farid Mohammed

    2016-01-01

    Tuberculosis of the musculoskeletal system is generally confined to bones and joints. The surrounding soft tissue is secondarily infected. Tuberculous bursitis, tenosynovitis and primary pyomyositis are rarer manifestations of the disease. Of these, primary tuberculouspyomyositis is probably the rarest entity. We report a case of tubercular myositis of infraspinatus in an 8 year-old female who presented with pain, low grade fever, weight loss, anorexia, progressively increasing pain in the sc...

  10. Headache associated with airplane travel: a rare entity.

    Science.gov (United States)

    Cherian, Ajith; Mathew, Mini; Iype, Thomas; Sandeep, P; Jabeen, Afshan; Ayyappan, K

    2013-01-01

    Airplane travel headache is rare and has recently been described as a new form of headache associated with a specific situation. Of the 1,208 patients with primary headaches attending a tertiary care neurology hospital, two (0.16%) patients satisfied the criteria for headache related to airplane travel. Both the patients fulfilled the proposed diagnostic criteria for airplane travel headache. This unique headache had a mean duration of 24 minutes, localized to the medial supraorbital region described as having an intense jabbing or stabbing character that occurred exclusively and maximally during aircraft landing or take-off, following which pain intensity subsided . This rare headache felt on aircraft descent is probably due to the squeeze effect on the frontal sinus wall, when air trapped inside it contracts producing a negative pressure leading to mucosal edema, transudation and intense pain. Use of nasal decongestants either alone or in combination with naproxen sodium prior to ascent and descent abated the headache episodes. Awareness about this unique entity is essential to provide proper treatment and avoid patient suffering.

  11. The Fourth Branchial Complex Anomaly: A Rare Clinical Entity

    OpenAIRE

    Patel, Alpen B.; Hinni, Michael L.

    2011-01-01

    Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies.

  12. The fourth branchial complex anomaly: a rare clinical entity.

    Science.gov (United States)

    Patel, Alpen B; Hinni, Michael L

    2011-01-01

    Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies.

  13. Post-traumatic cervical pneumorrhachis--a rare entity.

    Science.gov (United States)

    Gill, Maneet; Sreenivas, Mukkamala; Beniwal, Rajveer Singh

    2011-02-01

    Pneumorrhachis(PR) is a rare phenomenon and post traumatic PR even more so. Presentation can vary from asymptomatic to significant neurological deficit and so the management has to be individualised. We present a case of post-traumatic cervical PR.

  14. Mucoepidermoid carcinoma of the anterior maxilla: A rare entity

    Directory of Open Access Journals (Sweden)

    Smitha Kullaje

    2014-01-01

    Full Text Available Mucoepidermoid carcinoma is the second most common salivary gland tumor primarily involving major salivary glands and intraoral minor salivary glands. Although rare, it can occur in the jaw bones, but it is very rare in the maxillary anterior region. Here, we present a case of mucoepidermoid carcinoma occurring in maxillary anterior region in a 33-year-old male patient presented as painless swelling clinically appearing similar to a periapical or nasopalatine cyst. The final diagnosis of this lesion was made after microscopic examination of biopsy specimen.

  15. A Rare Presentation of Odontogenic Keratocyst Mimicking an Antral Polyp

    Directory of Open Access Journals (Sweden)

    S Sudhakar

    2014-02-01

    Full Text Available Maxillary sinus harbours many pathological lesions. Many of those presents as a sinonasal mass and are rarely symptomatic. These masses are usually an antral polyp, mucoceles or mucous retention cysts. Odontogenic keratocyst, a benign odontogenic lesion presenting within the maxillary sinus is a rare entity. We present a case of odontogenic keratocyst of the maxillary sinus in a 35 years old female.

  16. Schwannoma of the Recurrent Laryngeal Nerve : A Rare Entity

    NARCIS (Netherlands)

    de Heer, Linda M; Teding van Berkhout, F; Priesterbach, Loudy; Buijsrogge, Marc P

    Neurogenic tumors are the most common posterior mediastinal tumors in adults. Schwannomas originating from the recurrent laryngeal nerve are rare. The present study describes a 46-year-old man with a tumor in the left superior mediastinum. Because of the narrow relationship with the aorta and the

  17. Enterobius vermicularis in the nose: A rare entity

    OpenAIRE

    Kaniyur, Vishnu; Chandra Prasad, Kishore H.; Devan, P. P.; Doddamani, S. S.; Balachandran, Bharati; Kulkarni, Vikram

    2005-01-01

    A rare case of enterobius vermicularis pin-worm is reported in the nose. An 11-year-old girl presented with the vague symptoms of crawling sensation in the nose for few weeks, who had received treatment for allergic rhinitis. The nasal secretions were examined and confirmed the diagnosis of pinworm infection and treated by albendazole.

  18. A case report on esophageal tuberculosis – A rare entity

    Directory of Open Access Journals (Sweden)

    Vatsal Khanna

    2017-01-01

    Full Text Available This is a case report of a rare form of tuberculosis in a patient presenting with dysphagia. Patient was subjected to upper gastrointestinal endoscopy, which revealed an ulcerative growth in the distal esophagus. Histopathology revealed esophageal tuberculosis. Patient was managed conservatively with Anti-Tuberculosis Treatment (ATT. Follow up endoscopy after two months revealed resolution of the growth and patient was symptomatically better. In spite of the rare nature of the disease, it can be managed effectively with ATT to avoid complications (fistula, stricture, and esophageal perforation, which might warrant surgery.

  19. Fournier′s gangrene of the penis: A rare entity

    Directory of Open Access Journals (Sweden)

    Ashutosh Talwar

    2010-01-01

    Full Text Available Fournier′s gangrene is a rare, fulminant, but and usually a localized disease of the scrotum and penis, with an occasional extension up to the abdominal wall. The usual organism is an anaerobic streptococcus synergistic with other organisms. A 45-year-old male presented with fever and pain and a brownish-black discolouration of the penis, of four days. Our case was unusual in that the penis was involved, which is very rare. Early therapy is the key, including hospitalization, debridement of the entire shaft of the penis distal to the devastated area, without excising the normal skin, parenteral broad-spectrum antibiotics, and skin grafting.

  20. Primary tubercular caecal perforation: a rare clinical entity

    Directory of Open Access Journals (Sweden)

    Moses Sonia

    2010-03-01

    Full Text Available Abstract Background Intestinal tuberculosis is a common problem in endemic areas, causing considerable morbidity and mortality. An isolated primary caecal perforation of tubercular origin is exceptionally uncommon. Case presentation We report the case of a 39 year old male who presented with features of perforation peritonitis, which on laparotomy revealed a caecal perforation with a dusky appendix. A standard right hemicolectomy with ileostomy and peritoneal toileting was done. Histopathology revealed multiple transmural caseating granulomas with Langerhans-type giant cells and acid-fast bacilli, consistent with tuberculosis, present only in the caecum. Conclusions We report this extremely rare presentation of primary caecal tuberculosis to sensitize the medical fraternity to its rare occurrence, which will be of paramount importance owing to the increasing incidence of tuberculosis all over the world, especially among the developing countries.

  1. The Fourth Branchial Complex Anomaly: A Rare Clinical Entity

    Directory of Open Access Journals (Sweden)

    Alpen B. Patel

    2011-01-01

    Full Text Available Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies.

  2. A Rare Presentation of Cyclitis Induced Myopia.

    Science.gov (United States)

    Ijaz, Umar; Habib, Asad; Rathore, Hassan Sajjad

    2018-03-01

    Unilateral cyclitis leading to myopia is a rare and clinical relevant entity. In clinical settings, pseudomyopia is generally encountered in the form of accommodative spasm, which is always bilateral. Cyclitis due to inflammation, on the other hand, can cause pseudomyopia unilaterally and it is a very rare presentation. A young male with acute anterior uveitis, presented with acute episode of unilateral myopia. When patient was examined on first visit, there were no cells in anterior chamber; so he was started on cycloplegic eye drops, but his condition didn't improve. Examination on subsequent visit revealed cellular reaction in anterior chamber and narrowing of anterior chamber angles on anterior segment optical coherence tomography (OCT). Treatment for uveitis was started and patient's visual acuity and refractive error improved. Pseudomyopia is a known complication of several drugs and certain medical conditions. The possible mechanism is supraciliary exudation causing relaxation of zonular fibers and increased convexity of the crystalline lens. Myopia in the setting of a mild cellular reaction can easily be missed and has not been reported yet to the best of authors' literature search.

  3. Tuberculous mastitis diagnosed on cytology - case report of a rare entity

    Directory of Open Access Journals (Sweden)

    Riti Tushar Kanti Sinha

    2017-01-01

    Full Text Available Tuberculous mastititis is a rare clinical entity and usually affects women from the Indian subcontinent. It often mimics breast carcinoma and pyogenic breast abscess. Fine needle aspiration cytology (FNAC is a very essential diagnostic tool when other routine laboratory investigations are not helpful in reaching to the conclusion. Tuberculosis (TB of the breast is an uncommon presentation of TB even in countries where the incidence of pulmonary and extrapulmonary TB is high. Radiological imaging is not diagnostic.

  4. Spontaneous common bile duct perforation—A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Melissa Amberger

    Full Text Available Introduction: Spontaneous common bile duct perforation is an uncommon clinical entity in both adults and children. Few case reports have been published since the first clinical description in 1882. Our work has been reported in line with SCARE criteria. Presentation of case: Herein, we describe the case of a 28 year-old female who suffered spontaneous common bile duct perforation while admitted for choledocholithiasis. Discussion: The perforation occurred while in-hospital, and extensive imaging and laboratory tests characterized the disease in detail. To our knowledge, this is the first report of spontaneous common bile duct perforation witnessed from pre-perforation through definitive management. Conclusion: Physicians and Surgeons should seek out this uncommon diagnosis in the patient with suspected Choledocholithiasis who suddenly become peritoneal on physical exam so that definitive care can be expedited. Keywords: Common bile duct, Biliary peritonitis, Choledocholithiasis

  5. Xanthogranulomatous urethritis and cystitis: a rare clinical and pathological entity

    Directory of Open Access Journals (Sweden)

    Lewkowicz Dorota

    2015-09-01

    Full Text Available Xanthogranulomatous inflammation of the urethra and the urinary bladder is a very rare pathological condition characterized by a chronic inflammatory infiltration composed mainly of foamy macrophages, with the presence of multinucleated giant cells. In a clinical examination, it can mimic urinary bladder carcinoma. This report presents the extremely rare case of a co-existing xanthogranulomatous urethritis and cystitis in a 64-year-old woman with recurrent dysuria, and with the suspicion of malignancy - as indicated on the basis of a cystoscopic examination. The standard treatment of this disorder is surgical resection, but in the presented case, only a diagnostic biopsy was performed. Because of the persistence of clinical symptoms, a cystoscopic examination and biopsy was repeated three times in a two year period. There was no malignancy seen in the repeated biopsies.

  6. Graves’ Disease With Unilateral Involvement: A Rare Entity

    OpenAIRE

    Gülsüm Gönülalan; Mehtap Çakır

    2011-01-01

    Graves’ disease usually affects both lobes of the gland, thus, unilateral Graves’ hyperthyroidism has been reported very rarely. Here, we report a case of Graves’ disease presenting with unilateral involvement of the thyroid gland. Thyroid function tests revealed thyrotoxicosis and scintigraphy with technetium-99m showed increased diffuse unilateral radioisotope uptake in the right lobe with suppressed activity in the left lobe. The patient underwent oral antithyroid drug treatment. Graves’ ...

  7. Macrodystrophia lipomatosa: a rare presentation

    Directory of Open Access Journals (Sweden)

    Sushil G. Kachewar

    2011-07-01

    Full Text Available Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that leads to soft tissue and bony enlargement. As imaging is the key to early diagnosis, findings on various modalities like plain radiographs, CT scan and MRI are highlighted here.

  8. Cardiovocal syndrome – A rare presentation of primary pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    Om Shankar

    2014-05-01

    Full Text Available Primary pulmonary hypertension is a well known entity with characteristic features more common in females presenting commonly with dyspnea. However primary pulmonary hypertension presenting as hoarseness of voice is rare occurring most likely due to compression of left recurrent laryngeal nerve between normal aorta and dilated tense pulmonary artery. Here we are presenting a case of 19 year old boy with primary pulmonary hypertension who presented with hoarseness of voice as predominant symptom.

  9. Infrasellar craniopharyngioma of the posterior nasal septum: a rare entity.

    Science.gov (United States)

    Chiun, Kian Chai; Tang, Ing Ping; Vikneswaran, Tharumalingam; Nurshaline Pauline, H Kipli

    2012-02-01

    To report an unusual location of infrasellar craniopharyngioma in a peadiatric patient. A six-year-old boy presented with persistent bilateral nasal obstruction for one year. Clinical examination revealed a posterior choanal mass arising from septum and the finding was confirmed by paranasal sinuses computed tomography scan. He then underwent wide local excision. Histopathological examination confirmed the diagnosis of craniopharyngioma (adamantinomatous type). There were no signs and symptoms of recurrence after a year of followup. Infracranial craniopharyngioma without sellar involvement is extremely rare. Persistent nasal obstruction without endocrine dysfunction is the common presentation. Radiological imaging is important to diagnose and assess the extent. The mainstay of treatment for infrasellar craniopharyngioma is surgery. Regular follow up is mandatory.

  10. Low-grade myofibroblastic sarcoma of the larynx: A rare entity with review of literature

    Directory of Open Access Journals (Sweden)

    Divya Khosla

    2013-01-01

    Full Text Available Mesenchymal neoplasms of the larynx are rare and make up approximately 0.3% to 1.0% of all malignancies at this location. Low grade myofibroblastic sarcoma (LGMS of larynx is a rare entity. We describe a rare case of LGMS of larynx who presented with complaint of hoarseness of voice. The patient was treated with total laryngectomy plus partial pharyngectomy followed by post-operative radiotherapy. Histopathologically, the lesion was composed of spindle cells that manifested variable cellular anaplasia and expressed smooth muscle actin (SMA and focally S-100.One of the resection limits was involved so patient was given post-operative radiotherapy. The patient is alive and disease free 14 months after surgery. The characteristic clinical, histopathological features and treatment of this case are described with a literature review.

  11. Hemorrhagic Transformation of Scrub Typhus Encephalitis: A Rare Entity.

    Science.gov (United States)

    Kim, H-C; Yoon, K-W; Yoo, D-S; Cho, C-S

    2015-12-01

    Central nervous system (CNS) involvement of scrub typhus infection is well known. Most CNS involvement of scrub typhus infection present as meningitis or encephalitis. We report on a patient suffering from hemorrhagic transformation of intracranial lesions caused by Orientia tsutsugamushi. A 53-year-old female farmer who was infected by scrub typhus was treated with doxycycline and recovered from the systemic illness. However, headache persisted. Brain radiologic studies revealed acute intracranial hemorrhage and enhancing lesion, which implied a CNS involvement. Hemorrhagic transformation of encephalitis by scrub typhus is very rare complication and to our best knowledge, this is the first report of hemorrhagic transformation of scrub typhus encephalitis. Clinician should consider the possibility of hemorrhagic transformation of encephalitis in cases of scrub typhus infection.

  12. Gingival leiomyomatous hamartoma of the maxilla: a rare entity.

    Science.gov (United States)

    Raghunath, Vandana; Manjunatha, Bhari Sharanesha; Al-Thobaiti, Yasser

    2016-05-09

    Hamartoma is a tumour-like malformation appearing as a focal overgrowth of normal cells. Leiomyomatous hamartomas (LHs) are rare in the oral cavity and commonly seen in the Japanese and less than 40 cases have been reported in the Japanese and English literature. The clinical differential diagnoses are irritational (traumatic) fibroma and congenital epulis. It has to be differentiated histopathologically from its neoplastic counterparts and mesenchymomas. Hence, we report such a case of LHs, which presented as a sessile gingival growth occurring in the midline in a 15-year-old girl. The final diagnosis was based on the histopathological appearance which was confirmed by immunohistochemical staining of various markers. A review of the literature of previous cases was also carried out. 2016 BMJ Publishing Group Ltd.

  13. Papillary poroid hidradenoma: a distinct histopathological entity a rare case report.

    Science.gov (United States)

    Nv, Dravid; As, Gadre; Rp, Damle; Ps, Bagale; Kh, Suryawanshi

    2014-03-01

    Papillary poroid hidradenoma is a very rare entity which belongs to poroid neoplasms, which represents 10% of sudoriferous tumours. It can be easily misdiagnosed as a malignant neoplasm. A 34-years-old male presented with an asymptomatic mass over the right chest wall, below the nipple, of 18 months duration. Clinical examination revealed tense cystic swelling which revealed hypoechoic and solid components on imaging. Clinically, a malignancy was suspected and a surgical excision was done. Histology and immunoreactivity to pan cytokeratin, epithelial membrane antigen and carcino- embryonic antigen confirmed the diagnosis. We are reporting this case because of the rarity of its presentation.

  14. Syringomatous carcinoma: Case report of a rare tumor entity | El ...

    African Journals Online (AJOL)

    Syringomatous carcinoma is an extremely invasive tumor, locally destructive and slowly growing adnexal tumour, derived from eccrine sweat glands. It is often mistaken, both clinically and microscopically, for other benign and malignant entities. The tumour recurrence is high due to extensive perineural invasion, but

  15. Rare presentation of a common disease: Idiopathic hypoparathyroidism presenting with extrapyramidal symptoms and status epilepticus

    Directory of Open Access Journals (Sweden)

    Kaushik Ghosh

    2012-01-01

    Full Text Available We report of an 18-year-old male who presented with an epileptiform disorder, features of hypocalcemia, and an extrapyramidal symptom in the form of choreoathetosis. On evaluation he had idiopathic hypoparathyroidism with extensive calcifications in the extrapyramidal system of the brain; basal ganglion, as well as in the cerebral cortex and cerebellum, which is a rare entity. We report the rare presentation of a common disorder, which requires to be considered in evaluating hypoparathyroidism.

  16. Extrapelvic endometriosis: a rare entity or an under diagnosed condition?

    Science.gov (United States)

    2013-01-01

    Endometriosis is a clinical entity characterized by the presence of normal endometrial mucosa abnormally implanted in locations other than the uterine cavity. Endometriosis can be either endopelvic or extrapelvicdepending on the location of endometrial tissue implantation. Despite the rarity of extrapelvic endometriosis, several cases of endometriosis of the gastrointestinal tract, the urinarytract, the upper and lower respiratory system, the diaphragm, the pleura and the pericardium, as well as abdominal scars loci have been reported in the literature. There are several theories about the pathogenesis and the pathophysiology of endometriosis. Depending on the place of endometrial tissue implantation, endometriosis can be expressed with a wide variety of symptoms. The diagnosis of this entity is neither easy nor routine. Many diagnostic methods clinical and laboratory have been used, but none of them is the golden standard. The multipotent localization of endometriosis in combination with the wide range of its clinical expression should raise the clinical suspicion in every woman with periodic symptoms of extrapelvic organs. Finally, the therapeutic approach of this clinical entity is also correlated with the bulk of endometriosis and the locum that it is found. It varies from simple observation, to surgical treatment and treatment with medication as well as a combination of those. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1968087883113362. PMID:24294950

  17. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    Science.gov (United States)

    Mukkunda, Ravindra; Venkitaraman, Ramachandran; Thway, Khin; Min, Toon; Fisher, Cyril; Horwich, Alan; Judson, Ian

    2009-01-01

    Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival. PMID:19478963

  18. Large gangliocytic paraganglioma of the duodenum: A rare entity.

    Science.gov (United States)

    Hernández, Alejandra Gordillo; Lanuza, Eduardo Dominguez-Adame; Matias, Auxiliadora Cano; Huertas, Rosario Perez; Rodriguez, Katherine Maria Gallardo; Perez, Purificacion Gallinato; Mompean, Fernando Oliva

    2015-08-27

    Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.

  19. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Ravindra Mukkunda

    2009-01-01

    Full Text Available Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival.

  20. Acral pityriasis versicolor – A rare clinical presentation

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    Tasleem Arif

    2015-04-01

    Full Text Available Pityriasis versicolor is a superficial fungal infection of the skin caused by the yeast of the genus Malassezia and presents as hypo or hyper pigmented scaly macules. The most commonly affected sites include upper trunk, upper arms, neck and the abdomen. Lesions confined to the acral parts like hands and feet have rarely been reported. In this article the author reports a 40 year old male who presented with multiple hypo pigmented scaly macules confined to the acral parts (hands and wrist. The acral variant of pityriasis versicolor is considered to be a very rare clinical entity which prompted the author to report this case.

  1. Diffuse corpus callosum infarction - Rare vascular entity with differing etiology.

    Science.gov (United States)

    Mahale, Rohan; Mehta, Anish; Buddaraju, Kiran; John, Aju Abraham; Javali, Mahendra; Srinivasa, Rangasetty

    2016-01-15

    Infarctions of the corpus callosum are rare vascular events. It is relatively immune to vascular insult because of its rich vascular supply from anterior and posterior circulations of brain. Report of 3 patients with largely diffuse acute corpus callosum infarction. 3 patients with largely diffuse acute corpus callosum infarction were studied and each of these 3 patients had 3 different aetiologies. The 3 different aetiologies of largely diffuse acute corpus callosum infarction were cardioembolism, tuberculous arteritis and takayasu arteritis. Diffuse corpus callosum infarcts are rare events. This case series narrates the three different aetiologies of diffuse acute corpus callosum infarction which is a rare vascular event. Copyright © 2015 Elsevier B.V. All rights reserved.

  2. Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias

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    Karina Portillo

    2015-01-01

    Full Text Available Pleuroparenchymal fibroelastosis (PPFE is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs. PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT, chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.

  3. Pseudomyxoma peritonei (PMP) – a rare entity | Ebrahim | SA ...

    African Journals Online (AJOL)

    Pseudomyxoma peritonei (PMP) is a rare complication of mucinous tumours of appendiceal or ovarian origin that results in peritoneal and omental implants. Clinical morbidity and mortality arise from the fact that copious amounts of extracellular and peritoneal mucin result in distortion and loss of function of visceral organs.

  4. Grade V cherubism: A rare and aggressive entity

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    Karthikeya Patil

    2009-01-01

    Full Text Available Cherubism is a non-neoplastic, rare, hereditary childhood disease of bone characterized by bilateral enlargement of the jaws caused by bone degradation and replacement by fibrous tissue. The affected child is reminiscent of the cherub portrayed in Renaissance art. We report the clinicopathological and radiographic features of cherubism and review of literature.

  5. Myxofibrosarcoma of maxilla: A case report of rare entity

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    Venkateswarlu Nallapu

    2015-01-01

    Full Text Available Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients that rarely affect the oral cavity. In this article we describe a case of primary myxofibrosarcoma of maxilla in a 19-year-old boy. Microscopically the tumor showed loosely arranged spindle-shaped tumor cells with mild pleomorphic spindle-shaped nuclei, some with minute nucleoli in a fibromyxoid stroma. The cells were strongly immunoreactive for vimentin and negative for CD-68, S-100 protein and desmin. Based on clinical, histological and immunohistochemical findings, a final diagnosis of myxofibrosarcoma was established. It is an unusual aggressive variant that frequently recurs and metastasizes, reinforcing the importance of its correct diagnosis. We report a rare case of myxofibrosarcoma in maxilla, with emphasis on immunohistochemical findings.

  6. Giant Interfrontal Encephalocele in an Infant: A Rare Entity.

    Science.gov (United States)

    Faheem, Mohd; Singh, Sunil Kumar; Ojha, Bal Krishna; Chandra, Anil; Srivastava, Chhitij; Jaiswal, Manish; Zeeshan, Qazi

    2016-01-01

    Interfrontal encephalocele is one of the rare varieties of anterior encephalocele, and a giant interfrontal encephalocele is extremely rare. The authors could find only one case report of giant interfrontal encephalocele in the literature. Anterior encephaloceles are more prevalent in South-East Asia and some northern parts of India. Giant encephalocele poses a great challenge to neurosurgeons and neuroanesthetists during surgery, as these infants usually have a low birth weight and a large sac, thus making the infant prone to hypothermia and blood loss among other risks. We encountered a patient with a giant interfrontal encephalocele aged 1 month. The rarity of this case prompted us to this report. © 2016 S. Karger AG, Basel.

  7. Soft-Tissue Chondroma of Anterior Gingiva: A Rare Entity

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    Dhana Lakshmi Jeyasivanesan

    2018-01-01

    Full Text Available Soft-tissue chondroma is a rare, benign, slow-growing tumor made up of heterotopic cartilaginous tissue. It occurs most commonly in the third and fourth decades in the hands and feet. Oral soft-tissue chondromas are uncommon and soft-tissue chondroma of gingiva is extremely uncommon. Here, we report an unusual case of soft-tissue chondroma of gingiva in a 50-year-old woman.

  8. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    OpenAIRE

    Ravindra Mukkunda; Ramachandran Venkitaraman; Khin Thway; Toon Min; Cyril Fisher; Alan Horwich; Ian Judson

    2009-01-01

    Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patien...

  9. Bifid mandibular canal: a rare or underestimated entity?

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    Ibrahim Nasseh

    2016-10-01

    Full Text Available One of the rare anatomical variations that can be of significant importance for the dentist is the bifid mandibular canal. Many complications can occur from this condition such as failure of anesthesia when performing inferior alveolar nerve block, difficulties during the surgical extraction of the third mandibular molar, and during implants placement. Therefore, good knowledge of this condition is essential. In this report, we describe the radiographic finding of a unilateral bifid mandibular canal.

  10. Riedel thyroiditis: Fine needle aspiration findings of a rare entity.

    Science.gov (United States)

    Weidner, Anna-Sophie; Molina, David; DeSimone, Robert A; Cohen, Marc A; Giorgadze, Tamar; Scognamiglio, Theresa; Hoda, Rana S

    2015-09-01

    Riedel thyroiditis is a rare fibrosing disorder characterized by extension of the fibroinflammatory process beyond the thyroid capsule. Due to the nature of this lesion, fine-needle aspiration often yields scant material and may be interpreted as non-diagnostic. In this report, we describe cytologic features that allow the cytopathologist to favor a diagnosis of Riedel thyroiditis, thereby guiding appropriate further work-up and management. © 2015 Wiley Periodicals, Inc.

  11. Cavitatory mesenteric lymph node syndrome: A rare entity

    International Nuclear Information System (INIS)

    Vibhuti; Vishal, Kalia; Gill, Anakhvir

    2010-01-01

    Celiac disease is a gluten sensitive enteropathy that involves an abnormal immunological response to glutens in wheat, rye etc. It predominantly involves the small intestinal mucosa, though, extra luminal manifestations can also occur. One rare extraluminal manifestation is cavitatory mesenteric lymph node syndrome. It occurs in refractory celiac disease and is associated with poor prognosis due to various complications. The diagnosis is often made on imaging when cystic mesenteric lymph nodes with fat-fluid levels are seen and this can then be confirmed by histopathological examination. We recently had a typical case where we were able to make this diagnosis

  12. Congenital insensitivity to pain: Case report of a rare entity

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    Swati Dahiya

    2018-01-01

    Full Text Available Hereditary sensory and autonomic neuropathies (HSANs are a group of disorders characterized by insensitivity to noxious stimuli and autonomic dysfunction, associated with pathological abnormalities of the peripheral nerves. Five types of HSAN have been reported in literature, out of which Type V known as congenital insensitivity to pain (CIP is a rare autosomal recessive condition. Self-mutilation is an invariable feature of this disorder, involving the teeth and orofacial structures. This case report describes a case of a 6-year-old girl with CIP brought by her parents for prostheses to replace her self-extracted primary teeth.

  13. Ipsilateral Floating Hip and Floating Knee – A Rare Entity

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    Yashavantha Kumar

    2013-04-01

    Full Text Available Introduction: Ipsilateral floating hip and floating knee are very rare injuries. These injuries so uncommon that only three cases of similar kind have been reported. These injuries are due to high velocity injuries following motor vehicle accidents. Management of such complex injuries is a challenging task even in experienced hands as there are no standard treatment guidelines for such fractures. Case Report: We hereby report a 20 yr old male who sustained ipsilateral floating hip and ipsilateral floating knee injuries following motor vehicle accident. Patient was stabilized initially and later taken up for surgery. Patient was treated with interlocking nail for femur and tibia in the same sitting whereas acetabulam fracture was managed conservatively. At five months all the fractures united well with restoration of good range of motion in both hip and knee. Conclusion: Ipsilateral floating knee and floating hip are very rare injuries seen following high velocity motor vehicle accidents. There are no standard guidelines for treatment of those fractures as only a few cases of similar kind have been reported in literature. Early fixation and aggressive mobilization ensures fracture union and fewer complications. Keywords: Floating hip, Floating Knee, Ipsilateral.

  14. A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

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    Ghulam Rehman Mohyuddin

    2012-01-01

    Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.

  15. Skull Base Langerhans Cell Histiocytosis with Diabetes Insipidus and Panhypopituitarism- A Rare Clinical Entity

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    Anirban Ghosh

    2017-12-01

    Case Report A 16 year old male presented with diminished vision, bilateral ptosis, left sided lateral rectus palsy, hypoesthesia of trigeminal nerve with nasal obstruction for last 5 months. There was polypoidal, bleeding mass in both nasal cavities. Contrast enhanced CT Scan showed a large homogenous mass arising from sphenoid extending into cavernous sinus and the suprasellar region. Endoscopic nasal biopsy revealed abundant Langerhans cell histiocytes, macrophages, neutrophils. Chemotherapy and radiotherapy were administered. But within 2 months the patient presented with Cushingoid features and further diminution of vision. Detailed work-up revealed Hypogonadotrophic hypogonadism and diabetes insipidus. Debulking of the tumour was done and left optic nerve decompression was done. PET scan was performed and showed large, well defined mass with increased FDG uptake in the skull base with suprasellar extension, reaching upto petrous temporal bone and causing bony erosion of ethmoid and sphenoid sinuses. Patient was then advised adjuvant chemotherapy.   Discussion Langerhans cell histiocytosis is a rare group of disorders characterised by abnormal clonal proliferation and accumulation of abnormal dendritic cells. Involvement of base of skull is even rarer. Though diabetes insipidus has been reported in Langerhans cell histiocytosis involving pituitary, panhypopituitarism is rare. These combinations of extensive Langerhans cell histiocytosis of base skull with clinical features of Diabetes insipidus and panhypopituitarism makes this case a rare clinical entity.

  16. A rare presentation of hydatid cyst

    International Nuclear Information System (INIS)

    Sabir, N.; Yildirim, B.; Alatas, E.; Cetin, B.

    2005-01-01

    Hydatid disease, although known to occur in most body areas, is extremely rare in the female reproductive system. There are different modes of presentation for the disease; however, we report and discuss a case presented with cystic vesicles passing through the vagina, which is considered as a rare presentation for secondary involvement of the uterus and both ovaries. We confirmed diagnosis with radiological examinations and serological tests. We operated on the patient, and studied the excised cysts microscopically. The gynecologist should be aware of hydatid cyst when vaginally passing a grape like vesicle is presented by the patient. (author)

  17. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

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    Gultekin Gulbahar

    2015-01-01

    Full Text Available First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.

  18. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures.

    Science.gov (United States)

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.

  19. Odontoameloblastoma: A rare case with unusual presentation

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    Supreet Jain

    2016-01-01

    Full Text Available The odontoameloblastoma (OA, also known as ameloblastic odontoma, is a very rare odontogenic tumor that is similar to ameloblastoma in its locally aggressive behavior. OA includes odontogenic ectomesenchyme in addition to odontogenic epithelium that resembles an ameloblastoma both in structure and in behavior. Its clinical presentation, however, often mimics the more innocuous odontoma, and hence, the recognition of its aggressive nature is commonly only ascertained after its histopathologic diagnosis following enucleation. This paper presents a rare case of OA with unusual clinical and radiological features.

  20. Rare presentation of pancreatic schwannoma: a case report

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    Tofigh Arash

    2008-08-01

    Full Text Available Abstract Introduction Schwannoma is a rare tumor among pancreatic neoplasms. Schwannomas vary in size, and most of them are cystic, mimicking pancreatic cystic lesions. Generally, a definitive diagnosis is made at the time of histological analysis. The mainstay treatment is surgical resection. Case presentation We report an unusual presentation of pancreatic schwannoma with abdominal pain and several episodes of cholangitis in a 54-year-old Caucasian (Iranian man. The condition was not diagnosed pre-operatively and Whipple's procedure was performed. Conclusion Pancreatic schwannoma is an important clinical entity to include in the differential diagnosis of pancreatic lesions. Pre-operative diagnosis is difficult but computed tomographic findings may be helpful. The tumor may also have atypical and rare presentations, such as cholangitis and weight loss. For benign tumors, simple enucleation is usually adequate, whereas malignant tumors require standard oncological resection.

  1. A rare presentation of methanol toxicity

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    Nikhil Gupta

    2013-01-01

    Full Text Available Methanol is a highly toxic alcohol resembling ethanol in smell and taste. Methanol poisoning is a lethal form of poisoning that can cause severe metabolic acidosis, visual disturbances, and neurological deficit. Brain lesions typically described in methanol toxicity are in the form of hemorrhagic and non-hemorrhagic necrosis of the basal ganglia and sub-cortical white matter. To our knowledge, lesions in the parietal, temporal, or frontal areas of cerebrum and cerebellar hemispheres have been rarely reported so far. We herewith report this rare presentation.

  2. An Erupted Dilated Odontoma: A Rare Presentation

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    Gaurav Sharma

    2016-01-01

    Full Text Available A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.

  3. Role of Imaging and Cytogenetics in Evaluation of DiGeorge Syndrome - A Rare Entity in Clinical Practice.

    Science.gov (United States)

    Ramachandran, Rajoo; Babu, Sellappan Rajamanickam; Ilanchezhian, Subramanian; Radhakrishnan, Prabhu Radhan

    2015-01-01

    DiGeorge syndrome is a congenital genetic disorder that affects the endocrine system, mainly the thymus and parathyroid glands. The syndrome produces different symptoms, which vary in severity and character between patients. It manifests with craniofacial dysmorphism and defects in the heart, parathyroid, and thymus. Patients can present with a palatal deformity and nasal speech. This rare entity is caused mainly due to deletion of chromosome 22q11.2. Radiographic evaluation of DiGeorge syndrome is necessary to define aberrant anatomy, evaluate central nervous system, craniofacial abnormalities, musculoskeletal system, and cardiothoracic contents. It also helps in planning surgical procedures and surgical reconstructions. We report a case of DiGeorge syndrome in a 4-month-old neonate and discuss the clinical, imaging, and cytogenetic findings that helped in the diagnosis of this rare entity.

  4. Role of Imaging and Cytogenetics in Evaluation of DiGeorge Syndrome - A Rare Entity in Clinical Practice

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    Rajoo Ramachandran

    2015-01-01

    Full Text Available DiGeorge syndrome is a congenital genetic disorder that affects the endocrine system, mainly the thymus and parathyroid glands. The syndrome produces different symptoms, which vary in severity and character between patients. It manifests with craniofacial dysmorphism and defects in the heart, parathyroid, and thymus. Patients can present with a palatal deformity and nasal speech. This rare entity is caused mainly due to deletion of chromosome 22q11.2. Radiographic evaluation of DiGeorge syndrome is necessary to define aberrant anatomy, evaluate central nervous system, craniofacial abnormalities, musculoskeletal system, and cardiothoracic contents. It also helps in planning surgical procedures and surgical reconstructions. We report a case of DiGeorge syndrome in a 4-month-old neonate and discuss the clinical, imaging, and cytogenetic findings that helped in the diagnosis of this rare entity.

  5. A Rare Cutaneous Adnexal Tumour with a Rare Presentation

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    Vijayalaxmi S. Patil

    2016-04-01

    Full Text Available Proliferating Trichilemmal Tumour (PTT is a rapidly growing large cutaneous adnexal neoplasm. Although biologically considered as benign, it may be locally aggressive. Malignant transformation of these lesions, known as Malignant Proliferating Trichilemmal Tumour (MPTT has rarely been reported. So far in the literature, only 39 well-documented cases of MPTT have been reported. MPTT has been stated to be a neoplasm of the older age group according to review of the literature. We present a case of MPTT in a young male. A 25 year old male presented with a scalp swelling of 2 years duration with a recent rapid enlargement. The swelling was excised and histopathological examination of the excised specimen revealed features of MPTT. The differential diagnosis of MPTTis squamous cell carcinoma as both share common features. Accurate diagnosis of MPTT is essential since it has a tendency to metastasize and recur more frequently than squamous cell carcinoma.

  6. Revisiting Cementoblastoma with a Rare Case Presentation

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    Vijayanirmala Subramani

    2017-01-01

    Full Text Available Cementoblastoma is a rare benign odontogenic neoplasm which is characterized by the proliferation of cellular cementum. Diagnosis of cementoblastoma is challenging because of its protracted clinical, radiographic features, and bland histological appearance; most often cementoblastoma is often confused with other cementum and bone originated lesions. The aim of this article is to overview/revisit, approach the diagnosis of cementoblastoma, and also present a unique radiographic appearance of a cementoblastoma lesion associated with an impacted tooth.

  7. Primary intraosseous squamous cell carcinoma in odontogenic keratocyst: A rare entity

    Science.gov (United States)

    Saxena, Chitrapriya; Aggarwal, Pooja; Wadhwan, Vijay; Bansal, Vishal

    2015-01-01

    Squamous cell carcinoma (SCC) arising from the wall of an odontogenic cyst (also known as primary intraosseous carcinoma) is a rare tumor which occurs only in jaw bones. This tumor was first described by Loos in 1913 as a central epidermoid carcinoma of the jaw. Primary intraosseous carcinomas (PIOC) may theoretically arise from the lining of an odontogenic cyst or de novo from presumed odontogenic cell rests. According to the new histological classification of tumors of the World Health Organization, odontogenic keratocyst is nowadays considered a specific odontogenic tumor and the PIOC derived from it is considered as a specific entity which is different from other PIOCs derived from the odontogenic cysts. The following report describes a case of such extremely rare entity that is primary intraosseous SCC of the mandible derived from an OKC in a 60-year-old male patient with brief review of literature. PMID:26980976

  8. Pure primary small cell carcinoma of urinary bladder: A rare diagnostic entity

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    Sonia Gon

    2013-01-01

    Full Text Available Small cell carcinoma of the bladder is a rare, aggressive, poorly differentiated neuroendocrine neoplasm accounting for only 0.3-0.7% of all bladder tumors. Since the tumor is very rare, pathogenesis is uncertain. Small cell carcinomas of the urinary bladder are mixed with classic urothelial carcinomas or adenocarcinomas of the bladder in 68% cases, making pure primary small cell carcinoma even a rarer entity. The unknown etiology and natural history of small cell carcinoma of the urinary bladder represent a challenge both to the pathologist and urologists for its diagnosis and treatment, respectively.

  9. Foreign body in the nasopharynx- A rare entity: a Case Report

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    Soumik Saha

    2013-06-01

    Full Text Available Abstract Nasopharynx is an exceptionally rare anatomical location for foreign body impactation . As an otorhinolaryngologist, one may face challenges in diagnosis and removal of foreign body from airways . A metallic foreign body after being inhaled and ultimately being lodged in the nasopharynx is a rare entity. We report a case of nasopharyngeal foreign body(NFB in a 1½ year old male child. The foreign body was diagnosed by diagnostic nasal endoscopy and documented by X-Ray of nasopharynx - lateral view [FIG.-2] and the same was removed under general anaesthesia. While keeping the child in Rose's position. Keywords foreign body nasopharynx

  10. A Rare Clinical Presentation of Darier's Disease

    Science.gov (United States)

    Ferizi, Mybera; Begolli-Gerqari, Antigona; Luzar, Bostjan; Kurshumliu, Fisnik; Ferizi, Mergita

    2013-01-01

    Darier's disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions are presented as suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by “corps ronds and grains”. This paper reports a case of a 53-year-old woman that was admitted to our clinic with more than 10-year history of keratotic papules, presented on the hands and feet, nose, ears, genitalia, and whitish lesions on palatal mucosae. PMID:23573430

  11. Ulcerative giant solitary trichoepithelioma of scalp: a rare presentation

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    Sundeep Chowdhry

    2016-07-01

    Full Text Available Trichoepithelioma is a trichogenic tumor which arises from the inferior segment of hair follicle epithelium as hamartoma. Giant solitary trichoepithelioma (GST has been defined as a solitary trichoepithelioma with a diameter greater than 2 cm. A 49-year-old female presented with a slow growing skin coloured swelling on the scalp of 8 years duration with recent history of ulceration and occasional bleeding. The local examination revealed a single well defined nodular swelling which was irregular in shape measuring approximately 2 × 2.5 cm. Histopathology from biopsy specimen revealed dark basaloid cells with scanty cytoplasm and darkly stained nucleus arranged in nests with horn cysts lacking high-grade atypia and mitosis, which was consistent with features of trichoepithelioma. Giant solitary trichoepithelioma of scalp is itself a rare entity and the present case is being reported with the additional component of ulceration in the lesion.

  12. Bilateral Supernumerary Kidney: A Very Rare Presentation

    International Nuclear Information System (INIS)

    Keskin, Suat; Batur, Abdussamet; Keskin, Zeynep; Koc, Abdulkadir; Firat Ozcan, Irfan

    2014-01-01

    To our knowledge, bilateral supernumerary kidney is a very rare renal abnormality and there are five cases presented in the literature. It is difficult to diagnose supernumerary kidney and clinicians have not detected most cases preoperatively. Laboratory and imaging studies were acquired and carefully examined. The normal laboratory tests were found. Emergency ultrasonography was performed and they revealed no signs of parenchymal abnormality in both kidneys. Serial imaging study including enhanced computed tomography (CT) was performed. An imaging study identified bilateral supernumerary kidney with expanded collecting systems. On each side, significant rotation anomaly was found. In addition, there were two different renal arteries originating from the aorta. This report presents radiological determinations of supernumerary kidney bilaterally in a young man. We think that CT commonly appears to be enough for the diagnosis of supernumerary kidneys

  13. Bilateral Supernumerary Kidney: A Very Rare Presentation

    Science.gov (United States)

    Keskin, Suat; Batur, Abdussamet; Keskin, Zeynep; Koc, Abdulkadir; Firat Ozcan, Irfan

    2014-01-01

    To our knowledge, bilateral supernumerary kidney is a very rare renal abnormality and there are five cases presented in the literature. It is difficult to diagnose supernumerary kidney and clinicians have not detected most cases preoperatively. Laboratory and imaging studies were acquired and carefully examined. The normal laboratory tests were found. Emergency ultrasonography was performed and they revealed no signs of parenchymal abnormality in both kidneys. Serial imaging study including enhanced computed tomography (CT) was performed. An imaging study identified bilateral supernumerary kidney with expanded collecting systems. On each side, significant rotation anomaly was found. In addition, there were two different renal arteries originating from the aorta. This report presents radiological determinations of supernumerary kidney bilaterally in a young man. We think that CT commonly appears to be enough for the diagnosis of supernumerary kidneys. PMID:25780543

  14. A rare presentation of craniopharyngioma: delayed puberty

    Science.gov (United States)

    İnci, Mehmet Fatih; Özkan, Fuat; Bozkurt, Selim; Demir, Caner Feyzi

    2012-01-01

    Craniopharyngiomas are the most frequently encountered suprasellar tumours in children. Owing to the slow growth rate of these tumours, they are often quite large before becoming symptomatic. They are more common among children and older adults (55–74 years). Depending upon the direction of growth and tumour size, craniopharyngiomas can affect the hypothalamus, pituitary stalk, optic nerves and chiasm and carotid arteries. Compression of these neural and vascular structures frequently precipitates endocrine disorders, visual loss and an increased intracranial pressure. Hypopituitarism leading to a delayed puberty is a rare presentation of craniopharyngioma. The diagnosis of craniopharyngioma is usually made with the classic radiological imaging features based on CT and MRI. PMID:23195827

  15. Multifocal bilateral metatarsal tuberculosis: a rare presentation.

    Science.gov (United States)

    Vijay, Vipul; Sud, Alok; Mehtani, Anil

    2015-01-01

    Tuberculosis, or phthisis (consumption) as it was popularly known in the Greek era, has been endemic in Southeast Asia and Sub-Saharan Africa; however, the human immunodeficiency virus epidemic has seen the re-emergence of this disease in the areas in which it was not very commonly reported. With this, the need for understanding and treatment of rare presentations of tuberculosis has become of paramount importance to achieve the World Health Organization millennium goal of a "reversal of incidence by 2015." Foot involvement has been reported in 0.1% to 0.3% of extrapulmonary cases. Multifocal lesions have an incidence of histopathologic owing to the paucibacillary nature of the disease. Early identification and treatment with antitubercular drugs will normally result in a good cosmetic and functional result. Copyright © 2015 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

  16. Spontaneous Pneumomediastinum with a Rare Presentation

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    Ehsan Bolvardi

    2014-01-01

    Full Text Available Spontaneous pneumomediastinum is an unusual and benign condition in which air is present in mediastinum. A 20-year-old male patient presented to ED with complaint of hoarseness and odynophagia from the day before, after weightlifting. The patient was nonsmoker and denied history of other diseases. On physical examination he had no dyspnea with normal vital signs. Throat examination and pulmonary auscultation were normal and no crepitation was palpable. We could not find subcutaneous emphysema in neck and chest examination. In neck and chest X-ray we found that air is present around the trachea. There was no apparent pneumothorax in CXR. In cervical and chest CT free air was present around trachea and in mediastinum. Subcutaneous emphysema was also evident. But there was no pneumothorax. The patient was admitted and went under close observation, oxygen therapy, and analgesic. The pneumomediastinum and subcutaneous emphysema gradually resolved within a week by conservative therapy and he was discharged without any complication. Many different conditions could be trigged because of pneumomediastinum but it is rarely seen in intense physical exertion such as weightlifting and bodybuilding. Two most common symptoms are retrosternal chest pain and dyspnea. But the patient here complained of hoarseness and odynophagia.

  17. Pleuroperitoneal Mesothelioma: A Rare Entity on 18F-FDG PET/CT

    Science.gov (United States)

    Sahoo, Manas Kumar; Mukherjee, Anirban; Girish; Parida, Kumar; Agarwal, Krishan Kant; Bal, Chandrasekhar; Tripathi, Madhavi; Das, Chandan Jyoti; Shamim, Shamim Ahmed

    2017-01-01

    Pleuroperitoneal mesothelioma is an extremely rare entity. Only few cases are reported worldwide. We hereby represent a case of pleural mesothelioma referred for F-18-Fluorodeoxyglucose positron emission tomography/computed tomography for response evaluation. Diffuse F-18-Fluorodeoxyglucose avid peritoneal and omental thickening noted which subsequently turned out to be mesothelial involvement on peritoneal biopsy. This case demonstrates the role of F-18-Fluorodeoxyglucose positron emission tomography/computed tomography in detecting other sites of involvement in case of malignant mesothelioma. PMID:28242997

  18. Myxedema Coma with Reversible Cardiopulmonary Failure: a Rare Entity in 21St Century.

    Science.gov (United States)

    Dhakal, Prajwal; Pant, Manisha; Acharya, Pranab Sharma; Dahal, Sumit; Bhatt, Vijaya Raj

    2015-09-01

    Myxedema coma, a rare entity in 21st century in developed nations, is a decompensated phase of hypothyroidism with high mortality rates. We describe a young woman with myxedema, who developed respiratory failure, congestive heart failure and significant pericardial effusion, some of the uncommon manifestations. Decreased cardiac contractility can result in cardiomyopathy and heart failure. As illustrated by this case, myxedema can also result in significant pericardial effusion due to increased vascular permeability. Myxedema can further be complicated by alveolar hypoventilation and respiratory failure secondary to the lack of central drive as well as respiratory muscle weakness. Prompt therapy with thyroid hormone replacement, glucocorticoid therapy, aggressive supportive care and management of the precipitating event can save lives and reverse the cardiopulmonary symptoms, as in our patient. Hence, physicians should have a high index of suspicion for myxedema coma in patients with unexplained cardiopulmonary failure. Our report is, therefore, aimed at bringing awareness about the rare but fatal manifestations of myxedema coma.

  19. Macrodystrophia Lipomatosa: A rare presentation | Kachewar ...

    African Journals Online (AJOL)

    Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in ...

  20. Present Status of Inclusive Rare $B$ Decays

    CERN Document Server

    Hurth, Tobias

    2003-01-01

    We give a status report on inclusive rare B decays, highlighting recent developments and open problems. We focus on the decay modes $B \\to X_{s,d} \\gamma$, $ B \\to X_s \\ell^+\\ell^-$ and $B \\to X_s \

  1. Angiofibroma on cheek mucosa: A rare entity and its management with laser

    Directory of Open Access Journals (Sweden)

    Rajesh Kumar Thakur

    2014-01-01

    Full Text Available A rare presentation of an angiofibroma in the oral cavity is reported, which was treated with a diode laser. The benefits of laser treatment include bloodless procedure with instant precise coagulation of vessels. Although rare and unusual, it is suggested that angiofibroma should be included as one of the differential diagnoses of soft tissue swellings in the oral cavity.

  2. Adult primary hypoparathyroidism: A rare presentation

    Directory of Open Access Journals (Sweden)

    Ashima Datey Chakrabarty

    2013-01-01

    Full Text Available The common causes of stridor in adults are abscesses or swelling of upper airway, tumors, paralysis or malfunction of vocal cords. Laryngospasm due to hypocalcemia is a rare cause of stridor in adults, although occasionally reported in the neonates. We report an elderly lady having stridor and laryngospasm, secondary to acquired hypoparathyroidism and secondary hypocalcemia, without risk factors for hypoparathyroidism such as recent neck surgery or irradiation. We did an extensive review of literature to find only a few cases of acquired primary hypoparathyroidism in adults with the only complaint being stridor. This case underlines the fact that a common symptom like stridor rarely occurs due to uncommon causes. This case is being reported for its rarity and amenability to complete cure in event of correct diagnosis.

  3. Adult primary hypoparathyroidism: A rare presentation

    OpenAIRE

    Chakrabarty, Ashima Datey

    2013-01-01

    The common causes of stridor in adults are abscesses or swelling of upper airway, tumors, paralysis or malfunction of vocal cords. Laryngospasm due to hypocalcemia is a rare cause of stridor in adults, although occasionally reported in the neonates. We report an elderly lady having stridor and laryngospasm, secondary to acquired hypoparathyroidism and secondary hypocalcemia, without risk factors for hypoparathyroidism such as recent neck surgery or irradiation. We did an extensive review of l...

  4. Congenital Uterine Arteriovenous Malformation Presenting as Postcoital bleeding: A Rare Presentation of a Rare Clinical Condition

    Directory of Open Access Journals (Sweden)

    Neha Agarwal

    2017-01-01

    Full Text Available Congenital uterine arteriovenous malformation (AVM is an extremely rare condition with <100 cases documented in literature. We report multiparous women presenting to us with a history of postcoital bleed. Initial Doppler ultrasonography was consistent with features suggestive of AVM. Subsequently, computed tomography (CT angiography confirmed the diagnosis. Embolization was chosen as the treatment because of the large extension of AVM and the risk of hemorrhage during hysterectomy. The patient was discharged in a stable condition with a plan of repeat embolization in the next setting. At 6 and 12 weeks of follow-up, she did not experience any further episodes of bleed. The purpose of this case report is to highlight the salient clinical features, diagnosis, and the management options available for this rare clinical condition.

  5. Simultaneous or Early Sequential Rupture of Multiple Intracranial Aneurysms: A Rare and Insufficiently Understood Entity.

    Science.gov (United States)

    Hou, Kun; Zhao, Jinchuan; Zhang, Yang; Zhu, Xiaobo; Zhao, Yan; Li, Guichen

    2016-05-01

    Simultaneous or early sequential rupture of multiple intracranial aneurysms (MIAs) is encountered rarely, with no more than 10 cases having been reported. As a result of its rarity, there are a lot of questions concerning this entity need to be answered. A 67-year-old woman was admitted to the First Hospital of Jilin University (Eastern Division) from a local hospital after a sudden onset of severe headache, nausea, and vomiting. Head computed tomography (CT) at the local hospital revealed diffuse subarachnoid hemorrhage (SAH) that was concentrated predominately in the suprasellar cistern and interhemispheric fissure. During her transfer to our hospital, she experienced another episode of sudden headache. CT on admission to our hospital revealed that the SAH was increased with 2 isolated hematomas both in the interhemispheric fissure and the left paramedian frontal lobe. Further CT angiography and intraoperative findings were in favor of early sequential rupture of 2 intracranial aneurysms. To further elucidate the characteristics, mechanism, management, and prognosis of this specific entity, we conducted a comprehensive review of the literature. The mechanism of simultaneous or early sequential rupture of MIAs is still obscure. Transient elevation of blood pressure might play a role in the process, and preventing the sudden elevation of blood pressure might be beneficial for patients with aneurysmal SAH and MIAs. The management of simultaneously or early sequentially ruptured aneurysms is more complex for its difficulty in responsible aneurysm determination, urgency in treatment, toughness in intraoperative manipulation and poorness in prognosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. A case of Occupational Methemoglobinemia (MetHb: A Rare Entity and Unique Treatment

    Directory of Open Access Journals (Sweden)

    Suvarna Patil

    2017-11-01

    Full Text Available Methemoglobinemia is acute emergency which have precise and effective treatment if instituted in time. Methemoglobinemia due to chemical exposure is a known entity. But it required a high index of suspicion to look for it in busy casualty. Treatment with methylene blue is safe and truly lifesaving if instituted in time. Here we are presenting a case of Occupational methemoglobinemia who was treated successfully.

  7. A rare clinical presentation of sarcoidosis; gingivitis.

    Science.gov (United States)

    Güzel, Aygül; Köksal, Nurhan; Aydın, Davut; Aslan, Kerim; Gören, Fikret; Karagöz, Filiz

    2013-10-01

    Gingivitis due to sarcoidosis is a relatively rare condition. Gingivitis or isolated gingival involvement may be the first sign of systemic sarcoidosis. We report the case of a 37 year-old woman with isolated gingivitis due to sarcoidosis confirmed by biopsy. Following treatment with a systemic corticosteroid (prednisolone 40 mg/day), all clinical and radiologic findings were completely improved. In cases of chronic and intractable gingivitis, systemic sarcoidosis should be suspected. It should be confirmed with a biopsy, and the patient should be referred to a chest disease clinic to exclude other organ involvement. Copyright © 2013 Elsevier Inc. All rights reserved.

  8. Lymphangioma causing duodenal obstruction in adult, rare presentation

    Directory of Open Access Journals (Sweden)

    Prashant W Khade

    2016-01-01

    Full Text Available A submucosal lymphangioma is a rare pathology in the alimentary tract. It is a benign entity of the lymphatic system. A duodenal lymphangioma is extremely rare and has an unknown etiology. Clinical and laboratory findings are nonspecific, and they are incidentally found by endoscopy and their treatment is surgical excision. Small lesions are often asymptomatic. Recently, the authors experienced a 62-year-old man, who had a duodenal lymphangioma, showing gastric outlet obstruction symptoms. A multidetector computed tomography abdomen study revealed a single submucosal cystic lesion in second part of duodenum. The lesion was successfully excised after Whipple′s procedure. Histopathology confirmed the diagnosis of lymphangioma.

  9. Orthokeratinized odontogenic cyst: A rare presentation

    Directory of Open Access Journals (Sweden)

    Neha Bhasin

    2014-01-01

    Full Text Available Orthokeratinized Odontogenic Cyst (OOC is a developmental cyst of odontogenic origin and was initially defined as the uncommon orthokeratinized variant of the Odontogenic Keratocyst (OKC, until the World Health Organization′s (WHO′s classification in 2005, where it was separated from the Keratocystic Odontogenic Tumor (KCOT. It is a relatively uncommon developmental cyst comprising of only 0.4% of all odontogenic cysts. It is rather mystifying that its radiographic features are similar to the dentigerous cyst and histological characteristics are similar to the odontogenic keratocyst; and it has inconsistent cytokeratin expression profiles overlapping with both the dentigerous cyst and odontogenic keratocyst as well as with the epidermis. It has a predilection for the posterior mandibular region. This is a report of a rare case of OOC in an unusual maxillary anterior region, with emphasis on its biological characteristics.

  10. Two rare entities in the same palate lesion: hyalinizing-type clear cell carcinoma and necrotizing sialometaplasia.

    Science.gov (United States)

    Arpaci, Rabia Bozdoğan; Kara, Tuba; Porgali, Canan; Serinsoz, Ebru; Polat, Ayse; Vayisoglu, Yusuf; Ozcan, Cengiz

    2014-05-01

    Hyalinizing clear cell carcinoma is a low-grade malignant epithelial neoplasm of the salivary glands. The tumor has epithelial cells and lacks myoepithelial cells. Necrotizing sialometaplasia is a benign, self-limiting lesion of the salivary glands. The clinical and histologic features mimic those of mucoepidermoid carcinoma or squamous cell carcinoma. The importance of these entities are the rarity of both of them and their potential to be misdiagnosed as other lesions. Pathologists and clinicians should be aware of these entities to prevent misdiagnosis. This is the first clinical report of 2 rare and consecutive different entities of the same location on the hard palate to our knowledge.

  11. Primary epithelial myoepithelial carcinoma of lung, reporting of a rare entity, its molecular histogenesis and review of the literature.

    Science.gov (United States)

    Arif, Farzana; Wu, Susan; Andaz, Shahriyour; Fox, Stewart

    2012-01-01

    Primary epithelial myoepithelial carcinoma of lung is a rare entity and is thought to arise from the submucosal bronchial glands distributed throughout the lower respiratory tract. Because of the rarity of this tumor, we describe one case of epithelial myoepithelial carcinoma arising in the bronchus intermedius and presenting as an endobronchial mass. A 57-year-old male patient presented with an incidental finding of an endobronchial mass located in the lumen of the right lower lobe bronchus and caused near total luminal occlusion of the bronchus. An endobronchial carcinoid tumor was entertained clinically. Subsequently the patient underwent an uneventful videothoracoscopic lobectomy of lower and middle lobes of the right lung. Morphologically and immunohistochemically the tumor was characterized by two cell populations with epithelial and myoepithelial cells forming duct-like structure. The final diagnosis of epithelial myoepithelial carcinoma of lung was rendered.

  12. Tentorial and dural calcification with tertiary hyperparathyroidism: a rare entity in chronic renal failure

    International Nuclear Information System (INIS)

    Dorenbeck, U.; Bretschneider, T.; Feuerbach, S.; Leingaertner, T.; Kraemer, B.K.

    2002-01-01

    A case of rare calcification of the tentorium cerebelli, the thoracal dura mater of the spine, and the sclera in an adult male patient with tertiary hyperparathyroidism is presented. The often reported feature in the skull is that of a combination of osteopenia and osteosclerosis giving a granular bone texture. Extensive dura calcification with this condition has very rarely been reported. It is the aim of this paper to document the latter in a patient with chronic renal failure and tertiary hyperparathyroidism. (orig.)

  13. Amyand’s Hernia: Rare Presentation of a Common Ailment

    Directory of Open Access Journals (Sweden)

    Sanjeev Singhal

    2015-01-01

    Full Text Available Inguinal hernia with vermiform appendix as content is known as Amyand’s hernia. It is a rare entity but we encountered four cases within six months. A 52-year-old female had high grade fever and evidence of inflammatory pathology involving the ileocaecal region. She was initially managed conservatively and subsequently underwent exploratory laparatomy. The appendix was perforated and herniating in the inguinal canal. Appendectomy was done with herniorrhaphy without mesh placement. A 74-year-old male with bilateral inguinal hernia, of which, the right side was more symptomatic, underwent open exploration. Operative findings revealed a lipoma of the sac and a normal appearing appendix as content. Contents were reduced without appendectomy and mesh hernioplasty was performed. A 63-year-old male with an obstructed right sided hernia underwent emergency inguinal exploration which revealed edematous caecum and appendix as content without any inflammation. Contents were reduced without any resection. Herniorrhaphy was performed without mesh placement. A 66-year-old male with an uncomplicated right inguinal hernia underwent elective surgery. The sac revealed an appendix with adhesions at the neck. Contents were reduced after adhesiolysis and hernioplasty was performed with mesh placement. Emphasis is made to the rarity of disease, variation in presentation, and difference in treatment modalities depending upon the state of appendix.

  14. Governmental entities accounting: domestic standards and foreign experience of preparation and presentation

    Directory of Open Access Journals (Sweden)

    Ambarchian V.S.

    2015-12-01

    Full Text Available The purpose of research refers to comparison of Ukrainian and the USA standards of preparation and presentation of governmental entities financial reports with the aim of implementation of foreign experience regarding financial data disclosure of a governmental entity activity in domestic reporting system of budgetary resources disposers. In order to achieve the aim the author applied the following general methods of cognition: induction, deduction, analogy, comparison, and historical one. Special economic methods include grouping, tabular, graphical, information and logical analysis. The research resulted in detailed analysis of the standards of the USA governmental entities financial reports presentation and comparison of their statements with domestic governmental accounting standards. As a result of performed research, the key differences in the Ukrainian and the USA rules of presentation of governmental entities financial reports are considered. The author determined the dependence of structure and a public level of the financial reports on the character of the state political and economic systems.

  15. Lipoleiomyoma of the Uterus and Primary Ovarian Leiomyoma in a Postmenopausal Woman: Two Rare Entities in the Same Individual

    Directory of Open Access Journals (Sweden)

    Sefa Kelekci

    2015-01-01

    Full Text Available Uterine lipoleiomyomas are rare benign tumours that are composed of various mixtures of smooth muscle and mature fat tissue. Leiomyomas, which arise primarily in the ovary, are extremely rare tumours that account for 0.5–1% of all benign ovarian tumours. To the best of our knowledge, we present the first case of an ovarian leiomyoma coexisting with a uterine lipoleiomyoma in the postmenopausal period. A 59-year-old, gravida 4, para 3, postmenopausal woman exhibited pelvic discomfort and increased frequency of micturition. A pelvic examination revealed a solid, tender mass on the left side that could not be clearly separated from the uterus. She underwent a laparotomy with an initial diagnosis of a left ovarian mass. She had previously undergone a total abdominal hysterectomy and bilateral salpingo-oophorectomy. A histopathological examination revealed a uterine lipoleiomyoma, composed of variable amounts of smooth muscle cells and mature adipocytes and a right ovarian leiomyoma composed of interlacing bundles and fascicles of spindle cells. The coexistence of these two rare entities in the same individual may represent a common pathway as a stimulating agent. This case may help to clarify the pathogenesis of these lesions.

  16. Osteolytic bone lesions – A rare presentation of AML M6.

    Directory of Open Access Journals (Sweden)

    Geetha N.

    2015-02-01

    Full Text Available Acute myeloid leukemia (AML M6 is a rare form of AML  accounting  for < 5 % of all AML.  Extramedullary involvement  is very rarely seen in this entity.  Skeletal lesion has not been described in AML M6 before. We discuss the case of  a 17 year old boy with AML M6 who presented with  osteolytic lesion of right humerus.  He was treated with  induction and consolidation chemotherapy. The present case is the first report in literature of AML M6 presenting with skeletal lesions.

  17. Merkel cell carcinoma: A rare presentation

    Directory of Open Access Journals (Sweden)

    Prosanta Kumar Bhattacharjee

    2014-01-01

    Full Text Available A 33-year-old man presented with a lump at the right side of chest wall of 4 months duration which started bleeding suddenly from an ulcer at its center. Examination revealed a globular ulcerated mass 2 cm in diameter, on the anterior axillary fold, with adherent clot at its center. No regional lymphadenopathy was noted. Wide local excision with 2 cm margin was done. Biopsy report revealed malignant small round-cell tumor. Immunohistochemistry showed it to be cytokeratin-20-positive and S100-negative, suggesting the diagnosis of Merkel cell carcinoma. The patient did not receive any other adjuvant therapy. He is being followed-up for the last 4 years and has shown no features of recurrence so far.

  18. Appendicitis with appendicular atresia: a rare presentation

    Science.gov (United States)

    Masood, Irfan; Majid, Zain; Rafiq, Ali; Fatima, Saba; Siddiqui, Osama Bin Zia

    2015-01-01

    Acute appendicitis is the most common acute surgical condition; making appendectomy the most commonly performed emergency surgical procedure in the world. Anomalies of the appendix are relatively uncommon. However, their presence may alter the course of pre-operative diagnosis and the surgical treatment provided, leading to medico-legal issues in certain cases as well. We hereby present the case of a 17 year-old female who had the suggestive signs, symptoms and investigations of appendicular lump. She was managed according to the Ochsner-Sherren regimen and then underwent interval open appendectomy 6 weeks later. During the procedure, the findings of a 5 cm long appendix were noted. The base of the appendix was attached to the caecum, however there was complete mucosal discontinuity between the base and the remaining portion of the appendix. A fibrous strand connected the two blind ending parts together. After thorough literature search, the authors concluded that this is only the fourth reported case of appendicular atresia ever to have been reported. Considering the rarity of this finding we feel this could be of valuable interest to surgeons and readers alike PMID:26090015

  19. Acromegaly without acral changes: A rare presentation

    Directory of Open Access Journals (Sweden)

    Nilanjan Sengupta

    2012-01-01

    Full Text Available Acromegaly is diagnosed clinically by the universal presence of acral enlargement and typical coarse facies. We report a short, elderly female developing acromegalic facies for last 10 years without acral overgrowth. She is a patient of primary hypothyroidism, well controlled for last 20 years. Acromegaly was proven by high level of serum insulin like growth factor-1 (IGF-1 and elevated and nonsuppressed level of growth hormone (GH, with other hormonal profile being undisturbed. She had mild insulin resistance and systemic hypertension in absence of any visual field defect. Magnetic resonance imaging (MRI of brain revealed pituitary hyperplasia without any detectable adenoma. No source of ectopic secretion of GH or growth hormone releasing hormone (GHRH could be localized. Therefore, atypical presentation of acromegaly needs high degree of suspicion even if some of the common features are lacking. Here, we have biochemically proved acromegaly with typical facies, short stature but no acral overgrowth and pituitary adenoma despite longstanding disease activity, and thus eluding diagnosis for years.

  20. An aggressive angiomyxoma of vulva - A rare entity - A case report

    Directory of Open Access Journals (Sweden)

    Bishnu Prasad Das

    2016-01-01

    Full Text Available Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor predominantly presenting in women of reproductive age and also having a moderate-to-high risk for local relapse. Hence, it needs to be differentiated from other mesenchymal tumors occurring in this region. We present here a case of a 40-year-old female presenting with a large, fleshy, pedunculated mass on the right labia majora.

  1. Bilateral Maxillary Dentigerous Cysts in a Nonsyndromic Child: A Rare Presentation and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Rakshit Vijay Khandeparker

    2018-01-01

    Full Text Available Dentigerous cysts represent the second most common odontogenic cysts of the jaws after radicular cysts and are usually associated with the crowns of unerupted permanent teeth and rarely deciduous teeth. They are usually solitary in their presentation. Multiple and bilateral dentigerous cysts are an extremely rare presentation in the absence of developmental syndromes or systemic diseases or the use of prescribed certain medications. We hereby present a case of a bilateral dentigerous cyst of the maxilla in a 10-year-old child involving the crowns of unerupted permanent second premolar on the right side and the unerupted permanent canine on the left side. An effort has also been made to review the existing literature on this entity and to stress the importance of radiographic and histopathological examinations in diagnosing such an entity.

  2. Periodic paralysis: rare presenting symptom of thyrotoxicosis.

    Science.gov (United States)

    Correa-Luna, Luis Daniel; Reyes-Ortiz, Luis M; Ramírez-Rivera, José

    2006-01-01

    Paralysis due to hypokalemia results from an acute shift of potassium into cells or excessive potassium deficit. In the absence of potassium deficit, it is observed in Familial Hypokalemic Periodic Paralysis and in Thyrotoxic Hypokalemic Periodic Paralysis (TPP). This report describes the initial presentation of hyperthyroidism as sudden quadriplegia associated with hypokalemia. A healthy 25-year-old Puerto Rican policeman came to the emergency room with sudden paralysis in the four extremities of five hours evolution. He woke up in the morning and could not get up. The day before admission his legs felt weak, and it was hard to get out of bed. He arrived home at 7:00 PM, ate pasta and vegetables, and went to sleep at 10:00 PM. He had no diarrhea or weight loss, no history of medications or illicit drugs. He has a cousin and an aunt with the diagnosis of hypo-thyroidism. The admission temperature was 36.0 degrees C, pulse 96 per minute, respiratory rate 18 per minute, blood pressure 160/70 mmHg. He was alert and oriented as to time, place and person. He could talk properly and was in no respiratory distress. He had no exophtalmos or lid lag. The thyroid was not enlarged or tender. No pseudoclubbing or pretibial edema was found. There was flaccid paralysis of all extremities, 0/5 legs and 1/5 arms. Deep tendon reflexes could not be elicited. The cranial nerves and sensory examination were normal. The hemogram was within normal limits as were the renal and liver functions. Serum sodium was 140 mEq/L, potassium 1.48 mEq/L, phosphorus 1.4 mEq/L. A random glucose was 155 mg/dl and the arterial Ph was 7.41. The urine potassium was 7.04 mEq/L, sodium 60.8 mg/dl. TSH levelwas < 0.03 ug/d], TUP 50.69% (24-40%), T4 17.6 ug/dl (4.7-11.4 ug/dl) Free T4 Index 28.23. He was managed with intravenous potassium chloride, 80 mEq in a period of seven hours with cardiac monitor. The serum potassium level, after the infusion was completed, was 6.70 mEq/L. No cardiac arrhythmia was

  3. Pleomorphic lipoma of the neck in an infant: A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Ram Samujh

    2017-01-01

    Full Text Available Lipomas are rare in the pediatric age group. A 10-month-old male child presented with an asymptomatic neck mass which was evaluated and excised completely. Histopathology was consistent with pleomorphic lipoma, not previously reported in children.

  4. Infratemporal fossa extension of primary parotid squamous cell carcinoma: a rare entity with diagnosis dilemma

    International Nuclear Information System (INIS)

    Karaf, J.H.; Gendeh, B.S.; Yunus, M.R.M.

    2015-01-01

    Primary squamous cell carcinoma of parotid gland is rare but it is an aggressive neoplasm that can have extension into the infratemporal fossa. We report a 60-year-old female presenting with toothache over left lower molar causing a diagnostic dilemma. (author)

  5. Post appendectomy acalculus bilateral ureteric obstruction: A rare entity in children

    Directory of Open Access Journals (Sweden)

    Vipul Gupta

    2013-01-01

    Full Text Available Bilateral acalculus ureteric obstruction is described as rare sequelae of acute appendicitis in two paediatric patients aged 6 and 11 years presented with features of anuria. Imaging and endoscopic evaluation confirmed bilateral ureteric obstruction secondary to bladder wall oedema as an inflammatory reaction to appendix. Both cases recovered following bilateral ureteric stenting and are doing well.

  6. Adult-onset cystic hygroma: A case report of rare entity.

    Science.gov (United States)

    Bahl, Sumit; Shah, Vandana; Anchlia, Sonal; Vyas, Siddharth

    2016-01-01

    Cystic hygroma is a benign congenital malformation of the lymphatic system that occurs in infant or children younger than 2 years of age. Although cystic hygroma is well recognized in pediatric practice, it seldom presents de novo in adulthood. These are commonly present in head and neck but can be present anywhere. Cystic hygroma is very rare in adults, but it should be considered in the differential diagnosis of adult neck swellings. Patients presenting with a painless, soft, fluctuant, and enlarging neck mass should have a careful history and physical examination along with radiological imaging to assist with diagnosis. Surgical intervention is the treatment of choice for this rare condition. Here, we are reporting a case of cystic hygroma in a 32-year-old male patient in the neck region. The objectives of this case report are to discuss the clinical presentation, diagnosis, histopathological findings and management of this malformation.

  7. Nasal Septal Angiofibroma in a Post-Menopausal Woman: A Rare Entity

    Science.gov (United States)

    Dayana, Farah; Fadzilah, Fazalina Mohd; Gendeh, Balwant Singh

    2015-01-01

    Juvenile angiofibromas (JAs) are well-characterised in literature, arising typically in the posterolateral wall of the nasal cavity of young males. Numerous theories have been proposed to explain the occurrence of this unique and rare tumour. Angiofibromas originating in other sites within the head and neck have been described but this is exceedingly rare, constituting less than 2% of all diagnosed cases. Extranasopharyngeal angiofibroma is a rare lesion, and more importantly, controversial. It is not known whether it is actually a relative of the well-known JA that is seen exclusively in adolescent males. We present the case of a post-menopausal woman with unilateral nasal obstruction who was unexpectedly diagnosed as nasal septal angiofibroma. PMID:26816925

  8. Intrinsic brainstem schwannoma – A rare clinical entity and a histological enigma

    Directory of Open Access Journals (Sweden)

    Anil Kumar Sharma

    2016-01-01

    Full Text Available Intraparenchymal schwannomas arising in the brainstem are very rare, and only eight cases have been reported in literature till now. We report an intraparenchymal brainstem schwannoma presenting with the classical clinical presentation of an intrinsic brainstem lesion, and discuss its clinicoradiological characteristics and histological origins. We highlight the importance of an intraoperative frozen section diagnosis in such cases. Intraoperative tissue diagnosis significantly may alter the surgical strategy, which should be aimed at near total intracapsular decompression of the schwannoma.

  9. Pulmonary hyalinizing granuloma presenting with dysphagia: a rare presentation.

    Science.gov (United States)

    Khan, Fazal; Hamid, Arsalan; Fatima, Benish; Hashmi, Shiraz; Fatimi, Saulat

    2017-01-01

    A 25-year-old man presented with a 2-month history of dysphagia and past history of pulmonary and intestinal tuberculosis. A barium swallow showed a point of constriction 42 mm above the gastroesophageal junction. Computed tomography revealed large opacities in bilateral lung fields, encroaching more on the esophagus. The lesion progressively compressed the esophagus as it moved inferiorly. A right posterolateral thoracotomy was performed for sub-anatomical resection of the mass. A biopsy revealed homogenous whirling hyalinized collagen fibers, highly suggestive of pulmonary hyalinizing granuloma, with no evidence of malignancy. Pulmonary hyalinizing granuloma should be considered in the differential diagnosis of longstanding dysphagia.

  10. Co-occurrence of Calcifying Odontogenic Cyst, Aggressive Central Giant Cell Granuloma and Central Odontogenic Fibroma: Report of a Very Rare Entity and Its Surgical Management

    Directory of Open Access Journals (Sweden)

    Touraj Vaezi

    2016-09-01

    Full Text Available Calcifying odontogenic cyst (COC, Central odontogenic fibroma (COF and aggressive central giant cell granuloma (CGCG are rare pathologic diseases affecting the jaws. While the Co-existence of two of them is reported in the literature, existence of all three conditions in one patient is an extremely rare entity. In the present report, initial biopsy revealed fibrosarcoma, therefore mandibular resection was performed for the subject. Sectional Histopathologic evaluation revealed the co-existence of three conditions through histopathologic evaluation. This report emphasizes the importance of precise microscopical evaluation of jaw lesions and thorough sectional examination of the lesions to reach the precise diagnosis. Treatment modalities and follow-up radiographs are also provided to help clinicians manage these entities.

  11. Perforating pilomatrixoma showing atypical presentation: A rare clinical variant

    Directory of Open Access Journals (Sweden)

    Nevra Seyhan

    2018-03-01

    Full Text Available Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a rare benign skin tumor arising from hair follicle stem cells. The most common localization is the head and neck region. Female/male ratio is 3/2. It shows deep subcutaneous placement and occurs in the first two decades of life. Its diameter ranges from 0.5 cm to 3 cm. Multiple lesions are rarely seen. Histopathologically it is characterized by basoloid and ghost cells. Perforating type is a rare clinical variant. Treatment is surgical excision. Our case is presented to draw attention to a rare clinical variant of pilomatrixioma.

  12. Salivary duct cyst on lower lip: A rare entity and literature review

    OpenAIRE

    Tandon, Ankita; Sircar, Keya; Chowdhry, Aman; Bablani, Deepika

    2014-01-01

    Mucocele forms because of salivary gland mucous extravasation or retention and is usually related to trauma in the area of the lower lips. Salivary duct cyst, however, is a type of mucous retention cyst which is almost never located on the lower lip. The aim of this paper is to report this extremely rare salivary duct cyst present on the lower lip and to critically review the literature to build important concepts that would help clinicians in the diagnosis and treatment of this pathology.

  13. Salivary duct cyst on lower lip: A rare entity and literature review.

    Science.gov (United States)

    Tandon, Ankita; Sircar, Keya; Chowdhry, Aman; Bablani, Deepika

    2014-09-01

    Mucocele forms because of salivary gland mucous extravasation or retention and is usually related to trauma in the area of the lower lips. Salivary duct cyst, however, is a type of mucous retention cyst which is almost never located on the lower lip. The aim of this paper is to report this extremely rare salivary duct cyst present on the lower lip and to critically review the literature to build important concepts that would help clinicians in the diagnosis and treatment of this pathology.

  14. Primary histiocytic sarcoma of the uterine cervix: an extremely rare entity

    Directory of Open Access Journals (Sweden)

    Binesh Fariba

    2017-03-01

    Full Text Available Objective. Histiocytic sarcoma (HS is an extraordinary rare tumor and it has an offensive clinical course. HS of the uterine cervix is a far uncommon tumor with just a few cases described so far. Here we presented a case of primary HS of the uterine cervix in a 62-year-old female initially misdiagnosed as large cell non-keratinizing squamous cell carcinoma. To the best of our knowledge, this is the first reported case of HS of the uterine cervix from Iran.

  15. A Rare Primary Pelvic Hydatid Cyst Presenting as Sciatica

    Directory of Open Access Journals (Sweden)

    Praveen S Rathod

    2012-04-01

    Full Text Available Primary hydatid cyst in the pelvis is rare, and usually presents with pressure symptoms affecting the adjacent abdominal organs. We describe a rare hydatid cyst which was eroding the sacral hallow, protruding into the right sciatic foramen and presenting as a radiating pain and weakness of right lower limb due to compression of the lumbosacral nerve roots. Laparotomy with removal of cyst and postoperative treatment with albendazole is effective in controlling the disease and preventing recurrence.

  16. Cavernous sinus thrombosis following dental extraction: a rare case report and forgotten entity.

    Science.gov (United States)

    Aggarwal, Karun; Rastogi, Sanjay; Joshi, Atul; Kumar, Ashish; Chaurasia, Archana; Prakash, Rajat

    2017-10-01

    Prior to the advent of efficacious antimicrobial agents, the mortality rate from cavernous sinus thrombosis (CST) was effectively 100%. There have been very few reports of CST associated with tooth extraction. A 40-year-old female presented to the emergency room with swelling over the right side of the face and history of extraction in the upper right region by an unregistered dental practitioner. The patient presented with diplopia, periorbital ecchymosis, and chemosis of the right eye. A computed tomography scan revealed venous dilatation of the right superior ophthalmic vein. The patient was immediately treated with incision and drainage, intravenous antibiotics, and heparin (low molecular weight). Unfortunately, the patient died two days after surgery due to complications from the disease. CST is a rare disease with a high mortality rate. Therefore, dental health education in rural areas, legal action against unregistered dental practitioners, early diagnosis, and aggressive antibiotic treatment can prevent future mortality resulting from CST.

  17. Aggressive NK-cell leukemia: A rare entity with diagnostic and therapeutic challenge

    Directory of Open Access Journals (Sweden)

    Alia Nazarullah

    2016-06-01

    Full Text Available Aggressive natural killer cell leukemia (ANKL is a rare neoplasm of mature natural killer cells, with an extremely poor overall survival, which is almost always EBV related, with majority of cases reported in East Asia. Here we report the case of an ANKL presenting in a young Hispanic male with secondary hemophagocytosis. Aggressive clinical course, high EBV DNA levels and leukemic presentation, often with associated hemophagocytosis, should raise suspicion of an NK/T-cell neoplasm like ANKL. Due to significant diagnostic overlap with extranodal NK/T-cell lymphoma, nasal type (ENKL, accurate diagnostic classification is crucial due to differing treatment and prognosis. L-asparaginase including chemotherapy followed by allogeneic stem cell transplantation appears to slightly prolong overall survival, but relapse is almost inevitable. Clinical monitoring of EBV DNA levels shows good correlation with disease activity.

  18. Epithelioid hemangioendothelioma and multiple thoraco-lumbar lateral meningoceles: two rare pathological entities in a patient with NF-1

    International Nuclear Information System (INIS)

    Reis, C.; Carneiro, E.; Fonseca, J.; Salgado, A.; Pereira, P.; Vaz, R.; Pinto, R.; Capelinha, A.F.; Lopes, J.M.

    2005-01-01

    Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the Neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. Biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles. (orig.)

  19. Primary angiosarcoma of the testis: report of a rare entity and review of the literature

    Directory of Open Access Journals (Sweden)

    Rao Uma NM

    2007-07-01

    Full Text Available Abstract Background Primary testicular angiosarcomas are extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary testicular angiosarcomas. Methods Six previously reported case reports were identified in the English language medical literature using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. After excluding 2 cases because they did not involve the testis, we identified 4 previously reported cases of true primary testicular angiosarcoma. We also searched the electronic medical archival records of our institution and identified one additional unreported case of true primary testicular angiosarcomas. Data were extracted on the demographics, predisposing factors, clinical presentation, gross pathology, microscopic pathology, immunophenotype, therapy, and outcomes of each of these 5 cases of true primary testicular angiosarcomas. Results Primary testicular angiosarcomas were found at a mean age of 43.4 years. None of the cases was associated with exposure to radiation, arsenic, thorium dioxide, or vinyl chloride. However, 1 case was associated with hydrocele. It typically presented with painless mass (mean size, 6.3 cm. Histologically, all showed classic anastomosing channels lined by plump hyperchromatic cells, though most showed epithelioid cytology and some showed solid architectural pattern. One patient had multiple metastatic recurrences but eventual outcome was not available, and 1 patient died a month after diagnosis from stroke but no autopsy was performed. The remaining 3 patients were alive at the time of publication of their respective cases (mean, 17 months. Conclusion Primary testicular angiosarcomas are typically rare tumors of men of all ages that appear to segregate into 2 groups; one associated with teratoma and occurring in young people, and the other occurring in the

  20. Schwanomma From Cervical Sympathetic Chain Ganglion – A Rare Presentation

    Science.gov (United States)

    Asma, A. Affee

    2015-01-01

    Schwanommas arising from cervical sympathetic chain are tumours that are rare in occurrence. These lesions are usually difficult to differentiate from a vagal schwanomma and a carotid body tumour during the initial workup. In this report, a rarely seen huge cervical sympathetic chain schwanomma case with partial Horner’s syndrome is being presented in detail, which to our known knowledge, is one of the few cases reported in literature. PMID:26557566

  1. Schwanomma From Cervical Sympathetic Chain Ganglion - A Rare Presentation.

    Science.gov (United States)

    Asma, A Affee; Kannah, E

    2015-10-01

    Schwanommas arising from cervical sympathetic chain are tumours that are rare in occurrence. These lesions are usually difficult to differentiate from a vagal schwanomma and a carotid body tumour during the initial workup. In this report, a rarely seen huge cervical sympathetic chain schwanomma case with partial Horner's syndrome is being presented in detail, which to our known knowledge, is one of the few cases reported in literature.

  2. Osteolytic Bone Lesions - A Rare Presentation of AML M6.

    Science.gov (United States)

    Geetha, N; Sreelesh, K P; Priya, M J; Lali, V S; Rekha, N

    2015-01-01

    Acute myeloid leukemia (AML) M6 is a rare form of AML accounting for M6 before. We discuss the case of a 17 year old boy with AML M6, who presented with osteolytic lesion of right humerus. He was treated with induction and consolidation chemotherapy. The present case is the first report in literature of AML M6 presenting with skeletal lesions.

  3. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: Two Unusual Presentations of a Rare Tumor

    Directory of Open Access Journals (Sweden)

    E. C. Castro

    2012-01-01

    Full Text Available Only few cases of primary renal Ewing's sarcoma have been reported in the literature to date. We present here two cases of renal ES/PNET with an uncanny presentation. The first case was discovered after the patient presented clinically with irradiating flank pain, mimicking the pain related with kidney stones. The second case had clinical presentation of pulmonary thromboembolism after the patient was involved in an automobilist accident. The tumors were mainly composed of small blue cells which by immunohistochemical were positive for neural markers, and FISH revealed the translocation 22q12 for the EWSR1 gene. The diagnosis of renal primitive neuroectodermal tumor/EWING tumor is very rare and usually involves several different diagnostic techniques. The differential diagnosis is usually broad with frequent overlapping features between the entities. The cases presented in this paper illustrated the difficulties with which routine anatomical pathologist is faced when dealing with rare renal poorly differentiated neoplasm in adults.

  4. Clinical implications of a rare renal entity: Pleomorphic Hyalinizing Angiectatic Tumor (PHAT).

    Science.gov (United States)

    Scalici Gesolfo, Cristina; Serretta, Vincenzo; Di Maida, Fabrizio; Giannone, Giulio; Barresi, Elisabetta; Franco, Vito; Montironi, Rodolfo

    2017-02-01

    Pleomorphic Hyalinizing Angiectatic Tumor (PHAT) is a rare benign lesion characterized by slow growth, infiltrative behavior and high rate of local recurrences. Only one case has been described in retroperitoneum, at renal hilum, but not involving pelvis or parenchyma. Here we present the first case of PHAT arising in the renal parenchyma. A nodular lesion in right kidney lower pole was diagnosed to a 61 year old woman. The patient underwent right nephrectomy. Microscopically, the lesion showed solid and pseudo-cystic components with hemorrhagic areas characterized by aggregates of ectatic blood vessels. Pleomorphic cells were characterized by large eosinophilic cytoplasm with irregular and hyperchromatic nuclei. Immunohistochemistry was performed and the lesion was classified as a Pleomorphic Hyalinizing Angiectatic Tumor (PHAT). Due to the clinical behavior of this tumor, in spite of its benign nature, review of the surgical margins and close follow up after partial nephrectomy are mandatory. Copyright © 2016. Published by Elsevier GmbH.

  5. Dental Management of a Pediatric Patient with Moyamoya Syndrome: A Rare Clinical Entity.

    Science.gov (United States)

    Ko, Brittany L; Unkel, John H

    2018-01-01

    Moyamoya disease (MMD) is a rare cerebrovascular disorder involving progressive constriction of the internal carotid artery and its branches. The disease has a particularly aggressive course in very young patients, and early surgical intervention is often necessary to prevent permanent neurological damage. MMD patients have an increased risk of stroke development, which may be provoked by pain or anxiety. Currently, no reports of pediatric patients with MMD exist in the dental literature. The purpose of this paper was to discuss the dental management of a two-year-old with moyamoya disease who presented with early childhood caries and dental fear, offering recommendations for dental providers with emphasis on stroke prevention, collaboration with the medical team, anesthesia considerations for patients with increased stroke risk, and the challenges to maintain the oral health of a patient undergoing complex medical treatment.

  6. Spinal angiolipomas: A puzzling case and review of a rare entity

    Directory of Open Access Journals (Sweden)

    Faris Shweikeh

    2017-01-01

    Full Text Available Patients with spinal epidural abscesses (SEAs may have a variable presentation. Such an infection has a typical appearance on magnetic resonance imaging (MRI and enhances with gadolinium. We present a case that was a diagnostic challenge where pre- and intra-operative findings resulted in conflicting impressions. The mimicker was a spinal angiolipoma (SAL. The authors then provide a thorough review of this rare spinal neoplasm. A 55-year-old man presented with back pain, paresis, paresthesia, and urinary retention. MRI was indicative of a longitudinal epidural thoracic mass with a signal homogeneous to nearby fat, curvilinear vessels, and lack of enhancement. Although at emergent surgery, the lesion was found to contain abundant purulent material. Microbiology was positive for methicillin-resistant Staphylococcus aureus and consistent with SEA without evidence of neoplasia. While the imaging features were suggestive of an angiolipoma, the findings at surgery made SEA more likely, which were validated histopathologically. The diagnosis of SEA is often clear-cut, and the literature has reported only a few instances in which it masqueraded as another process such as lymphoma or myelitis. The case highlights SEA masquerading as an angiolipoma, and further demonstrates to clinicians that obtaining tissue diagnosis plays a crucial role diagnostically and therapeutically. SALs, on the other hand, are slow-growing tumors that can be infiltrating or noninfiltrating. They typically present with chronic symptoms and T1-MRI shows an inhomogeneous picture. Complete surgical excision is standard of care and patients tend to do well afterward.

  7. Impact of hypothyroidism on primary anal malignant melanoma: a rare entity.

    Science.gov (United States)

    Singh, Siddharth; Verma, Satyajeet; Kala, Sanjay

    2014-01-01

    Primary melanoma of the anal canal is rare and highly malignant condition, which is 1% of all invasive tumors in this site. This condition is often mistaken for benign conditions as either hemorrhoids or rectal polyp. Thyroid-stimulating hormone stimulation causes high proliferation of malignant melanoma. The association of hypothyroidism with primary malignant melanoma of anal canal is very rare. We are reporting such a very rare case.

  8. Impact of hypothyroidism on primary anal malignant melanoma: A rare entity

    Directory of Open Access Journals (Sweden)

    Siddharth Singh

    2014-01-01

    Full Text Available Primary melanoma of the anal canal is rare and highly malignant condition, which is 1% of all invasive tumors in this site. This condition is often mistaken for benign conditions as either hemorrhoids or rectal polyp. Thyroid-stimulating hormone stimulation causes high proliferation of malignant melanoma. The association of hypothyroidism with primary malignant melanoma of anal canal is very rare. We are reporting such a very rare case.

  9. A rare presentation in two cases with review of literature

    African Journals Online (AJOL)

    2011-07-29

    Jul 29, 2011 ... liver, lungs, and the eyes.[1] Lymphadenopathy is a rare mode of presentation of cysticercus infestation.[2,3] We report two cases of cysticercus lymphadenitis involving the cervical lymph nodes. Case Report. A 7-year-old male child and a 28-year-old woman, both vegetarians by their food habit, presented ...

  10. Longitudinal stress fracture of the femur: A rare presentation

    International Nuclear Information System (INIS)

    Bilreiro, Carlos; Bahia, Carla; Castro, Miguel Oliveira e

    2016-01-01

    We present the case of an 80 year old woman with hip pain, caused by a longitudinal femoral insufficiency stress fracture, depicted with radiographs, CT and MR. This type of fracture is very rare, with only a few cases reported. We conducted a literature review and compared the findings with the present case

  11. Verrucous Lichen Planus: A Rare Presentation of a Common Condition

    Science.gov (United States)

    Audhya, Moutusi; Varughese, Jenny S.; Nakhwa, Yuti C.

    2014-01-01

    Lichen planus is a chronic papulosquamous dermatoses in which both skin and mucosae are involved. There are various morphological forms of lichen planus. Hypertrophic lichen planus is one of the rare clinical variants. Herein, we report a very unusual presentation of hypertrophic lichen planus. A similar presentation has not been reported in literature yet, to the best of our knowledge. PMID:25386324

  12. Rare Asymptomatic Presentation of Omental Mass from Cesarean ...

    African Journals Online (AJOL)

    A case of big oozing lump of unknown origin through a healed cesarean section stitchline, completely asymptomatic at presentation, origin discovered as omentum on exploratory laparotomy came on 10th postoperative day of an uneventful emergency surgery. This rare and silent presentation highlights the need to ...

  13. A Rare Entity: Traumatic Thoracic Aortic Injury in a Patient with Aberrant Right Subclavian Artery.

    Science.gov (United States)

    Patel, Hiten Mohanbhai; Banerjee, Shubhabrata; Bulsara, Shahzad; Sahu, Tapish; Sheorain, Virender K; Grover, Tarun; Parakh, Rajiv

    2017-05-01

    Aberrant right subclavian artery is an uncommon entity incidence ranging from 0.5 to 2.5%. Management of thoracic aortic injury in the presence of such anomalies can be a challenge. We present here a case of traumatic aortic injury, which was incidentally found to have an asymptomatic aberrant right subclavian artery. The patient was managed by an endovascular repair of thoracic aortic injury with an endograft and a right carotid to subclavian artery bypass as a hybrid procedure. A 40-year male patient was brought to the emergency in shock with an alleged history of road traffic accident an hour back. After initial resuscitation as per advance trauma life support protocol, imaging revealed thoracic aortic injury with aberrant right subclavian artery with multiple rib and bilateral humerus fracture. After primary stabilization of arm fractures, the patient was shifted to a hybrid operation room. As the aortic injury was within 10 mm of the origin of both subclavian arteries, it was decided to cover the origin of both subclavian arteries and land the endograft distal to the left carotid artery origin. Since there was a right dominant vertebral artery on imaging, right carotid to right subclavian artery bypass was done with expanded polytetrafluoroethylene graft to prevent posterior circulatory stroke along with thoracic endovascular aortic repair to seal the thoracic aortic injury. After endovascular repair of thoracic aortic injury, left subclavian artery perfusion was maintained through left vertebral artery; and hence, revascularization of left subclavian artery was deferred. After management of all fractures, the patient was discharged 3 weeks after the date of admission without any complications. At 6 months follow-up, patient was stable and images showed patent bypass graft and sealed aortic injury. In a trauma setting with multiple injuries, hybrid procedure with a thoracic endograft is associated with low mortality and morbidity; hence, it is the treatment

  14. Psychomotor delay, a possible rare presentation of moyamoya disease

    International Nuclear Information System (INIS)

    Ashrafi, M. R.; Alizadeh, H.; Yazdani, Sh.; Mohseni, M.; Mohamadi, M.

    2011-01-01

    Moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of Willis leading to ischemic strokes in young people and cerebral hemorrhage, which is more frequent in adults. Secondarily, an abnormal network of fine collateral vessels arises at the base of the brain. The term moyamoya refers to the angiographic appearance of the cerebral vasculature. We present such a disease in an 18-month-old Iranian girl with global developmental delay, which is a very rare presentation of moyamoya disease. She was diagnosed by magnetic resonance imaging and magnetic resonance angiography.

  15. A rare case of primary lymphoma of the caecum presenting as intussusception

    Directory of Open Access Journals (Sweden)

    Leena Jayabackthan

    2013-01-01

    Full Text Available Mucosa associated lymphoid tissue (MALT lymphomas are rare neoplasms. They are most common in the stomach followed by small intestine and colon. The symptoms are nonspecific and generally do not present with intussusception. Here we report a rare clinical entity in which a 35-year-old female presented to the emergency with severe abdominal pain which was sudden in onset. History revealed that she had been having vague mild abdominal pain for 2 years. Ultrasonography showed ileocolic intussusception with hypoechoic lesion of 54 × 46 mm seen at the lead point. Emergency laparotomy with the right hemi-colectomy was done. The specimen was sent for histopathological examination which revealed a diagnosis of MALT lymphoma. Awareness of the varied clinical presentation helps in formulating the appropriate therapeutic strategy.

  16. Bone Involvement by Adenocarcinoma of the Uterine Cervix: A Rare Entity

    International Nuclear Information System (INIS)

    Crisci, Vincenzina; Mainenti, Pier Paolo; Corvino, Fabio; Lauria, Rossella; Maurea, Simone

    2015-01-01

    Adenocarcinoma is the second most frequent cancer of the uterine cervix after squamous carcinoma, and the most frequent histotype is the mucinous one. Endo-cervical adenocarcinoma accounts for about 10–30% of all cervical cancers and clinically the lesion can be asymptomatic or, more frequently, presenting with anomalous bleeding and/or vaginal discharge. A 41-year-old woman with a diagnosis of adenocarcinoma of the uterine cervix was subjected to chemotherapy after radical surgery. During the follow-up, the patient underwent a Positron Emission Tomography integrated with Computed Tomography and pelvic Magnetic Resonance, which showed rapid and diffuse disease progression from the site of the lesion to the pelvic bones. Bone involvement in patients with cervical cancer, being a rare event, is significant since it greatly reduces life expectancy. The majority of metastatic bone lesions in cervical cancer seem to be of osteolytic nature. In our patient, Positron Emission Tomography integrated with Computed Tomography and Magnetic Resonance were the imaging methods used during the follow-up and both techniques clearly showed diffuse and rapid tumour spread to the bones

  17. Compound composite odontome erupting into the oral cavity: A rare entity

    Directory of Open Access Journals (Sweden)

    Sunira Chandra

    2010-01-01

    Full Text Available Odontomas are the most common odontogenic tumors. They are usually asymptomatic and are often discovered during routine radiography. Eruption of an odontome into the oral cavity is rare. Odontomas are the most common odontogenic tumors. They are usually asymptomatic and are often discovered during routine radiography. Eruption of an odontome into the oral cavity is rare. We report an unusual case of erupting compound composite odontoma. we report an unusual case of erupting compound composite odontoma.

  18. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    OpenAIRE

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare en...

  19. Congenital bronchial atresia (CBA). A critical review of CBA as a disease entity and presentation of a case series

    International Nuclear Information System (INIS)

    Pedicelli, Giovacchino; Ciarpaglini, Luisa Laura; De Santis, Marcello; Leonetti, Clara

    2005-01-01

    Purpose. To analyse the state of the art of diagnostic imaging in the rare disease entity known as congenital bronchial atresia (CBA) and to suggest new guidelines for diagnosis. Materials and methods. From January 2002 to December 2003 we examined 6 patients, 4 males and 2 females. Four of them complained of relapsing bronchitis; one patient was admitted with a diagnosis of pulmonary abscess. All patients underwent chest X-ray and MDCT. Results. Five patients presented at chest x-ray the typical association of hilar mass and distal parenchymal hyperinfIation that raised the suspicion of CBA; the remaining patient presented an area of parenchymal translucency that prompted us to perform MDCT, which showed severe stenosis of the segmental bronchus. CBA involved the left upper lobe (LUL) in 4 patients and the right upper lobe (RUL) in the remaining 2. In 50% of cases there were associated anomalies: distal bronchiectasis, bronchogenic cyst, anomaly of branching of bronchial tree and vascular structure. Conclusions. CBA is a relatively rare, generally oligo symptomatic malformation. Knowledge of the signs present at chest X-ray can prompt the radiologist to perform a CT scan. MDCT with multiplanar reconstruction is fundamental for the characterisation, localisation and study of the distribution of the lesion, helping to make a precise diagnosis. Surgery is seldom required and at any rate only performed in case of complications [it

  20. POLYCYSTIC OVARIES WITH STROMAL HYPERTHECOSIS: A RARE PRESENTATION

    Directory of Open Access Journals (Sweden)

    Shaila

    2016-05-01

    Full Text Available Presenting a 47-year-old peri-menopausal lady with hypomenorrhoea, temporal baldness, alopecia, hirsutism. The histopathology was polycystic ovaries with stromal hyperthecosis. Hughesdon described hyperthecosis as a severe form of PCOS. Hyperthecosis is rare in young women, with clinical features similar to PCOS. However, these women are usually more virilised.

  1. Occult spontaneous lateral intrasphenoidal encephalocele: A rare presentation

    Directory of Open Access Journals (Sweden)

    Shahina Bano

    2013-01-01

    Full Text Available Basal encephaloceles are extremely rare congenital malformations. Advanced cross-sectional imaging modalities like computed tomography and magnetic resonance imaging are necessary for diagnosing the asymptomatic, occult basal encephalocele and planning the surgical approach. We present an interesting case of clinically silent right-sided lateral intrasphenoidal encephalocele through a large bony defect.

  2. Hiccup: An Extremely Rare Presentation of Thyrotoxicosis of Graves’ Disease

    Directory of Open Access Journals (Sweden)

    Irshad Parray

    2011-03-01

    Full Text Available Persistent hiccup is a rare but potentially severe condition that can be symptomatic of a variety of diseases or idiopathic. Most episodes last only a few minutes and are self-limited, but hiccup can get persistent and become a real problem for physician and patient alike. The center of hiccup may be activated by a great variety of stimuli travelling along different nerve pathways and bring different effecter responses. We report a case of persistent hiccup as a presentation of impending thyroid storm of Graves’ disease. Though the condition is rare, clinicians should remain alert to the possibility of this diagnosis.

  3. Neuroendocrine tumor of the inguinal node: A very rare presentation

    Directory of Open Access Journals (Sweden)

    Niharika Bisht

    2017-12-01

    Full Text Available Neuroendocrine tumors are a broad family of tumors arising most commonly in the gastrointestinal tract and the bronchus pulmonary tree. The other common sounds are the parathyroid, pituitary and adrenal gland. Inguinal node as a primary presentation of a neuroendocrine tumor is an extremely rare presentation. We present the case of a 43-year-old-male who presented with the complaints of an inguinal node swelling without any other symptoms and on further evaluation was diagnosed to have a non-metastatic neuroendocrine tumor of the inguinal node. He was treated with a combination of chemotherapy and surgery and is presently awaiting completion chemotherapy.

  4. Extranasopharyngeal angiofibroma of the posterior nasal septum: a rare clinical entity.

    Science.gov (United States)

    Atmaca, Sinan; Bayraktar, Cem; Yıldız, Levent

    2013-01-01

    Angiofibroma of extranasopharyngeal origin is very rare. Although it is usually originated from any mucosal structure in the head and neck region, maxilla is the most common involvement site. The nasal septum is an exceptional anatomic site of an angiofibroma. Surgery is the best treatment modality and recurrence is very rare. Nasal septal angiofibromas must be considered in the differential diagnosis of nasal vascular masses arising from the nasal septum. In this article, we report a 37-year-old male case with nasal septal angiofibroma who underwent surgical resection of the tumor. This is the 16th case in the literature.

  5. Rare Presentation of Left Lower Lobe Pulmonary Artery Dissection

    Directory of Open Access Journals (Sweden)

    René Hako

    2017-01-01

    Full Text Available Background. Pulmonary arterial dissection with chronic pulmonary arterial hypertension as its major cause is a very rare but life-threatening condition. In most cases the main pulmonary trunk is the affected site usually without involvement of its branches. Segmental or lobar pulmonary artery dissection is extremely rare. Case Presentation. We report a unique case of left lower lobe pulmonary artery dissection in a 70-year-old male, with confirmed chronic pulmonary hypertension. To confirm dissection MDCT pulmonary angiography was used. Multiplanar reformation (MPR images in sagittal, coronal, oblique sagittal, and curved projections were generated. This case report presents morphologic CT features of rare chronic left lobar pulmonary artery dissection associated with chronic pulmonary hypertension at a place of localised pulmonary artery calcification. CT pulmonary angiography excluded signs of thromboembolism and potential motion or flow artefacts. Conclusion. To the best of our knowledge, no case of lower lobe pulmonary artery dissection with flap calcification has been reported yet. CT imaging of the chest is a key diagnostic tool that is able to detect an intimal flap and a false lumen within the pulmonary arterial tree and is preferred in differential diagnosis of rare complications of sustained pulmonary arterial hypertension.

  6. A Rare Case Presentation of a Perforated Giant Sigmoid Diverticulum

    Directory of Open Access Journals (Sweden)

    Jennifer C. Kam

    2013-01-01

    Full Text Available Giant sigmoid diverticulum (GSD is a rare complication of diverticulosis. These lesions arise from herniations of the mucosa through the muscle wall which progressively enlarge with colonic gas to become large air-filled cysts evident on plain X-ray and CT scans. We present a rare case of a 72-year-old female presenting with abdominal distention, abdominal tenderness, and fever who developed a type 1 giant sigmoid diverticulum (pseudodiverticulum that subsequently formed an intra-abdominal abscess and an accompanying type 2 diverticulum as well. The patient was treated with surgical resection of the diverticulum with a primary anastomosis and abscess drainage. The patient’s postoperative course was uneventful. This case helps to support the need for the consideration of GSD in patients aged 60 and older with a history of diverticulosis and presenting with abdominal discomfort and distension.

  7. Vulvar Lobular Capillary Hemangioma: A Rare Location for a Frequent Entity

    Directory of Open Access Journals (Sweden)

    F. Abreu-dos-Santos

    2016-01-01

    Full Text Available Lobular capillary hemangioma, or pyogenic granuloma, is an acquired hemorrhagic benign vascular lesion of the skin and mucous membranes. The pyogenic granuloma of the vulva is a rare finding and a limited number of case reports are available in the literature. To the best of our knowledge this is the first case described as a single pyogenic granuloma on the vulva.

  8. The Amyand's Hernia: A Rare Clinical Entity Diagnosed by Computed Tomography.

    Science.gov (United States)

    Keskin, Suat; Simşek, Cihan; Keskin, Zeynep

    2013-01-01

    Amyand's hernia, named for the first person to describe an inguinal hernia containing the vermiform appendix, is an uncommon variant of an inguinal hernia. Amyand's hernia is an extremely rare condition and is often misdiagnosed. Traditionally, these hernias have been diagnosed at surgery but are increasingly diagnosed by abdominal computed tomography (CT) scans. CT of the abdomen may help in guiding the diagnosis.

  9. Evolutions and Trends in Presenting. The Balance Sheet as a Financial Position Picture of an Entity

    Directory of Open Access Journals (Sweden)

    Ecaterina Necsulescu

    2011-08-01

    Full Text Available In this article we proposed to achieve a synthesis of the key moments in the evolution of the concept of balance, of the theories formulated over time, of the presentation forms of the balance sheet, a taxonomy of balance sheets, supplemented by a breakdown of the concept of financial position and of the structures related to the assessment of the financial position. In other words, we will consider a gradual presentation of the concept of balance sheet over time and a presentation of the balance as the main tool for highlighting the patrimonial situation, of the assets of enterprise owners and of the picture on the financial position of an economic entity.

  10. Microscopic haematuria: A rare presentation of typhoid fever.

    Science.gov (United States)

    Nisahan, Balasingam; Thirunavukarasu, Kumanan; Selvaratnam, Gowry

    2015-04-01

    Typhoid fever can cause a number of renal manifestations heretofore dubbed 'nephrotyphoid'. Haematuria in the absence of renal impairment is extremely rare among typhoid patients. We report a case of an adult who presented with a prolonged febrile illness and microscopic haematuria. Blood culture confirmed the diagnosis of typhoid and the patient was treated successfully with ceftriaxone. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  11. Mid Arm Swelling- A Rare Presentation of Filariasis

    OpenAIRE

    Haren Oza; Jignasa Bhalodia; Ami Shah; Palak Modi

    2014-01-01

    Bancroftian Filariasis is a tropical and subtropical disease caused by Wuchereria bancrofti and transmitted by the Culex mosquitoes. The diagnosis of it is conventionally made by demonstrating microfilariae in the peripheral blood smear. Microfilaria and adult filarial worm have been incidentally detected in fine needle aspirates of various lesions. We here report a rare case presentation of Bancroftian filariasis in 20 years old asymptomatic male coming from an endemic area with swelling in ...

  12. A rare presentation of a transethmoidal encephalocele at birth.

    Science.gov (United States)

    Upasani, Anand Vishwanath; Patel, Dhiren Nanjibhai; Chandna, Sudhir Bhisham

    2014-10-01

    Transethmoidal encephalocele is a very rare condition. Herein, we report the case of a neonate with a transethmoidal encephalocele, who presented with an externally visible intranasal mass at birth. Clinical suspicion of intracranial extension was confirmed by radiological imaging. A bifrontal craniotomy was done to divide the narrow communicating duct. The mass was delivered through the nostril and duraplasty was completed. The postoperative recovery was uneventful. Copyright © 2013. Published by Elsevier B.V.

  13. A rare presentation of lipoma on mandibular mucogingival junction

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    Gaurav Sharma

    2016-01-01

    Full Text Available Lipoma is the most common tumor of mesenchymal tissues of body, but its occurrence in oral cavity is infrequent. Buccal mucosa is the most common intraoral site of lipoma followed by tongue, floor of the mouth, and buccal vestibule. The involvement of mucogingival junction is rare. We present a unique case report of oral lipoma occurring on mandibular mucogingival junction with review of literature which has emphasis on differential diagnosis.

  14. A Rare Presentation of a Transethmoidal Encephalocele at Birth

    Directory of Open Access Journals (Sweden)

    Anand Vishwanath Upasani

    2014-10-01

    Full Text Available Transethmoidal encephalocele is a very rare condition. Herein, we report the case of a neonate with a transethmoidal encephalocele, who presented with an externally visible intranasal mass at birth. Clinical suspicion of intracranial extension was confirmed by radiological imaging. A bifrontal craniotomy was done to divide the narrow communicating duct. The mass was delivered through the nostril and duraplasty was completed. The postoperative recovery was uneventful.

  15. Bilateral Facial Diplegia: A Rare Presenting Symptom of Lyme

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    John Ashurst

    2017-01-01

    Full Text Available Lyme disease is a common disease that is faced by the physician but also acts a mimicker of many other disease processes. Facial palsies, especially bilateral, are a relatively rare presenting symptom of Lyme disease and may warrant further investigation. A thorough history and physical examination coupled with precision testing may aid the physician when faced with a patient with the diagnostic dilemma of facial diplegia.

  16. Complete eventration of right hemidiaphragm: A rare presentation

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    Krushna Makwana

    2017-01-01

    Full Text Available Eventration of the diaphragm is a term used to describe an abnormal elevation of part or whole of the hemidiaphragm, where the whole or part of the diaphragm is made up of a thin fibro membranous sheet replacing normal diaphragmatic musculature. Complete and partial eventration both can occur, however, complete eventration of the right hemidiaphragm in an adult female, as presented in this patient, is rarely seen.

  17. Peripheral Presentation of Periapical Cyst: A Rare Finding

    OpenAIRE

    Nilesh Kumar; Sameer A Zope; Mohan S Sannale

    2015-01-01

    Periapical cyst is a slow growing dental cyst, which is discove­ red on routine intraoral radiograph or cause localized intra­ osseous jaw swelling. It is typically found at root apex of the involved tooth and is discovered on routine radiography. This paper reports a rare presentation of periapical cyst. The cyst was localized in the premaxillary subcutaneous tissue, causing diagnostic difficulty. The steps in diagnosis of the lesion are also discussed

  18. A Rare Clinical Presentation of Darier’s Disease

    OpenAIRE

    Ferizi, Mybera; Begolli-Gerqari, Antigona; Luzar, Bostjan; Kurshumliu, Fisnik; Ferizi, Mergita

    2013-01-01

    Darier’s disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomat...

  19. Intraosseous polymorphous low-grade adenocarcinoma of mandible: A rare entity

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    Gayathri Ramesh

    2012-01-01

    Full Text Available Polymorphous low-grade adenocarcinoma is a rare type of minor salivary gland malignancy. The characteristic features of these tumors are the varied histomorphology and the malignant, albeit indolent behavior. It occurs commonly in the minor salivary glands, with the palate (58.5% being the most common intra oral site. Maxillary area (2%, mandibular mucosal area (1.5% and posterior trigone region (0.5% are the least affected areas. An occasional case has been reported arising from an intraosseous location i.e the maxilla, and only two cases have been reported in the English literature originating from the mandible. One such very rare case of polymorphous low-grade adenocarcinoma of the mandible, which radiographically has a soap bubble appearance, is reported here.

  20. Idiopathic pleuroparenchymal fibroelastosis, a rare entity of interstitial pneumonia: A case report

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    Park, Jong Chun; Chae, Eun Jin; Song, Joon Seon [University of Ulsan College of Medicine, Asan Medical Center, Seou (Korea, Republic of)

    2014-04-15

    Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described, very rare type of fibrotic interstitial lung disease predominantly involving subpleural areas of both upper lungs. IPPFE has distinctive radiologic and pathologic features: progressive subpleural opacity with fibrotic changes, predominantly in upper lungs, and dense elastic component on histology. We experienced one case of surgically confirmed IPPFE, with progression of radiologic findings on the serial CT examinations. We herein report the characteristic radiologic features of IPPFE with pathologic and clinical manifestations.

  1. PRIMARY HEMANGIOMA OF A SUBMENTAL LYMPH NODE –A RARE ENTITY

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    Shri LakshmiS, Durga PrasadD, Subba Rao D, PrasanthiC, Vandana Gangadharan, Kishore Kumar C

    2015-04-01

    Full Text Available ABSTRACT Primary vascular tumors occurring in lymph nodes are extremely rare. Nodal hemangiomas are benign vascular tumors that can occur at any age and seen mostly in females. It is usually asymptomatic, affects only one node, and does not recur. Four histologic types of hemangioma have been identified: capillary/cavernous, lobular capillary, cellular, and epithelioid. This case has been reported for its rarity

  2. The Amyand’s Hernia: A Rare Clinical Entity Diagnosed by Computed Tomography

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    Suat Keskin

    2013-01-01

    Full Text Available Amyand’s hernia, named for the first person to describe an inguinal hernia containing the vermiform appendix, is an uncommon variant of an inguinal hernia. Amyand’s hernia is an extremely rare condition and is often misdiagnosed. Traditionally, these hernias have been diagnosed at surgery but are increasingly diagnosed by abdominal computed tomography (CT scans. CT of the abdomen may help in guiding the diagnosis.

  3. A Rare Manifestation of Tuberculosis Presenting in the United States

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    Osman Bhatty

    2016-01-01

    Full Text Available A 64-year-old Bangladeshi female presented to her primary care physician with a tender right breast lump that had been present for 4-5 days along with subjective fevers and malaise. Initial biopsy revealed granulomas, but Ziehl-Neelsen and Gram stain were negative for TB so antibiotics were prescribed for abscess until culture came positive for tuberculosis. She was started on triple therapy for extrapulmonary tuberculosis, an exceedingly rare presentation that requires high clinical suspicion in the Western world.

  4. A Very Rare Presentation of Multiple Myeloma: Unilateral Raccoon Eye

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    Ceyhun Varım

    2015-08-01

    Two thirds of patients complain of bone pain, especially lower back pain. MM could be diagnosed after a pathologic fracture occurs in one third of patients. Presentation with symptoms related to hyperviscosity, hypercalcemia and bleeding tendency could also be observed. A rare presentation of MM is peri-orbital ecchymotic lesion (raccoon eye. Here, we report a 64 years old, male patient presented with unilateral raccoon eye and high erythrocyte sedimentation rate (ESR to internal medicine outpatient. The patient was referred to hematology outpatient and was diagnosed with multiple myeloma.

  5. An Unusual Presentation of Metanephric Adenofibroma: A Rare Case Report

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    Kiran Agarwal

    2017-10-01

    Full Text Available Metanephric adenofibroma is a rare renal neoplasm with only a few case reports in literature. In majority of cases, it is asymptomatic. However, it may present with haematuria, polycythemia or hypertension. Radiologically, it is indistinguishable from other solid renal tumours. Definitive diagnosis can only be made on the basis of histopathology. It is a benign neoplasm and requires only surgical excision with no need for chemotherapy. Involvement of urinary bladder and presentation as bladder mass has never been reported. In this case report, we present a case of metanephric adenofibroma in a two-year-old male child manifesting with haematuria and urinary bladder mass.

  6. Fatal methemoglobinemia complicating alkaptonuria (ochronosis): a rare presentation.

    Science.gov (United States)

    Freeman, Amanda R; Wills, Stephen M

    2018-06-01

    A 61-year-old female died in hospital with multiple organ failure 4 weeks following presentation with acute kidney injury, hemolytic anemia and methemoglobinemia. At autopsy, brown to black discoloration of cartilages was observed. Histology revealed brown pigmentation of the hyaline cartilage, with focal full-thickness erosion of the articular hyaline cartilage, characteristic of alkaptonuria (ochronosis). Although alkaptonuria is rarely fatal, this case illustrates a rare acute fatal complication. Accumulation of circulating homgentisic acid secondary to acute derangement of renal function is believed to have overwhelmed the endogenous antioxidant processes, resulting in hemolysis and methemoglobinemia, which were refractory to treatment. Small numbers of cases have previously been reported in the literature in patients known to suffer with the disease, all of which were preceded by acute kidney injury. Whilst the clinical diagnosis of alkaptonuria may be challenging, the autopsy findings of this rare condition are striking and this case illustrates the utility of the autopsy, albeit retrospectively, in arriving at a diagnosis. To our knowledge this is the first reported case where previously undiagnosed alkaptonuria has presented with methemoglobinemia.

  7. Multiple Autoimmune Syndromes Associated with Psoriasis: A Rare Clinical Presentation

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    Sadia Masood

    2014-03-01

    Full Text Available Autoimmune diseases are known to have association with each other but it is very rare to see multiple autoimmune diseases in one patient. The combination of at least three autoimmune diseases in the same patient is referred to as multiple autoimmune syndrome. The case we are reporting features multiple autoimmune syndrome with five different conditions. The patient had type 1 diabetes mellitus, autoimmune hemolytic anemia, systemic lupus erythematosus, vitiligo, and psoriasis. Psoriasis has rarely been reported previously under the spectrum of autoimmune syndrome. Although the relationship of autoimmune conditions with each other has been explored in the past, this case adds yet another dimension to the unique evolution of autoimmune pathologies. The patient presented with a combination of five autoimmune diseases, which makes it consistent type three multiple autoimmune syndromes with the addition of psoriasis. The current case is unique in this aspect that the combination of these five autoimmune disorders has never been reported in the past.

  8. Synovial sarcoma: a rare presentation of parapharyngeal mass.

    Science.gov (United States)

    Shaariyah, Mohd Mokhtar; Mazita, Ami; Masaany, Mansor; Razif, Mohd Yunus; Isa, Mohamed Rose; Asma, Abdullah

    2010-06-01

    Synovial sarcoma is a rare soft tissue sarcoma of the head and neck region involving the parapharyngeal space. The diagnosis of synovial sarcoma can be very challenging to the pathologists. We present a rare case of parapharyngeal synovial sarcoma in a young female patient who had a two-month history of left cervical intumescent mass at level II. The fine needle aspiration cytology of the mass was proved inconclusive. Transcervical excision of the mass was performed and the first case of parapharyngeal sarcoma was identified in our center by fluorescence in situ hybridization (FISH) technique. Repeat imaging revealed residual tumor. The patient successfully underwent a second excision of the residual tumor and received adjuvant radiotherapy.

  9. Unusual Origin and Rare Presentation of Primary Cardiac Lymphoma

    Science.gov (United States)

    Mohamed, Amir; Cherian, Sanjay; El-Ashmawy, Ahmed; Abdelmoneim, Salah Eldin; Soliman, Maher; Abu-Rayan, Mohamed; Kalangos, Afksendyios

    2011-01-01

    Non-Hodgkin lymphoblastic lymphomas are very uncommon tumors that rarely involve the heart; however, when they do, they typically cause cardiac symptoms. Herein, we describe the case of a young woman who presented with respiratory symptoms. These were caused by a high-grade lymphoblastic lymphoma, which originated in the left inferior pulmonary vein and extended into the left atrium. The tumor was surgically debulked, but it recurred in 1 month, and the patient underwent chemotherapy. Six months later, she had recurrent respiratory symptoms, and echocardiography revealed a persistent mass in the left lower lobar vein. A modified chemotherapy regimen led to complete resolution of the tumor within 2 months. We are unaware of other reports of a primary cardiac non-Hodgkin lymphoblastic lymphoma with this unusual site of origin and rare manifestation of symptoms. PMID:21841872

  10. Splenic Infarct: A Rare Presentation in a Pediatric Patient

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    Palla Bhattarai

    2014-12-01

    Full Text Available A previously healthy 16-year-old male presented with a two day history of persistent epigastric pain. His physical examination was significant for tenderness in the left hypochondriac region with a palpable spleen 2cm below the left sub-costal margin. A CT scan of the abdomen showed a splenic infarct. Heterophile and EBV VCA IgM antibody test were positive. This is a rare case of infectious mononucleosis presenting with splenic infarct in an adolescent male without comorbidities. Keywords: infectious mononucleosis; splenic infarct.

  11. Intraepidermal Merkel cell carcinoma: A case series of a rare entity with clinical follow up.

    Science.gov (United States)

    Jour, George; Aung, Phyu P; Rozas-Muñoz, Eduardo; Curry, Johnathan L; Prieto, Victor; Ivan, Doina

    2017-08-01

    Merkel cell carcinoma (MCC) is a rare but aggressive cutaneous carcinoma. MCC typically involves dermis and although epidermotropism has been reported, MCC strictly intraepidermal or in situ (MCCIS) is exceedingly rare. Most of the cases of MCCIS described so far have other associated lesions, such as squamous or basal cell carcinoma, actinic keratosis and so on. Herein, we describe 3 patients with MCC strictly in situ, without a dermal component. Our patients were elderly. 2 of the lesions involved the head and neck area and 1 was on a finger. All tumors were strictly intraepidermal in the diagnostic biopsies, and had histomorphologic features and an immunohistochemical profile supporting the diagnosis of MCC. Excisional biopsies were performed in 2 cases and failed to reveal dermal involvement by MCC or other associated malignancies. Our findings raise the awareness that MCC strictly in situ does exist and it should be included in the differential diagnosis of Paget's or extramammary Paget's disease, pagetoid squamous cell carcinoma, melanoma and other neoplasms that typically show histologically pagetoid extension of neoplastic cells. Considering the limited number of cases reported to date, the diagnosis of isolated MCCIS should not warrant a change in management from the typical MCC. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. Rare presentation of Kyrle′s disease in siblings

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    Viswanathan Seethalakshmi

    2008-01-01

    Full Text Available Background: Kyrle′s disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings. Materials and Methods: Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction. Results and Conclusions: A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle′s disease. This helps to differentiate the rare primary Kyrle′s disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.

  13. Low-grade central osteosarcoma in proximal humerus: a rare entity

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    Tang F

    2017-10-01

    Full Text Available Fan Tang,1,2 Li Min,1,2 Yong Zhou,1 Yi Luo,1 Chongqi Tu1 1Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, People’s Republic of China; 2Sarcoma Biology Laboratory, Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, MA, USA Abstract: Low-grade central osteosarcoma is a rare subtype of tumor with low-grade malignancy. Currently, wide resection with negative resection margin is the standard treatment for this disease. The role of neoadjuvant chemotherapy in low-grade central osteosarcoma was controversial and was mostly considered for tumors containing high-grade focal areas. Local tumor recurrences often exhibited a tumor with higher histologic grade or differentiation with the potential for metastases. In low-grade central osteosarcoma, timely wide resection after definite diagnosis can result in 5-year survival for almost 90%. However, the relatively nonspecific radiological and pathological findings make diagnosis very difficult. MDM2 and CDK4 are specific and provide sensitive markers for the diagnosis of low-grade central osteosarcoma, helping to differentiate low-grade central osteosarcoma from some benign lesions, including fibrous dysplasia, bone giant cell tumor, and chondrosarcoma. Here, we report the case of a 19-year-old woman with low-grade central osteosarcoma located at the proximal humerus. The affected site was rare, but the sensitive biomarkers CDK4 and MDM2 were positive. The patient recovered well after wide tumor resection following a proximal humerus endoprosthesis replacement. Our case highlighted the management strategies in low-grade central osteosarcoma. Being familiar with radiographic features, understanding the biological characteristics, and mastering diagnostic biomarkers can help oncologists avoid embarrassing situations in treatment when this rare tumor is highly suspected, even when located at an uncommon site. The discussion in this report

  14. Neuroendocrine Merkel cell nodal carcinoma of unknown primary site: management and outcomes of a rare entity.

    Science.gov (United States)

    Kotteas, E A; Pavlidis, N

    2015-04-01

    Merkel cell nodal carcinoma of unknown primary (MCCUP) is a rare neuroendocrine tumour with distinct clinical and biological behaviour. We conducted a review of retrospective data extracted from 90 patients focusing on the management and outcome of this disease. We also compared life expectancy of these patients with the outcome of patients with known Merkel primaries and with neuroendocrine cancers of unidentifiable primary. There is a limited body of data for this type of malignancy, however, patients with Merkel cell nodal carcinoma of unknown primary site, seem to have better survival when treated aggressively than patients with cutaneous Merkel tumours of the same stage and equal survival with patients with low-grade neuroendocrine tumour of unknown origin. The lack of prospective trials, and the inadequate data, hamper the management of these tumours. Establishment of treatment guidelines is urgently needed. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  15. Glial Heterotopia of the orbit: A rare presentation

    Science.gov (United States)

    2011-01-01

    Background Glial heterotopias are rare, benign, congenital, midline, non-teratomatous extracranial glial tissue. They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness. Case presentation A 6 year-old-boy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed since birth. On examination, the globe was displaced laterally by a firm, regular, mobile, non-pulsatile and non-tender medial mass. The affected eye had profound loss of vision. Computed tomography scan showed a large hypodense mass in the extraconal space with no intracranial connectivity and bony erosion. The child underwent total surgical excision of the mass and histopathological examination confirmed glial heterotopia of the orbit. Conclusion Though the incidence of this condition is rare, the need of appropriate diagnosis and management of such mass to prevent the visual and cosmetic deterioration is warranted. To our knowledge this is the first reported case of Glial heterotopia of orbit causing unilateral blindness. PMID:22088230

  16. Glial Heterotopia of the orbit: A rare presentation

    Directory of Open Access Journals (Sweden)

    Sitaula Ranju

    2011-11-01

    Full Text Available Abstract Background Glial heterotopias are rare, benign, congenital, midline, non-teratomatous extracranial glial tissue. They may masquerade as encephalocoele or dermoid cyst and mostly present in nose. Herein, we present an unusual case of glial heterotopia of the orbit with unilateral blindness. Case presentation A 6 year-old-boy presented with a progressive painless mass over the nose and medial aspect of the left eye noticed since birth. On examination, the globe was displaced laterally by a firm, regular, mobile, non-pulsatile and non-tender medial mass. The affected eye had profound loss of vision. Computed tomography scan showed a large hypodense mass in the extraconal space with no intracranial connectivity and bony erosion. The child underwent total surgical excision of the mass and histopathological examination confirmed glial heterotopia of the orbit. Conclusion Though the incidence of this condition is rare, the need of appropriate diagnosis and management of such mass to prevent the visual and cosmetic deterioration is warranted. To our knowledge this is the first reported case of Glial heterotopia of orbit causing unilateral blindness.

  17. Encephalocele presenting as lower lid swelling: A rare case report

    Directory of Open Access Journals (Sweden)

    Vaibhav Kumar Jain

    2018-01-01

    Full Text Available Encephalocele is a rare congenital abnormality characterized by abnormal protrusion of brain and meninges through an opening in the skull. We report an 8-year-old girl who presented with a swelling in the right lower lid for the last 6 years. In her infancy, she had undergone surgery for a very small swelling located in the right nasolacrimal area. On further clinicoradiological evaluation, anterior encephalocele was diagnosed. This case highlights the uncommon site of anterior encephalocele; misdiagnosis and mismanagement of which could result in dreaded complications such as meningitis and cerebrospinal fluid leaking fistula formation.

  18. Serous Carcinoma of the Uterine Cervix, an Extremely Rare Aggressive Entity: A Literature Review.

    Science.gov (United States)

    Jonska-Gmyrek, Joanna; Zolciak-Siwinska, Agnieszka; Gmyrek, Leszek; Michalski, Wojciech; Poniatowska, Grazyna; Fuksiewicz, Malgorzata; Wiechno, Pawel; Kucharz, Jakub; Kowalska, Maria; Kotowicz, Beata

    2018-01-23

    Serous carcinoma of the uterine cervix (USCC) is an extremely rare subtype. To establish the treatment strategy in patients with USCC is an important issue. MEDLINE (PubMed) was searched for all articles published after the first publication by Lurie et al. [Eur J Obstet Gynecol Reprod Biol 1991; 40: 79-81], reporting woman diagnosed with USCC. Because of limited numbers of studies on the topic of the study, we could not keep a restriction of eliminating smaller sample sizes. A search of PubMed demonstrated that 113 cases of USCC have been reported in the literature since the first publication. The current treatment modality adopted for early cervical cancer is hysterectomy with bilateral iliac-obturator lymphadenectomy and postoperative radiotherapy (RT) or radiochemotherapy (RT-CT) if risk factors for cervical carcinoma appear. The treatment strategy for locally advanced USCC is preoperative RT-CT or chemotherapy (CHTH) with the intention to treat the patient surgically. The treatment option for disseminated disease is CHTH with paclitaxel and carboplatin. Risk factors and a more advanced clinical stage of USCC have an impact on poor outcomes despite the use of standard treatment methods, adapted for cervical cancer. The outside-pelvic failures tend to seek effective systemic treatment. © 2018 S. Karger AG, Basel.

  19. Invasive lobular carcinoma: a rare presentation in the male breast.

    Science.gov (United States)

    Melo Abreu, Elisa; Pereira, Pedro; Marques, José Carlos; Esteves, Gonçalo

    2016-05-05

    Breast cancer in men is uncommon, accounting for cancers. Even though lobular structures are quite infrequent in the male breast, rare cases of invasive lobular breast carcinoma have been described, representing 1-2% of all breast cancers in men. Risk factors include undescended testes, congenital inguinal hernia, orchiectomy, orchitis, testicular injury, infertility and Klinefelter's syndrome, previous thoracic radiotherapy, alterations of the oestrogen-testosterone ratio and familial history (BRCA 2 and 1). The authors present a case of a 52-year-old man with no relevant predisposing factors to breast cancer, who presented with a painless, firm nodule, fixed to the nipple on the left breast, associated with nipple retraction and ulceration, and fully characterised by mammogram and ultrasound. Histopathological and immunohistochemical analysis revealed the diagnosis of invasive lobular breast carcinoma and the patient underwent left radical mastectomy, followed by adjuvant chemotherapy, radiotherapy and hormonotherapy. A brief review of the literature is presented. 2016 BMJ Publishing Group Ltd.

  20. A Rare Clinical Presentation of Darier’s Disease

    Directory of Open Access Journals (Sweden)

    Mybera Ferizi

    2013-01-01

    Full Text Available Darier’s disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules, primarily affecting seborrheic areas on the head, neck, and thorax and less frequently on the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They are usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions are presented as suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by “corps ronds and grains”. This paper reports a case of a 53-year-old woman that was admitted to our clinic with more than 10-year history of keratotic papules, presented on the hands and feet, nose, ears, genitalia, and whitish lesions on palatal mucosae.

  1. Bullosis Diabeticorum: Rare Presentation in a Common Disease

    Directory of Open Access Journals (Sweden)

    Vineet Gupta

    2014-01-01

    Full Text Available A 27-year-old African American male presented with a sudden onset of blisters. He had a past medical history of uncontrolled diabetes mellitus type I, diabetic vasculopathy, and neuropathy. The physical examination revealed nonerythematous skin denudations on both elbows and lateral aspect of arm bilaterally. Investigations which included skin biopsies confirmed the diagnosis of bullosis diabeticorum. The bullae were treated with hydrotherapy and healed with no complications in 4 weeks. We present this case to illustrate the rare occurrence of diabetic bulla in a diabetic patient especially with poor glycemic control. The case is also a reminder of the importance of diabetes screening in nondiabetic patients who are diagnosed with diabetic bulla.

  2. Phaeochromocytoma presenting with polyuria: an uncommon presentation of a rare tumour

    OpenAIRE

    Atapattu, N; Imalke, K A C P; Madarasinghe, M; Lamahewage, A; de Silva, K S H

    2014-01-01

    Summary Children rarely present with phaeochromocytoma. Their presentation differs from that of adults. The classic triad of sweating, headache and palpitation may not always present in children with phaeochromocytoma. In this study, we present a 6-year-old girl who came to us with polyuria and polydipsia for evaluation of suspected diabetes insipidus. She gave a clear history of increased sweating in the recent past. On clinical examination, she was noted to have high blood pressure. Subsequ...

  3. Analysis of primary intrathoracic extrapulmonary hydatid cysts a rare clinical entity

    International Nuclear Information System (INIS)

    Ahmad, M.; Ullah, M.K.; Imran, M.; Bilal, A.; Khan, M.A.; Ali, S.Z.

    2015-01-01

    prescribed to all patients for as long as 3 months postoperatively. No complication, recurrence, or death occurred during the follow up period of 13 ± 15.4 months (range, 2-36 months). Conclusion: The extrapulmonary location of Hydatid cysts within the thorax is very rare. This rarity may cause difficulties in diagnosis. To avoid recurrence, it is necessary to resect the affected tissues completely and an anthelmintic medical regimen post operatively. (author)

  4. Cavernous sinus thrombosis following dental extraction: a rare case report and forgotten entity

    OpenAIRE

    Aggarwal, Karun; Rastogi, Sanjay; Joshi, Atul; Kumar, Ashish; Chaurasia, Archana; Prakash, Rajat

    2017-01-01

    Prior to the advent of efficacious antimicrobial agents, the mortality rate from cavernous sinus thrombosis (CST) was effectively 100%. There have been very few reports of CST associated with tooth extraction. A 40-year-old female presented to the emergency room with swelling over the right side of the face and history of extraction in the upper right region by an unregistered dental practitioner. The patient presented with diplopia, periorbital ecchymosis, and chemosis of the right eye. A co...

  5. Metastasis of the epididymis and spermatic cord from pancreatic adenocarcinoma: A rare entity. Description of a case and revision of literature.

    Science.gov (United States)

    Di Franco, Carmelo Agostino; Rovereto, Bruno; Porru, Daniele; Zoccarato, Valeria; Regina, Cesare; Cebrelli, Tiziano; Fiorello, Nicolò; Viglio, Alessandra; Galvagno, Lavinia; Marchetti, Carlo; Ringressi, Andrea; Barletta, Davide; Giliberto, Giovanni

    2018-03-31

    Metastatic epididymal and spermatic cord adenocarcinoma from epithelial tumors are a rare condition. The most frequent primary cancers are prostate, lung, kidney, gastrointestinal tumors and breast. In literature, there are very low number of cases reporting metastasis from pancreatic cancer to epididymis and spermatic cord. We report a case of 70-years old man with history of left orchiectomy for undescended testicle, who presented to our department with a palpable nodule in the right scrotum. Scrotal ultrasound revealed an inhomogeneous hypoechoic nodule of epididymis and/or spermatic cord. Neoplastic markers showed high levels of CEA (carcinoembryonic antigen) and bHCG (beta Human Chorionic Gonadotropin). The patient underwent right surgical scrotal exploration with orchifunicolectomy. Pathologic examination revealed pathologic tissue showing rare glandular structures. Immunohistochemistry profile was compatible with malign epithelial neoplasm with glandular differentiation. Total body CT-scan revealed pathologic tissue in pancreas between head and body and a suspect pathologic lesion in liver and 18-FDG PET-scan confirmed the pancreatic neoplastic mass and a suspect secondary hepatic lesion. Biopsy of pancreatic pathologic area was positive for ductal pancreatic adenocarcinoma. The patient was sent to oncologic evaluation and started chemotherapy. Malignancies of epididymis and spermatic cord are rare entities and, in literature, very low number of cases of metastasis from pancreatic carcinoma to epididymis and spermatic cord are described. Early differential diagnosis is fundamental mostly in those patients with age range unusual for testis cancers.

  6. Ellis-van Creveld syndrome with unusual oral and dental findings: A rare clinical entity

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    Sameeulla Shaik

    2016-01-01

    Full Text Available Ellis-van Creveld (EVC syndrome, a form of skeletal and chondroectodermal dysplasia, is an autosomal recessive disorder characterized by a tetrad of disproportionate dwarfism, postaxial polydactyly, ectodermal dysplasia, and heart defects. In the present article, we hereby present a case of a 13-year-old girl of Indian ethnicity with EVC syndrome with a remarkable number of classical oral and dental features, with unusual findings such as taurodontism and talons cusp. Such dental findings were reported in few cases only. Despite the fact that oral manifestations play an important role in the diagnosis of EVC, only a few detailed reports have been published in the dental literature.

  7. Bilateral Subcutaneous Fibroadenomata On The ARM : A Rare Entity With Embryological Curiosity

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    Kini Usha

    1997-01-01

    Full Text Available We report a case of multiple bilateral fibroadenomata on the arm away from the milk line in a 33 year old female who in addition presented with fibrocystic disease of the breast and secondary infertility. Possible origin of these cutaenous fibroadenomas have been hypothesized. This is the second report of its kind being reported in the English literature.

  8. Krukenberg tumor in a young woman: A rare presentation

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    Deepa Hatwal

    2014-01-01

    Full Text Available Krukenberg tumors mostly occur after 40 years. Metastatic ovarian tumors in young age are very rare and reported to be 2% of all the cases. Thirty percent of all ovarian neoplasms occurring during childhood and adolescence are malignant. A 25-year-old woman, parity- 2, presented with abdominal distension, pain in abdomen and amenorrhea. On examination, 18 weeks lump was palpable, firm to hard in consistency, non-tender and mobile. On ultrasonography bilateral ovarian tumors were reported, without any peritoneal free fluid. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Microscopic examination revealed signet ring cells with glandular differentiation, diffusely invading the ovarian parenchyma. Tumor cells exhibited strong, diffuse immunopositivity for CEA with focal strong immunopositivity for CK7 and CK20 and immunonegativity for SATB2. Diagnosis of Krukenberg tumor was made. Endoscopic biopsy confirmed the diagnosis of adenocarcinoma stomach. This case is reported because of its rarity in younger age group.

  9. A rare presentation of Klippel-Trenaunay syndrome

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    Tanweer Karim

    2014-01-01

    Full Text Available The Klippel-Trenaunay syndrome (KTS is a congenital disorder characterized by capillary malformation, varicosities and bony and soft tissue hypertrophy. This disease has several morbidities like bleeding, deep venous thrombosis, embolic complications and in some cases enlargement of limb that may require amputation. Vascular malformations are segmented and never cross midline. However, we came across a case, a 45-year-old male, who presented with varicosity of veins and deformity of left lower limb besides cavernous hemangiomas (port-wine stains scattered all over his face, chest, back, gluteal region, groin and legs since birth. Multiple paravertebral soft tissue masses and bladder hypertrophy were also noted due to involving neurofibromatosis. Simultaneous occurrence of KTS and neurofibromatosis is rarely seen in clinical practice.

  10. Joubert syndrome with autism in two siblings: A rare presentation.

    Science.gov (United States)

    Raghavan, D Vijaya; Doshi, V Vimal; Nambi, Shanthi

    2016-01-01

    Joubert syndrome is a rare autosomal recessive disorder with partial or complete agenesis of cerebellar vermis. This syndrome is identified mainly by the presence of molar tooth sign in magnetic resonance imaging of the brain since it has a varied phenotypic presentation. Of the 200 cases reported so far in the literature, only three reports show the presence of autistic symptoms in siblings suggesting a link between the cerebellar vermis and autistic spectrum disorders. In this case report of two siblings, the female child satisfied the criterion for autistic spectrum disorder in accordance with Diagnostic and Statistical Manual of Mental Disorders Fifth Editon. The boy showed developmental delay with autistic features (not amounting to diagnostic threshold). This report is important in that it adds evidence to the literature that abnormalities of cerebellum are involved in the cognitive development and autistic symptoms.

  11. EXCEPTIONALLY RARE VARIANTS OF THE URINARY SYSTEM ANOMALIES - ROENTGEN PRESENTATION

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    Rade R. Babić

    2002-07-01

    Full Text Available The results of the radiological study of the urinary system anomalies are presented on the material consisting of 8,568 urographies done from 1990 to 2001 at the Institute for Radiology, Niš. The paper shows exceptionally rare anomalies of the urinary system: a horse-shoe shaped kidney with pyelocaliceal systems in its arms and isthmus, heterolateral ectopia of the kidney with fusion, abdominal-medial ectopia of the kidney with ventral malrotation and cup hyperplasia, hypoplastic cup, triple pyeolcaliceal system, M. Lenarduzzi and blind-ending of the Y-shaped urethra. The author concludes that, for the sake of performing every day professional work, it is necessary to possess detailed knowledge of the rarest urinary system anomalies.

  12. Extraosseous Ewing′s tumor of larynx: A rare presentation

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    Vinod Shinde

    2015-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. PNET of the larynx is extremely rare. We report a case of a 41-year-old male who presented with the complaints of progressively increasing stridor of 3 months duration, which was diagnosed as a case of neuroectodermal tumor in the subglottis. Patient was subjected to microlaryngeal surgery and the tumor was excised. Postoperatively, patient was given three cycles of chemotherapy comprising of ifosfamide, etoposide, and mesna, along with granulocyte colony stimulating factor, with 21 days interval. After chemotherapy repeat computed tomography scan showed no evidence of the tumor and no lymphadenopathy. Patient is symptom free for 18 months following completion of treatment. He is under regular follow-up and is undergoing monthly serial endoscopic evaluation.

  13. Breast Cancer with Synchronous Renal Cell Carcinoma: A Rare Presentation.

    Science.gov (United States)

    Arjunan, Ravi; Kumar, Durgesh; Kumar, K V Veerendra; Premlatha, C S

    2016-10-01

    Primary cancer arising from multiple organs is a well known fact. Synchronous tumours have been most commonly associated with kidney cancer. Bladder, prostate, colorectal and lung cancer are the most common synchronous primaries with Renal Cell Carcinoma (RCC) identified till date. We found metachronous tumours of breast with RCC in literature search which included both metastatic tumours as well second primaries. Overall, 25 cases of metastatic breast tumours and eight cases of second primary in previously treated RCC have been reported in the literature. Here, we are reporting a case of synchronous presentation of carcinoma breast with RCC which is very rare because most of the multiple malignancies reported in the literature are metastatic tumours or metachronous breast malignancy with RCC.

  14. Meige's Syndrome: Rare Neurological Disorder Presenting as Conversion Disorder.

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    Debadatta, Mohapatra; Mishra, Ajay K

    2013-07-01

    Meige's syndrome is a rare neurological syndrome characterized by oromandibular dystonia and blepharospasm. Its pathophysiology is not clearly determined. A 35-year-old female presented to psychiatric department with blepharospasm and oromandibular dystonia with clinical provisional diagnosis of psychiatric disorder (Conversion Disorder). After thorough physical examination including detailed neurological exam and psychiatric evaluation no formal medical or psychiatric diagnosis could be made. The other differential diagnoses of extra pyramidal symptom, tardive dyskinesia, conversion disorder, anxiety disorder were ruled out by formal diagnostic criteria. Consequently with suspicion of Meige's syndrome she was referred to the department of Neurology and the diagnosis was confirmed. Hence, Meige's syndrome could be misdiagnosed as a psychiatric disorder such as conversion disorder or anxiety disorder because clinical features of Meige's syndrome are highly variable and affected by psychological factors and also can be inhibited voluntarily to some extent.

  15. Rare presentation of acute urinary retention secondary to herpes zoster.

    Science.gov (United States)

    Ginsberg, P C; Harkaway, R C; Elisco, A J; Rosenthal, B D

    1998-09-01

    There are many causes of acute urinary retention. Reported here is a case of one of the more rare causes: herpes zoster. Fewer than 70 cases have been reported in the literature since 1890. In the present clinical environment where many patients are immunocompromised, reports of herpes zoster and its sequelae are no longer thought of as anecdotal. The virus may interrupt the detrusor reflex due to involvement of the sacral dorsal root ganglia. Urinary retention with sensory loss of both bladder and rectum as well as flaccid paralysis of the detrusor can develop in patients with herpes zoster. Fortunately, the outcome of this process is benign and full recovery of the detrusor is likely.

  16. Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease.

    Science.gov (United States)

    Priyamvada, P S; Morkhandikar, S; Srinivas, B H; Parameswaran, S

    2015-01-01

    Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig) light chains. In heavy chain amyloidosis (AH), deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL), the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda) AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.

  17. Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease.

    Science.gov (United States)

    Abu Omar, Mohannad; Tylski, Emily; Abu Ghanimeh, Mouhanna; Gohar, Ashraf

    2016-09-26

    Congenital pulmonary airway malformation (CPAM) is a rare congenital abnormality with unknown exact aetiology or clear genetic association. It is characterised by a failure of bronchial development and localised glandular overgrowth. Typically, it is diagnosed on prenatal ultrasound, only infrequently in children, and even less commonly in adults. We present a case of a 25-year-old man, with no previous lung diseases who presented with right-sided chest pain, fever and cough suggestive of pulmonary infection. Chest imaging, including CT scan, showed a large focal cystic mass within the right lower lobe along with ground glass opacities suggestive of CPAM. He was started on intravenous antibiotics. Bronchoscopy showed a large amount of pus in the right lung and bronchoalveolar lavage confirmed the microbiological diagnosis of methicillin-resistant Staphylococcus aureus. He improved with antibiotic treatment. He was discharged with 6-week course of antibiotics and follow-up afterward. 2016 BMJ Publishing Group Ltd.

  18. Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease

    Directory of Open Access Journals (Sweden)

    P S Priyamvada

    2015-01-01

    Full Text Available Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig light chains. In heavy chain amyloidosis (AH, deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL, the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.

  19. Soft tissue chondroma: a rare tumor presenting as a cutaneous nodule

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    Dibakar Podder

    2015-04-01

    Full Text Available Soft tissue chondroma (STC, also known as extraskeletal chondroma or chondroma of soft parts is a benign cartilaginous tumor which arise de novo from soft tissue. Also, it is an extremely rare entity predominantly involving extremities, especially fingers. A 26 year old male presented with 3 year history of swelling in left index finger. On local examination a hard 2 × 2 cm swelling was seen over the volar aspect of left 2nd proximal phalanx. Swelling was mobile on contraction of tendons. X-ray showed a soft tissue shadow on volar aspect of left second proximal phalanx. Histopathology showed a well encapsulated, hypo cellular nodule composed of benign chondrocytes surrounded by hyaline chondroid matrix. Nuclear pleomorphism, mitosis or necrosis was not seen. Based on radiological and histopathological findings a diagnosis of STC was made. STC should be considered in patients with slow growing, soft tissue masses.

  20. Solitary Osteochondroma of the Thoracic Spine with Compressive Myelopathy; A Rare Presentation

    International Nuclear Information System (INIS)

    Mehrian, Payam; Karimi, Mohammad Ali; Kahkuee, Shahram; Bakhshayeshkaram, Mehrdad; Ghasemikhah, Reza

    2013-01-01

    A 19-year-old man presented with a 5-year history of back pain radiating to the lower extremities and paresthesis of the toes during the last year. Plain X-ray revealed a large cauliflower shaped exophytic mass at the level of T8, T9 and T10 vertebrae. Computed tomography (CT) and magnetic resonance imaging (MRI) showed an abnormal bony mass arising from the posterior arch of T9 with protrusion to the spinal canal and marked cord compression. The cortex and medulla of the lesion had continuity with those of the T9 vertebra. Surgical en bloc resection was performed and the patient’s symptoms resolved. The histopathologic diagnosis was osteochondroma. In patients with symptoms of myelopathy, in addition to more common etiologies, one should also be aware of rare entities such as osteochondroma

  1. Non-resolving pneumonia: A rare presentation of progressive disseminated histoplasmosis

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    Supriya Sarkar

    2014-01-01

    Full Text Available Histoplasmosis, a fungal disease caused by Histoplasma capsulatum, is endemic in North and South America. Except few scattered cases, the disease is considered to be a non-entity in India. Furthermore, disseminated histoplasmosis is rare in the immunocompetent individuals. We report an adolescent boy presenting as middle lobe consolidation which did not respond to antibiotics. His condition deteriorated with the development of mediastinal lymphadenopathy, pleural effusion and hepatosplenomegaly. A diagnosis of progressive disseminated histoplasmosis was established by his clinical findings as well as bronchoscopic biopsy, transbronchial needle aspiration cytology and bronchoalveolar lavage culture demonstrating Histoplasma capsulatum. The case represents a unique example of progressive disseminated histoplasmosis in an immunocompetent individual in India.

  2. Caudal duplication syndrome: imaging evaluation of a rare entity in an adult patient

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    Tianshen Hu, BS

    2016-03-01

    Full Text Available Several theories have been put forth to explain the complex yet symmetrical malformations and the myriad of clinical presentations of caudal duplication syndrome. Hereby, reported case is a 28-year-old female, gravida 2 para 2, with congenital caudal malformation who has undergone partial reconstructive surgeries in infancy to connect her 2 colons. She presented with recurrent left lower abdominal pain associated with nausea, vomiting, and subsequent feculent anal discharge. Imaging reveals duplication of the urinary bladder, urethra, and colon with with cloacal malformations and fistulae from the left-sided cloaca, uterus didelphys with separate cervices and vaginal canals, right-sided aortic arch and descending thoracic aorta, and dysraphic midline sacrococcygeal defect. Hydronephrosis of the left kidney with left hydroureter and inflammation of one of the colons were suspected to be the cause of the patient’s acute complaints. She improved symptomatically over the course of her hospitalization stay with conservative treatments. The management for this syndrome is individualized and may include surgical intervention to fuse or excise the duplicated organs.

  3. CLINICAL AND LABORATORY PROFILE OF PATIENTS WITH IDIOPATHIC CD4 LYMPHOCYTOPENIA- A RARE CLINICAL ENTITY

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    Vijayashree Thyagaraj

    2017-01-01

    Full Text Available BACKGROUND Since 1989, several investigators have reported unusual cases of severe opportunistic infections associated with CD4 lymphocytopenia in the absence of human immunodeficiency virus infection. The cause of this condition is unknown. The Centres for Disease Control and Prevention (CDC defines Idiopathic CD4 T Lymphocytopenia (ICL as a clinical condition in which patients with depressed numbers of circulating CD4+ T-cell lymphocytes (<300 cells/μL or <20% of total T cells at a minimum of two separate time points at least 6 weeks apart, have no laboratory evidence of infection with human HIV-1 or HIV-2, or any defined immunodeficiency or therapy associated with depressed levels of CD4 T cells. The aim of the study is to analyse the clinical profile, opportunistic infections, laboratory parameters and outcome in terms of survival of patients diagnosed with ICL. MATERIALS AND METHODS Eight HIV negative patients who presented with opportunistic infections and who were diagnosed with ICL from 2007 to 2015 were included in the study. A detailed history was taken; physical examination was performed and the nature of illness with which they presented was documented. Then, CD4 and CD8 counts were done and CD4 count was repeated after a 6-week interval. The patients were followed up until discharge or death. RESULTS The mean age was 37.50±9.55 years. There were six males (75% and two females (25%. Fever was a presenting symptom among six (75% of them. Two were diagnosed to have cutaneous cryptococcosis (25%, two with invasive aspergillosis (25% and four with tuberculosis (50%. Absolute lymphocyte count was less than 1200 in seven patients (87.5%, which roughly correlates with a CD4 count of less than 200 cells/μL, among PLWHIV. The mean CD4 count was 183.63±63.74 cells/μL during the first measurement and 214.43±103.98 cells/μL during the second one. Two patients died (37.5%. None of the patients were recorded to have any form of malignancy

  4. Catamenial pneumothorax: a rare entity? Report of 5 cases and review of the literature

    Science.gov (United States)

    Visouli, Aikaterini N.; Darwiche, Kaid; Mpakas, Andreas; Papagiannis, Antonios; Tsakiridis, Kosmas; Machairiotis, Nikolaos; Stylianaki, Aikaterini; Katsikogiannis, Nikolaos; Courcoutsakis, Nicolaos; Zarogoulidis, Konstantinos

    2012-01-01

    Objective Spontaneous recurrent pneumothorax during menstruation is reported as catamenial pneumothorax. It is encountered in 3-6% of spontaneous pneumothorax cases among menstruating women. The percentage among women referred for surgery is significantly higher (25-30%). Although it usually involves the right-side (85-95%) it can be left-sided or bilateral. It is associated with diaphragmatic perforations and/or thoracic endometriosis. There is pelvic endometriosis in up to 30-51% of cases. The lesions that are not always found may present as small or larger holes at the central tendon of the diaphragm, as red, blueberry, brown spots or larger nodules at the diaphragm, the visceral or parietal pleura. Lesion histology may reveal endometriosis. We present 5 cases of catamenial pneumothorax treated surgically during the last 6 years. Patients and methods Five women, with a mean age of 34+/-9.9 years (median 38, range, 19-45 years) presented with right-sided recurrent catamenial pneumothorax. In 3 patients diaphragmatic perforation(s) were found; perforation suturing (n=1), and diaphragmatic plication reinforced with bovine pericardial patch (n=1) were performed. All patients underwent atypical resection of upper and/or middle lobe segments of lung parenchyma that appeared abnormal (haemorrhagic/emphysematous or blebs). Four patients underwent pleurodesis and 1 patient underwent pleurectomy. Four interventions were performed through video assisted thoracoscopic surgery, while diaphragmatic plication was performed through a video assisted mini-thoracotomy. Histology did not reveal endometriosis tissue. Results The postoperative course was uneventful. The patients were extubated in theatre and were discharged home at a mean of 7+/-4 days (median 6 days, range, 4-14 days). Two of them received hormonal therapy [Gonadotropin Releasing Hormone (GnRH) analogue] postoperatively. At a follow-up of 14.16 patient-years (mean 2.83+/-1.08 years, range, 1.33-3.83 years) there was

  5. Primary tuberculous gingival enlargement - A rare clinical entity: Case report and brief review of the literature

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    Suhail Majid Jan

    2014-01-01

    Full Text Available Tuberculosis (TB is a chronic specific granulomatous disease and a major cause of death in developing countries. The clinical presentation of TB lesions of the oral cavity varies widely and can manifest as ulcerations, diffuse inflammatory lesions, granulomas and fissures. Oral lesions generally appear secondary to primary TB infection elsewhere, although primary infection of the oral mucosa by Mycobacterium tuberculosis has also been described. We hereby report a case of primary TB of the gingiva manifesting as gingival enlargement. Diagnosis was based on histopathological examination, complete blood count, X-ray chest and immunological investigations with detection of antibodies against M. tuberculosis. Anti-tuberculous therapy was carried out for over 6 months and was followed by surgical excision of the residual enlargement under local anesthesia. After 1-year follow-up, there was no recurrence of the disease. This case report emphasizes the need for dentists to include TB in the differential diagnosis of various types of gingival enlargements.

  6. Late onset ‘en coup de sabre’ following trauma: Rare presentation of a rare disease

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    Tasleem Arif

    2015-01-01

    Full Text Available En coup de sabre (linear scleroderma of face is a rare type of morphea (localized scleroderma involving frontoparietal area of the forehead and scalp. Many triggering factors have been implicated in the development of morphea like trauma, immobilization, bacille Calmette–Guérin (BCG vaccination, injections of vitamin K, mechanical compression from clothing, etc. Linear scleroderma primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. In this article, we describe a case of 26 year old female who presented with a three months history of brownish indurated plaque of skin on the frontal and forehead regions of the head. The patient gave a history of trauma at the same site six years back. The diagnosis of morphea was made clinically supported by histopathological features of the skin biopsy. Her neurological examination was normal. ANA was negative. Brain MRI didn’t reveal any abnormality. She was treated with topical tacrolimus 0.1% ointment. The late onset en coup de sabre is a rare presentation and hence reported.

  7. Parathyroid carcinoma: an unusual presentation of a rare neoplasm

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    Shruti, Sharma

    2017-12-01

    Full Text Available Parathyroid carcinoma is an extremely rare malignant endocrine neoplasm that is very challenging in its diagnosis as well as its treatment. Clinically the disease is detected earlier in patients who present with hyperparathyroidism with signs of profound hypercalcemia. Differentiation between benign and malignant disease of the parathyroid is challenging both for the clinician and for the pathologist. Complete surgical resection at the time of first operation offers the best chance of cure. Even after radical excision which is the standard management, local recurrence and metastases are frequent. The disease usually has a slow indolent course and most patients suffer from complications of hypercalcemia rather than tumor invasion or metastasis.We report a case of a 31-year-old woman who presented with renal colic. Various hematological, biochemical and radiological investigations were performed and a slightly enlarged right parathyroid was found. A clinical diagnosis of parathyroid adenoma was made and a right parathyroidectomy was done. Intraoperatively the surgeon had no suspicion of malignancy but microscopically the lesion was malignant and a final diagnosis of parathyroid carcinoma was rendered based on the criteria of invasion. Since there is no gold standard, a multidisciplinary approach, including the entire clinical, biochemical, radiological and pathological profile of the disease aids in an accurate diagnosis. Here we are reporting a case of a functional parathyroid carcinoma presenting in a relatively young patient with all the biochemical and radiological investigations and findings pointing towards a benign parathyroid disease.

  8. Cyclopia: A Rare Condition with Unusual Presentation - A Case Report

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    Ghassan S.A. Salama

    2015-01-01

    Full Text Available Introduction Cyclopia (alobar holoprosencephaly (OMIM% 236100 is a rare and lethal complex human malformation, resulting from incomplete cleavage of prosencephalon into right and left hemispheres occurring between the 18th and the 28th day of gestation. Holoprosencephaly occurs in 1/16,000 live births, and 1/250 during embryogenesis. Approximately 1.05 in 100,000 births are identified as infants with cyclopia, including stillbirths. Cyclopia typically presents with a median single eye or a partially divided eye in a single orbit, absent nose, and a proboscis above the eye. Extracranial malformations described in stillbirths with cyclopia include polydactyl, renal dysplasia, and an omphalocele. The etiology of this rare syndrome, which is incompatible with life, is still largely unknown. Most cases are sporadic. Heterogeneous risk factors have been implicated as possible causes. Case Presentation A live full-term baby with birth weight of 2900 g, product of cesarean section because of severe fetal bradycardia, was born at Prince Hashem Military Hospital – Zarqa city/Jordan. This newborn was the first baby to a non-consanguineous family, and a healthy 18-year-old mother, with no history of drug ingestion or febrile illnesses during pregnancy. Antenatal history revealed severe hydrocephalus diagnosed early by intrauterine ultrasound but the pregnancy was not terminated because of the lack of medical legitimization in the country. On examination, the newborn was found to have a dysmorphic face, with a median single eye, absence of nose, micrognathia, and a proboscis above the eye, all of which made cyclopia the possible initial diagnosis. Multiple unusual abdominal defects were present that include a huge omphalocele containing whole liver and spleen, urinary bladder extrophy, and undefined abnormal external genitalia, which called for urgent confirmation. Brain MRI was done and revealed findings consistent with alobar holoprosencephaly (cyclopia

  9. Bilateral olecranon bursitis – A rare clinical presentation of gout

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    Betul Sargın

    2018-04-01

    Full Text Available Background: Gout is the most common form of crystal arthropathy. Monoarthritis affecting the first metatarsophalangeal joint is the common initial presentation. Bilateral olecranon bursitis is a rare presentation of gout. Aim of the work: To describe the clinical features of bilateral olecranon bursitis as an initial presentation of gout. Case report: A 62-year old male patient presented to the rheumatology clinic , Adnan Menderes University with sudden bilateral elbow pain and swelling for three months . Swellings gradually increased to the size of a golf ball with minimal restriction in the elbow extension (170°. He didn’t have arthritis in the elbows. The patient had medical history of heart failure and chronic obstructive pulmonary disease and medications received included acetylsalicylic acid and diuretics. Blood tests revealed elevated serum uric acid (12.6 mg/dL, with normal renal function tests, erythrocyte sedimentation rate (ESR 43 mm/h and C-reactive protein (CRP 8.8 mg/L. Rheumatoid factor and hepatitis markers were negative. Diagnostic bursal aspiration excluded septic bursitis and under polarized microscopy monosodium urate (MSU crystals were identified with typical negative birefringence. A diagnosis of gout was established. ESR and CRP were normalized after diclofenac potassium (100 mg and colchicine (1.5 mg. Allopurinol 300 mg was added when his joint pain was relieved. Conclusion: This is the first gout case initially presenting with bilateral olecranon bursitis. Bursal fluid analysis is important in such atypical presentation to look for MSU crystals and establish a diagnosis. Keywords: Olecranon bursitis, Gout, Crystal arthropathy, Monosodium urate

  10. Metastatic Choriocarcinoma Following Live Birth – A Rare Presentation

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    Joya Sree Roy

    2011-09-01

    Full Text Available Choriocarcinoma is the most malignant tumor of gestational trophoblastic neoplasia. Post-partum choriocarcinoma is an infrequent event with poor prognosis. The diagnosis is usually delayed due to failure to recognize the mode of presentation of this disease. Being a rare occurrence, limited data is available regarding its clinical features. We present a 24 years old women with parity one delivered by caesarean section indicated for premature rupture of membrane with fetal distress at 39 wks of pregnancy. Frequent episodes of heavy vaginal bleeding had started 28 days following C/S. For this, she had H/O uterine evacuatin twice within 5 days interval. Biopsy report of second curettage showed choriocarcinoma. On admission to Dhaka Medical College Hospital (DMCH, the pretreatment Human Chorionic Gonadotrophin (βhCG level was >200000.0 IU/L, uterine mass of about 18 wks pregnancy size & X-Ray chest showed segmental consolidation in left mid zone of lung. After consultation with oncologist Etoposide, Methotrexate, Dactinomycin, Cyclophosphamide & Vincristine (EMACO therapy was started but before completion of her proposed cycles, she developed a live threatening condition which was managed very urgently and meticulously. Now the patient is under regular monitoring. Key words: GTD; Choriocarcinoma DOI: http://dx.doi.org/10.3329/bsmmuj.v4i2.8642 BSMMU J 2011; 4(2:116-118

  11. Basaloid carcinoma of the pancreas--clinicopathological presentation and oncogenetic snapshot of a rare entity.

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    Szasz, A Marcell; Szirtes, Ildiko; Tihanyi, Balazs; Barkaszi, Bernadett; Baranyai, Zsolt; Tihanyi, Tibor; Harsanyi, Laszlo; Timar, Jozsef; Kulka, Janina

    2015-02-01

    We report a case of basaloid pancreatic carcinoma with clinical, pathological, and genomic data. The 73-year-old male patient had jaundice, acholic stool, diarrhea, weight loss, and a large, painless gall bladder. His GGT was highly elevated. The pancreatic head contained a tumor, which was resected by partial pancreatoduodenectomy with pancreato-gastric anastomosis, cholecystectomy, and lymphadenectomy. On gross examination, a 3.8-cm white firm nodule was found, which microscopically was composed of basaloid cell nests with a less than usual desmoplastic stromal background and focally PANIN. Immunohistochemical profile displayed strong CK5/6, CK19, p63, EGFR, vimentin, and evident CK14 expression and absence of expression of CK7, chromogranin, synaptophysin, and BRCA1. A high Ki-67 index and p53 expression were noted. Sequencing of the most frequent 46 oncogenes with ionTorrent (AmpliSeq PCR) method identified PIK3CA, KRAS, and TP53 genes as drivers and variants of the FGFR3, PDGFRA, KIT, KDR, EGFR, RET, and ATM genes. The tumor we report displays histopathological appearances similar to the previously described case and a genomic landscape fitting to the general population of pancreatic carcinomas. We hypothesize that this tumor may belong to the group of DNA damage repair-deficient pancreatic carcinoma subgroup.

  12. Effect of compositional variations on charge compensation of AlO4 and BO4 entities and on crystallization tendency of a rare-earth-rich aluminoborosilicate glass

    International Nuclear Information System (INIS)

    Quintas, A.; Caurant, D.; Majerus, O.; Charpentier, T.; Dussossoy, J.-L.

    2009-01-01

    This paper presents the structural and crystallization study of a rare-earth-rich aluminoborosilicate glass that is a simplified version of a new nuclear glass proven to be a potential candidate for the immobilization of highly concentrated radioactive wastes that will be produced in the future. In this work, we studied the impact of changing the nature of alkali (Li + , Na + , K + , Rb + , Cs + ) or alkaline-earth (Mg 2+ , Ca 2+ , Sr 2+ , Ba 2+ ) cations present in glass composition on glass structure (by 27 Al and 11 B nuclear magnetic resonance spectroscopy) and on its crystallization tendency during melt cooling at 1 K/min (average cooling rate during industrial process). From these composition changes, it was established that alkali cations were preferentially involved in charge compensation of (AlO 4 ) - and (BO 4 ) - entities in the glassy network comparatively to alkaline-earth cations. Whatever the nature of alkali cations, glass compositions containing calcium gave way to the crystallization of an apatite silicate phase bearing calcium and rare-earth (RE) cations (Ca 2 RE 8 (SiO 4 ) 6 O 2 , RE = Nd or La) but melt crystallization tendency during cooling strongly varied with the nature of alkaline-earth cations.

  13. A case of bone marrow necrosis of an idiopathic aetiology: the report of a rare entity with review of the literature.

    Science.gov (United States)

    Bhasin, Tejinder Singh; Sharma, Sonam; Chandey, Manish; Bhatia, Puneet Kaur; Mannan, Rahul

    2013-03-01

    Bone Marrow Necrosis (BMN) is regarded as a rare entity in the aspirates and the trephine biopsies which are obtained from living patients and it is a rare antemortem diagnosis. Usually, BMN is associated with a poor prognosis and it has a 90% association with malignancies (mainly haematological). Its other causes include infections, drugs and other non malignant haematological causes. BMN which occurs due to unknown causes (idiopathic), is very rare. The present case report describes a case of BMN in a 14 year old male child from rural background, who came to the medicine outpatient department of a tertiary care centre in north India, with the complaints of generalized weakness, severe bone pains, especially in the ribcage area and fatigue. His peripheral blood film revealed pancytopaenia. His bone marrow aspiration yielded a watery, sero-sanguinous fluid from 2 different sites. Microscopy revealed "ghost like" haematopoietic cells lying in eosinophilic, proteinaceous material. Bone marrow biopsies from both the sites revealed extensive foci of gelatinous transformation and necrosis. A diagnosis of BMN was rendered. No underlying neoplastic / non neoplastic cause was identified, even after a thorough systematic investigation (haematological/biochemical/ radiological and immunological). Thus, a final diagnosis of BMN due to an idiopathic cause, was finally given. The patient was given supportive treatment, after which he was referred to a higher centre. BMN which arises idiopathically is usually very rare and often precedes the aetiological diagnosis and obscures the diagnosis. Traditionally, the prognoses of the patients with BMN were considered as poor, but with better treatment aids (chemotherapy and supportive treatment), the prognoses of these patients have been found to improve. Vigorous supportive care, along with a disease specific treatment, is the key to its management. This case was worth reporting, as no cause of BMN was elicited in it and we have

  14. Suprasellar ganglioglioma presenting with diabetes insipidus in a young boy: a rare clinical presentation.

    Science.gov (United States)

    Gupta, Ruchika; Suri, Vaishali; Arora, Raman; Sharma, Mehar C; Mishra, Shashwat; Singh, Manmohan; Sarkar, Chitra

    2010-02-01

    Gangliogliomas are rare tumors composed of an admixture of glial and neuronal components. These usually occur in young patients, who present with therapy-resistant seizures. Clinical presentation of ganglioglioma with diabetes insipidus is extremely rare with only one case reported earlier in the available literature. Due to this rarity, ganglioglioma is not considered in the differential diagnosis in a patient with diabetes insipidus. A 7-year boy presented with polyuria, polydipsia, and progressive visual loss for 18 months. Investigations revealed diabetes insipidus. Radiographic studies of the brain showed a solid and cystic mass in the suprasellar region effacing the third ventricle. Intraoperatively, diffuse thickening of bilateral optic nerves and optic chiasma was noted and a diagnosis of optic glioma was considered. A biopsy of the mass was taken, which on histopathological examination showed features of ganglioglioma. The patient was referred for further radiotherapy but was lost to follow-up. Diabetes insipidus as a presenting symptom of ganglioglioma is extremely rare. This benign tumor should be kept in mind in patients with central diabetes insipidus and a suprasellar mass lesion. This report describes the second such case in the literature.

  15. A rare case of vulvar squamous cell carcinoma; case presentation

    Directory of Open Access Journals (Sweden)

    Cristina N. Cozma

    2018-05-01

    Full Text Available Objectives. Vulvar cancer is a rare gynecological malignancy, with an incidence of 1.5 per 100 000 women/year. The most common vulvar cancer is developed in squamous cells, the most encountered type of skin cells. Case report. We report a case of a 72-year-old female admitted in the Department of Plastic Surgery of Emergency Clinical Hospital “Prof. Dr. Agrippa Ionescu” with a 5/4.2 cm painful ulcerated tumoral mass located in the vulvar area. The lesion slowly increased in size over the past 12 months. The tumour was surgically removed with oncological safety margins and sent for histopathological evaluation. The histopathological examination revealed an ulcerated squamous carcinoma with lymphovascular and perineural invasion, but with negative margins. Postoperative results were favorable, and no local or general complications were observed. Conclusion. We highlight this case due to its unusual presentation in the clitoral area. Moreover, considering the potential for recurrence we point out the importance of the radical vulvectomy with regional lymphadenectomy and histopathological examination, in order to put a precise diagnosis and ensure the best possible treatment for the patient.

  16. Chronic meningococcemia: a rare presentation of meningococcal disease: case report

    Directory of Open Access Journals (Sweden)

    Antonio Adolfo Guerra Soares Brandão

    2012-03-01

    Full Text Available Chronic meningococcemia is a rare clinical presentation within the spectrumof infections due to Neisseria meningitidis, which was first described in 1902.It is defined as a chronic and benign meningococcal bacteremia withoutmeningeal signs or symptoms with at least one week’s duration, characterizedby intermittent or continuous fever, polymorphic cutaneous rash, and migratoryarthropathy. The incidence is believed to be around 1:200,000 inhabitants. Itaffects predominantly young people and adults, and it is equally distributedbetween genders. Diagnosis may be challenging in the early stages of thedisease because of the difficulty in isolating Neisseria meningitidis (it reaches74% of positivity in advanced stages. Recently, the use of PCR for detectingNeisseria sp antigen in skin biopsies specimens has been considered for thoseculture-negative cases. The authors report a case of a 54-year-old femalepatient who sought medical attention for a five-day fever followed by arthralgiaand skin lesions predominantly in the lower limbs. The patient progressed toa toxemic clinical status that improved after the administration of antibiotictherapy, which consisted of oxacillin and ceftriaxone. The diagnosis of chronicmeningococcemia was performed after the isolation of Neisseria meningitidisin two different blood sample cultures. This is, to our knowledge, the firstcase of chronic meningococcemia described in Brazil (up to the writing of thisreport.

  17.  Papillary Cystadenocarcinoma of Ovary Presenting as Obstructive Jaundice: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Sunil Kumar

    2012-03-01

    Full Text Available  Obstructive jaundice resulting from malignancy of gastrointestinal tract and hepatobiliary tract has been reported in various studies. Ovarian malignancy leading to obstructive jaundice due to portal lymphadenopathy is of rare occurrence. We report a case presented with obstructive jaundice and on further evaluation, found to have ovarian papillary cyst adenocarcinoma with secondaries at porta hepatis which was managed successfully by neoadjuvant chemotherapy followed by cytoreductive surgery.

  18. Typhoid Fever Presenting With Acute Renal Failure And Hepatitis Simultaneously - A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Rajput R.

    2016-05-01

    Full Text Available Typhoid fever is an important health problem worldwide but its incidence is more in developing countries. Hepatic involvement is common, but both hepatic and renal involvement is rare in typhoid fever. We report a case of typhoid fever presenting with hepatitis and acute renal failure. A 17 year old male presenting with fever and pain abdomen was found to have raised blood urea, creatinine, liver enzymes and bilirubin. Widal and typhidot (IgM,IgG test were positive. His symptoms subsided and deranged parameters resolved with treatment of typhoid fever.

  19. The present status of rare gas release control

    International Nuclear Information System (INIS)

    Yamamoto, Hiroshi

    1974-01-01

    Of the rare gases Ar, Kr and Xe released from nuclear facilities, the problem of release control can be confined to 41 Ar, 85 Kr and 133 Xe. The cases of the latter two are described, as 41 Ar is not much significant. 133 Xe, having relatively short half-life, can be dealt sufficiently by holding-up in case of light water reactors. 85 Kr of long half-life must be removed : the methods are low temperature adsorption, liquefaction distillation, absorption and diaphragm method. As for future problem, there is disposal of concentrated rare gas. (Mori, K.)

  20. A rare presentation of disseminated tuberculosis: Prostatic abscess.

    Science.gov (United States)

    Verma, Ajay; Singh, Anubhuti; Kishore, Kislay; Kant, Surya

    2017-10-01

    Involvement of the prostate by tuberculosis (TB) occurs rarely and tuberculosis prostate abscess is an even rarer occurrence. It has been reported in immunocompromised patients, mainly human immunodeficiency virus seropositive individuals. We are reporting a case of tuberculosis prostatic abscess in an immunocompetent patient with relapse of TB. Copyright © 2016 Tuberculosis Association of India. Published by Elsevier B.V. All rights reserved.

  1. Fine-needle aspiration findings of a rare hematopoietic neoplasm presenting as obstructive jaundice.

    Science.gov (United States)

    Tracht, Jessica; Ahmed, Ali M; Rosenblum Donath, Frida

    2017-12-01

    A 51-year-old female who presented with obstructive jaundice was found to have masses in the pancreatic head and tail as well as suspicious liver and periaortic masses on imaging. Aspiration cytology of the pancreatic tail mass showed abundant large single cells with vacuolated eosinophilic cytoplasm, marked nuclear pleomorphism, large bizarre irregular nuclei, binucleation, and prominent nucleoli. Numerous cells also showed intracytoplasmic black to brown pigmentation. A cell block was obtained and extensive immunohistochemical staining was performed. S-100, HMB-45, Sox10, pancytokeratin, CK7, RCC antigen, synaptophysin, HepPar 1, inhibin, CD45, CD21, and CD123 were negative, making melanoma, epithelial malignancies, lymphoma, follicular dendritic and plasmacytoid dendritic cell neoplasms less likely. CD4 and CD56 showed partial positivity, and CD68, CD163, and CD14 were positive, supporting the diagnosis of histiocytic sarcoma. Surgical specimens and immunohistochemistry confirmed the cytologic findings. Histiocytic sarcoma is a rare aggressive malignancy of histiocytic origin with most cases presenting in adults in extranodal sites, most commonly the intestinal tract. Few cases are reported in the literature, presenting diagnostic challenges for cytopathologists when seen on fine-needle aspiration. We present the first reported case of histiocytic sarcoma presenting as a pancreatic mass, diagnosed by endoscopic ultrasound guided fine-needle aspiration (EUS-FNA). This entity is rarely described on cytology and arose in a location in which EUS-FNA is the diagnostic modality of choice. This case study highlights that cytopathologists should be aware of histiocytic sarcoma occurring in extranodal locations accessible by EUS-FNA and be familiar with the cytomorphologic appearance. © 2017 Wiley Periodicals, Inc.

  2. Glial heterotopia of the lip: A rare presentation.

    Science.gov (United States)

    Dadaci, Mehmet; Bayram, Fazli Cengiz; Ince, Bilsev; Bilgen, Fatma

    2016-01-01

    Glial heterotopia represents collections of normal glial tissue in an abnormal location distant to the central nervous system or spinal canal with no intracranial connectivity. Nasal gliomas are non-neoplastic midline tumours, with limited growth potential and no similarity to the central nervous system gliomas. The nose and the nasopharynx are the most common sites of location. Existence of glial heterotopia in the lip region is a rare developmental disorder. We report a case of large glial heterotopia in the upper lip region in a full-term female newborn which had intracranial extension with a fibrotic band. After the surgery, there was no recurrence in the follow-up period of 3 years. When glial heterotopia, which is a rare midline anomaly, is suspected, possible intracranial connection and properties of the mass should be evaluated by magnetic resonance imaging. By this way, lower complication rate and better aesthetic results can be achieved with early diagnosis and proper surgery.

  3. Glial heterotopia of the lip: A rare presentation

    Directory of Open Access Journals (Sweden)

    Mehmet Dadaci

    2016-01-01

    Full Text Available Glial heterotopia represents collections of normal glial tissue in an abnormal location distant to the central nervous system or spinal canal with no intracranial connectivity. Nasal gliomas are non-neoplastic midline tumours, with limited growth potential and no similarity to the central nervous system gliomas. The nose and the nasopharynx are the most common sites of location. Existence of glial heterotopia in the lip region is a rare developmental disorder. We report a case of large glial heterotopia in the upper lip region in a full-term female newborn which had intracranial extension with a fibrotic band. After the surgery, there was no recurrence in the follow-up period of 3 years. When glial heterotopia, which is a rare midline anomaly, is suspected, possible intracranial connection and properties of the mass should be evaluated by magnetic resonance imaging. By this way, lower complication rate and better aesthetic results can be achieved with early diagnosis and proper surgery.

  4. Acute Abdomen: A Rare Presentation of Lung Cancer Metastasis

    OpenAIRE

    Guérin, E.; Gilbert, O.; Dequanter, D.

    2009-01-01

    Surgical emergencies caused by bowel metastases from carcinoma of the lung are very rare. We describe two cases of symptomatic gastrointestinal metastatic small cell carcinoma: the first one concerns a 69-year-old man with an acute abdomen and the second is a 72-year-old man complaining of a gastric ulcer symptoms. We also discuss the current management and the prognosis of these patients.

  5. [Acute renal failure: a rare presentation of Addison's disease].

    Science.gov (United States)

    Salhi, Houda

    2016-01-01

    Addison's disease is a rare condition. Its onset of symptoms most often is nonspecific contributing to a diagnostic and therapeutic delay. Acute renal failure can be the first manifestation of this disease. We report the case of a patient with Addison's disease who was initially treated for acute renal failure due to multiple myeloma and whose diagnosis was adjusted thereafter. Patient's condition dramatically improved after treatment with intravenous rehydration; injectable hydrocortisone.

  6. A rare presentation of an ectopic breast tissue in axilla

    Directory of Open Access Journals (Sweden)

    Radhika Vidyasagar

    2015-10-01

    Full Text Available Accessory breast tissue is rare accounting to less than 1% cases seen in females. It is usually bilateral. We report a case of 24-year-old woman with a lump in the left axilla in view of its rarity and made a differential diagnosis of fibroadenoma, which following the investigations and histopathological report was confirmed as revealed fibroadenoma in the axilla. It should also be considered as a differential diagnosis for all axillary swellings.

  7. Pseudohypertension-Like Presentation: An Exceptionally Rare Presentation in an Athletic Female Patient with Morphea

    Directory of Open Access Journals (Sweden)

    Ahmed Al-Imam

    2016-01-01

    Full Text Available Introduction. Pseudohypertension is a condition which mainly occurs due to thickening-calcification of tunica intima of the arterial wall, leading to a faulty measurement of the intra-arterial blood pressure. To the best of our knowledge, this is the first case report in literature, of a pseudohypertension-like presentation in association with Morphea en plaque. Case Presentation. This is a rare presentation of a young athletic female and a professional tennis player, with pseudohypertension-like presentation. The patient had a traumatic injury to the right elbow joint; the injury occurred during a professional tennis match. The injury was managed by immobilization, physiotherapy, and Low-Level Laser Therapy. Soon after that, the patient had a circumscribed sclerotic ivory plaque affecting the skin of the right cubital fossa. The histopathology analysis, together with the serological-hematological tests and the clinical picture, along with positive Osler’s signs, leads to the conclusive diagnosis of Morphea en plaque. The peculiar anatomic localization of the plaque anterior to the brachial artery leads to faulty blood pressure measurement as recorded by mercurial sphygmomanometer. Conclusion. This unique presentation of Morphea en plaque carries an important message in relation to the basic medical practice and in relation to the accurate measurement of the vital signs.

  8. Ischemic Gastritis: A Multicenter Case Series of a Rare Clinical Entity and a Review of the Literature.

    Science.gov (United States)

    Elwir, Saleh; Shaukat, Aasma; Mesa, Hector; Colbach, Christine; Dambowy, Paul; Shaw, Michael

    2016-10-01

    To report a case series of ischemic gastritis and discuss its etiology, management, and associated mortality according to our results and the published English literature. Ischemic gastritis is rare, given the rich blood supply of the stomach. It has been reported in isolated case reports and small case series. Most cases are vascular in origin and associated with a high mortality. Pathology databases from 3 hospitals affiliated with the University of Minnesota Medical School were searched for cases of ischemic gastritis in the last 10 years. Patients' demographics, clinical course, and 1-month and 1-year mortalities were collected from electronic medical records. A total of 12 patients were identified (age range, 32.1 to 83.2), the largest series reported to date. The presenting symptom was gastrointestinal bleeding (8), abdominal pain (2), nausea (1), and symptomatic anemia (1). The etiology included postinterventional radiology embolization (2), hemodynamic changes in the setting of celiac axis stenosis (2), vasculitis (1), systemic hypotension (1), and unknown (6). Treatment included steroid therapy, revascularization by interventional radiology, surgery, or supportive treatment. Thirty-day and 1-year mortalities were 33% and 41%, respectively. Ischemic gastritis is rare, but associated with a high mortality. Evaluation for treatable etiologies should be sought and corrected if present.

  9. Late onset ‘en coup de sabre’ following trauma: Rare presentation of a rare disease

    OpenAIRE

    Tasleem Arif; Imran Majid; Mir Laieq Ishtiyaq Haji

    2015-01-01

    En coup de sabre (linear scleroderma of face) is a rare type of morphea (localized scleroderma) involving frontoparietal area of the forehead and scalp. Many triggering factors have been implicated in the development of morphea like trauma, immobilization, bacille Calmette–Guérin (BCG) vaccination, injections of vitamin K, mechanical compression from clothing, etc. Linear scleroderma primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age....

  10. A Rare Presentation of Lymphoma of the Cervix with Cross-Sectional Imaging Correlation

    Directory of Open Access Journals (Sweden)

    Brinda Rao Korivi

    2014-01-01

    Full Text Available Non-Hodgkin’s lymphoma of the cervix is an extremely uncommon entity, with no standard established treatment protocol. A 43-year-old asymptomatic female with a history of dual hit blastic B-cell lymphoma/leukemia in complete remission presented with an incidental cervical mass, which was initially felt to represent a cervical fibroid on computed tomography (CT. It was further evaluated with ultrasound, biopsy, and positron emission tomography-computed tomography (PET-CT, which demonstrated a growing biopsy-proven lymphomatous mass and new humeral head lesion. The patient was started on chemotherapy to control the newly diagnosed humeral head lesion, which then regressed. She then underwent radiation to the cervix with significant improvement in the cervical lymphoma. A review of cross-sectional imaging findings of lymphoma of the cervix is provided, including how to differentiate it from other more common diseases of the cervix. Clinical awareness of rare cervical masses such as lymphoma is very important in order to achieve timely diagnosis and appropriate treatment.

  11. Primary carcinoid tumor arising within mature teratoma of the kidney: report of a rare entity and review of the literature

    Directory of Open Access Journals (Sweden)

    Parwani Anil V

    2007-05-01

    Full Text Available Abstract Background Primary carcinoid tumor arising within mature teratoma of the kidney is extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary carcinoid tumor arising within mature teratoma of the kidney. Methods Six previously reported case reports were identified using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. We also searched the electronic medical archival records of our institution and identified one additional unreported case. Data were extracted on the demographics, predisposing factors, clinical presentation, radiographic features, gross pathology, microscopic pathology, immunophenotype, therapy, and outcome of each of these seven cases. Results Primary carcinoid tumor arising within mature teratoma of the kidney was found at a mean age of 41.4 years. Of the 7 cases, 3 were female and 4 were male. Two of the 7 cases (28.6% were associated with horseshoe kidney. It typically presented with abdominal pain without carcinoid syndrome. It typical radiologic appearance was well circumscribed partly calcified Bosniak II-III lesion. Histologically, the carcinoid tumor showed monotonous small round cells arranged in classic anastomosing cords/ribbons intermixed with solid nests. Surgery was curative, no additional treatment was required, no local recurrences occurred, and no metastases occurred in all 7 cases. The 3 cases with available outcome data were alive at the time of publication of their respective cases (mean, 5 months. Conclusion Primary carcinoid tumor arising within mature teratoma of the kidney is a rare tumor that typically presents with abdominal pain without carcinoid syndrome. It is not associated with local recurrence and metastasis, is surgically curable, and has excellent prognosis.

  12. Vogt-Koyanagi-Harada disease, a rare entity in Spain: the challenge of worldwide immigration and globalization

    Directory of Open Access Journals (Sweden)

    Alberto Benavente Fernández

    2018-05-01

    Full Text Available Vogt–Koyanagi–Harada disease is rare, mediated by autoimmune melanocyte inflammation and facilitated by genetic predisposition[1-3]. The main clinical features include uveitis, meningitis, tinnitus and sensorineural deafness, and skin and hair depigmentation. It usually develops in four consecutive stages: prodromal, acute uveitic, convalescent, and chronic or recurrent[4]. In view of the first two stages, the differential diagnosis takes into account uveo-meningeal syndromes. Treatment is based on high dose corticosteroids. We present the case of a 14-year-old girl admitted to hospital with fever, progressive uveo-meningeal symptoms, and sensorineural hearing loss. After work-up, the final diagnosis of Vogt–Koyanagi–Harada disease was made.

  13. Primary hyperparathyroidism with rare presentation as multiple brown tumours

    Directory of Open Access Journals (Sweden)

    Smit Doshi

    2012-04-01

    Full Text Available We present a case of primary hyperparathyroidism with an uncommon presentation as multiple brown tumours, which may easily be mistaken for a primary bone neoplasm. A brief literature review and its clinical and surgical management are also discussed here.

  14. Mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland: A rare presentation

    Directory of Open Access Journals (Sweden)

    Lily Daniel

    2014-01-01

    Full Text Available Carcinoma ex pleomorphic adenoma in lacrimal gland is a rare entity unlike its salivary gland counterpart. This rare tumor poses a diagnostic challenge to clinicians as pre-operative diagnosis is difficult and diagnosis is only by careful pathological assessment. We report this uncommon lesion in a 62-year-old lady, wherein the malignant component was mucoepidermoid carcinoma. The elderly patient remained clinically and radiologically free of the tumor for two years after complete excision of the tumor but computed tomography at the end of two and a half years showed a recurrent lesion in the region of the lacrimal gland. This makes long term follow up of patients with these rare lacrimal tumors imperative with a minimum period of at least five years.

  15. Massive hemoptysis as a rare presentation of metastatic choriocarcinoma

    International Nuclear Information System (INIS)

    Husain, S.J.; Zubairi, A.B.S.; Yunus, A.; Jamil, S.

    2006-01-01

    Choriocarcinoma is an uncommon but rapidly invasive and widely metastasizing tumor. Lung is the commonest organ of spread, usually presenting as mild hemoptysis and chest pain in a patient with a recent miscarriage or known molar pregnancy. One such case of a 25 years old woman is reported who did not have any history of recent pregnancy or miscarriage and presented with a life-threatening massive hemoptysis. She responded well to therapy once the diagnosis of metastatic choriocarcinoma was established. (author)

  16. Localized cutaneous mucinosis associated with multiple myeloma: A rare presentation

    Directory of Open Access Journals (Sweden)

    Parvaiz Anwar Rather

    2014-01-01

    Full Text Available Lichen myxoedematosus (LM, a form of primary cutaneous mucinosis, may present either as localized less severe form called papular mucinosis or diffuse more severe form called scleromyxoedema. The diffuse form is almost always associated with monoclonal gammopathy, whereas localized form is not. We report an atypical case of localized form of LM associated with multiple myeloma in a 66-year-old male, who presented with asymptomatic waxy papular eruption on extremities, which on histopathological examination confirmed the diagnosis of cutaneous mucinosis. After initially being put on steroids and hydroxychloroquine with minimal improvement, patient subsequently presented with encephalopathy and on evaluation revealed hypernatremia, hypercalcemia, hypergammaglobulinemia, reversal of albumin-globulin (A/G ratio, azotemia, and lytic lesions in skull X-ray. Bone marrow aspiration and biopsy confirmed multiple myeloma. Patient was successfully treated with standard treatment regimen for multiple myeloma with bortezumib and dexamethasone and his skin lesions subsided completely.

  17. Bilateral Morgagni Hernia: A Unique Presentation of a Rare Pathology

    Directory of Open Access Journals (Sweden)

    Michael Leshen

    2016-01-01

    Full Text Available Morgagni hernia is an unusual congenital herniation of abdominal content through the triangular parasternal gaps of the anterior diaphragm. They are commonly asymptomatic and right-sided. We present a case of a bilateral Morgagni hernia resulting in delayed growth in a 10-month-old boy. The presentation was unique due to its bilateral nature and its symptomatic compression of the mediastinum. Diagnosis was made by 3D reconstructed CT angiogram. The patient underwent medical optimization until he was safely able to tolerate laparoscopic surgical repair of his hernia. Upon laparoscopy, the CT findings were confirmed and the hernia was repaired.

  18. RARE PRESENTATION OF SYMPTOMATIC BILATERAL PROXIMAL POPLITEAL ARTERY ANEURYSM

    Directory of Open Access Journals (Sweden)

    Thulasikumar Ganapathy

    2016-07-01

    Full Text Available True Popliteal artery aneurysm is the most common of all the peripheral artery aneurysms. We present a case of proximal popliteal artery aneurysm involvement both lower limb presented with gangrene in one lower limb and incapacitating claudication pain on the other lower limb. We have successfully repaired both sides aneurysm in the same sitting with Poly Tetra Fluro Ethylene (PTFE graft, as the patient also had multiple venous perforators’ involvement on both sides, which left us only with synthetic graft repair option rather than venous graft repair.

  19. Nephrogenic Diabetes Insipidus: A Rare Presentation in Multiple Myeloma

    Directory of Open Access Journals (Sweden)

    Mehdi Dehghani

    2012-04-01

    Full Text Available We report a case of multiple myeloma that presented with anorexia, fatigue, high erythrocyte sedimentation rate, bone marrow plasmacytosis of more than 30%, polyuria,and low urine specific gravity. This unusual presentation was diagnosed as nephrogenic diabetes insipidus secondary to a proximal tubular dysfunction. The tubular functional disturbance appeared to be related to thepresence of lambda-type light chains. The patient was treated withdesmopressine without response. After one month of treatment with thalidomide and dexamethasone for myeloma there was a dramatic response with decreased urine output.

  20. Rare presentations of hyperthyroidism--Basedow's paraplegia and pancytopenia.

    Science.gov (United States)

    Chen, Yi-Hsien; Lin, Hung-Jung; Chen, Kuo-Tai

    2009-02-01

    Typical presentations of hyperthyroidism are palpitation, nervousness, tremor, malaise, and weight loss. Hyperthyroidism affects nearly every system in the body, and some patients may manifest neurologic or hematologic symptoms. Atypical presentations of hyperthyroidism often pose a great challenge in diagnosis and treatment. We report a case of Basedow's paraplegia and pancytopenia that was precipitated by hyperthyroidism. The unusual manifestations led to unnecessary examinations and delayed the treatment of hyperthyroidism. The classical symptoms of Basedow's paraplegia are subacute symmetric weakness of the lower extremities with areflexia and sparing sensation or sphincter involvement. Control of the hyperthyroidism mitigated the neurologic and hematologic complications and prevented unnecessary studies.

  1. Rare manifestation of echinococcus cysticus presented in CT and MRI

    International Nuclear Information System (INIS)

    Wrazidlo, W.; Rieber, A.; Schneider, S.; Brambs, H.J.; Friedel, P.; Heidelberg Univ.

    1989-01-01

    A case of primary spinal echinococcus with bone destruction is presented and the findings in CT and MR imaging are discussed. Although CT and MRI are complementary studies for the diagnosis of the spinal echinococcosis, MRI is the study of choice for prolonged follow-up of complicated cases. (orig.) [de

  2. Primary Breast Tuberculosis Presenting as a Lump: A Rare Modern ...

    African Journals Online (AJOL)

    mammary area. A 25. year.old female, presented with a lump in the breast and infra.mammary area. She was having off and on fever without any other complaints. There was no positive family history. Primary breast tuberculosis was diagnosed on fine ...

  3. Hypertrophic pyloric stenosis, an unusual presentation and rare ...

    African Journals Online (AJOL)

    The symptoms were present since birth with nonprojectile, nonbilious vomiting and gastric distension. A contrast study showed a gastric outlet obstruction with the possibility of gastric volvulus. Emergency surgery established the diagnosis. To the best of our knowledge, the association of HPS with diaphragmatic defect and ...

  4. Primary Breast Tuberculosis Presenting as a Lump: A Rare Modern ...

    African Journals Online (AJOL)

    A 25‑year‑old female presented with a painless lump in her right breast for 1 month duration. She gave history of low grade fever off and on for the last 2 weeks. There were no other complaints like weight loss, loss of appetite, and any cough. She was unmarried and there was no positive family history of breast tuberculosis.

  5. Unusual presentation of rare primary lymphoma of bone ...

    African Journals Online (AJOL)

    Primary bone lymphoma is a distinct disease. It represents only 3% of all malignant bone tumours and less than 1% of non-Hodgkin's lymphoma. It is essential to differentiate it from other tumours because of its good prognosis. We report a case of 45 years old male who presented one year ago with a painful left arm.

  6. Polycythemia vera presenting with bilateral papilledema: A rare case report

    Directory of Open Access Journals (Sweden)

    Parija S

    2008-01-01

    Full Text Available A 45-year-old male patient presented with gradual onset of headache, vomiting and blurring of vision of 28 days duration. Ophthalmological examination revealed normal anterior segment and pupillary reflex. No abnormality was detected in the vitreous. Optic disc showed features of advanced papilledema with normal macula and retinal periphery in both eyes. Visual acuity was 20/200 in the right eye and counting fingers close range in the left eye. Non-contrast computed tomography of brain was normal and magnetic resonance imaging showed sagittal sinus thrombosis without any evidence of venous infarction or intracranial mass. Routine hematological investigations revealed increased hemoglobin level, packed cell volume and leucocytosis. Further investigation revealed increased Vitamin B12 and decreased serum erythropoietin. A diagnosis of polycythemia vera was made from the above findings. This case is being presented for the rarity of association of polycythemia vera with bilateral advanced papilledema due to sagittal sinus thrombosis.

  7. Recurrent ovary cancer presenting with scleroderma - A rare case report

    OpenAIRE

    Sargin, Betul; Gurer, Gulcan; Bozbas, Gulnur; Noyan, Fatih; Barut, Kayra; Tataroglu, Canten

    2017-01-01

    Scleroderma is a chronic autoimmune multisystem disorder which is characterizedby progressive fibrosis of the skin and internal organs. Ovary cancers with sclerodermahave been reported in the literature. But recurrent ovary cancer with sclerodermahas not been reported before. Here, we report a 65 -year old female patient presentingwith recurrent ovary cancer and subsequently diagnosed with scleroderma. Due toliterature sources, this is the first case of presenting with recurrent ovary cancera...

  8. A Rare Disorder with Common Clinical Presentation: Neonatal Bartter Syndrome.

    Science.gov (United States)

    Hussain, Shabbir; Tarar, Saba Haider; Al-Muhaizae, Muhammad

    2015-04-01

    Bartter syndrome is an autosomal recessive renal tubulopathy that presents with hypokalemic, hypochloremic metabolic alkalosis associated with increased urinary loss of sodium, potassium, calcium and chloride. There is hyperreninemia and hyperaldosteronemia but normotension. A full term male neonate was referred at 20-day of age with features of sepsis and respiratory distress. He was evaluated and managed as case of septicemia with all supportive paraphernalia including mechanical ventilation. Investigations revealed electrolytes imbalance and metabolic alkalosis suggestive of Neonatal Bartter Syndrome (NBS). Raised aldosterone and renin levels confirmed the diagnosis. Electrolyte imbalance was corrected with fluids and indomethacin, treated successfully, discharged and parents counseled. He was thriving well at 9 months of age. Another 2 months old male baby presented with recurrent episodes of lethargy with dehydration and failure to gain weight. Investigations confirmed the diagnosis of NBS. He was also successfully treated with same medication. We report these 2 cases because of the rarity of NBS, presentation of which may mimic common illnesses like sepsis and gastroenteritis.

  9. Giant Sigmoid Diverticulum: A Rare Presentation of a Common Pathology

    Directory of Open Access Journals (Sweden)

    A. Guarnieri

    2009-02-01

    Full Text Available Although colonic diverticulum is a common disease, affecting about 35% of patients above the age of 60, giant sigmoid diverticulum is an uncommon variant of which only relatively few cases have been described in the literature. We report on our experience with a patient affected by giant sigmoid diverticulum who was treated with diverticulectomy. Resection of the diverticulum is a safe surgical procedure, provided that the colon section close to the lesion presents no sign of flogosis or diverticula; in addition, recurrences are not reported after 6-year follow-up.

  10. Intracranial hypertension: A rare presentation of lupus nephritis.

    Science.gov (United States)

    Yadav, Praveen; Nair, Anishkumar; Cherian, Ajith; Sibi, N S; Kumar, Ashwini

    2010-07-01

    A 14-year-old male presented with bilateral papilledema, growth retardation and absent secondary sexual characters. He had a past history of fever, headache and fatigue of 6 months duration. The diagnosis of intracranial hypertension (IH) was confirmed by an increased intracranial pressure and normal neuroimaging studies of the brain, except for partial empty sella, prominent perioptic cerebrospinal fluid (CSF) spaces and buckling of optic nerves. Evaluation showed erythrocyte sedimentation rate (ESR) of 150 mm/hr, positive antinuclear antibody (ANA), anti dsDNA and anti ribosomal P protein. Renal biopsy revealed diffuse segmental proliferative lupus nephritis (LN) class IV S (A) confirming the diagnosis of systemic lupus erythematosus (SLE). Treatment of LN with intravenous pulse methyl prednisolone and cyclophosphamide was effective in normalizing the CSF pressure, resulting in express and dramatic resolution of symptomatology. In a case of IH, SLE must be considered. IH, growth retardation and absence of sexual characters may be presenting manifestations of a chronic systemic inflammatory disease like SLE. These manifestations may act as a pointer to associated advanced grades of LN, which can be totally asymptomatic and missed without a renal biopsy.

  11. CENTRAL GIANT CELL GRANULOMA OF THE MANDIBLE: A RARE PRESENTATION

    Directory of Open Access Journals (Sweden)

    Virendra SINGH

    2012-06-01

    Full Text Available Central giant cell granuloma (CGCG is an intra-osseous lesion consisting of cellular fibrosis tissue containing multiple foci of hemorrhage, multinucleated giant cells and trabecules of woven bone. This lesion accounts for less than 7% of all benign jaw tumours. Jaffe considered it as a locally reparative reaction of bone, which can be possibly due to either an inflammatory response, hemorrhage or local trauma. Females are affected more frequently than males. It occurs over a wide age range.It has been reported that this lesion is diagnosed during the first two decades of life in approximately 48% of cases, and 60% of cases are evident before the age of 30. It is considerably more common in the mandible than in the maxilla. Most lesions occur in the molar and premolar area, some of these extending up to the ascending ramus. The presence of giant cell granuloma in the mandibular body area, the entire ramus, condyle and coronoid represents a therapeutic challenge for the oral and maxillofacial surgeons. The aim of this report is to describe an unusual presentation of central giant cell granuloma involving the mandibular body, ramus, condylar and coronoid processes, and to discuss the differentiated diagnosis, the radiographic presentation and the management of this lesion.

  12. Basaloid squamous carcinoma of skin associated with xeroderma pigmentosum in an 8-year-old child: A rare entity

    Directory of Open Access Journals (Sweden)

    Tashnin Rahman

    2014-01-01

    Full Text Available Xeroderma pigmentosum (XP is a rare autosomal recessive genodermatosis associated with hypersensitivity to ultraviolet (UV light, due to defects in deoxyribonucleic acid (DNA repair. Basaloid squamous cell carcinoma is a rare aggressive variant of squamous cell carcinoma. Patients with XP are at increased risk of developing cutaneous malignancy and are commonly associated with squamous carcinoma. We report an extremely rare case of 8-year-old child with XP along with basaloidsquamous carcinoma of skin; and review of literature related to it.

  13. Breast abscess as the initial manifestation of primary pure squamous cell carcinoma: a rare presentation and literature review.

    Science.gov (United States)

    Salemis, Nikolaos S

    2011-01-01

    Primary squamous cell carcinoma of the breast is a very rare tumor accounting for less than 0.4% of all breast cancers. Fewer than 100 cases have been reported in the literature so far. The diagnosis requires strict pathologic criteria to be fulfilled. Due to the rarity of this tumor the optimal treatment and prognosis are both unclear. Breast abscess as the initial presentation of a primary squamous cell breast carcinoma is an extremely rare clinical entity. In this study, we describe a case of a 61-year-old postmenopausal woman who presented with typical manifestations of a breast abscess and was diagnosed with a pure primary squamous cell breast carcinoma. Diagnostic evaluation and management of the patient are discussed along with a review of the literature. Despite its rarity, the possibility of a primary pure squamous cell breast carcinoma should always be considered in the differential diagnosis in postmenopausal patients presenting with manifestations of a breast abscess, especially in those who respond poorly to the initial treatment. Physicians should be aware of this rare malignancy in order to avoid delays in diagnosis and treatment.

  14. Holt-Oram Syndrome in Adult Presenting with Heart Failure: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Rupesh Kumar

    2014-01-01

    Full Text Available Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.

  15. Lower urinary tract symptoms and prostatic calculi: A rare presentation of alkaptonuria

    Directory of Open Access Journals (Sweden)

    F K Sridhar

    2012-01-01

    Full Text Available Alkaptonuria is a rare tyrosine metabolic disorder. A deficiency of homogentisic acid oxidase leads to accumulation of homogentisic acid in the body. Dark-colored urine, cutaneous pigmentations and musculoskeletal deformities are characteristic features. Storage and voiding lower urinary tract symptoms due to prostatic calculi is a rare presentation.

  16. Intestinal Duplication Cyst presenting as Volvulus: A rare case report with Review of Literatures

    Directory of Open Access Journals (Sweden)

    Prasan Kumar Hota

    2015-03-01

    Full Text Available Intestinal duplication cyst is a rare congenital anomaly occurring any where along the alimentary tract, from mouth to anus. They can present with numerous complications like perforation, bleeding or intestinal obstruction. We report a rare case  of intestinal duplication cyst of ileum with acute intestinal obstruction due to volvulus with review of literature.

  17. Pigmented epidermal cyst with dense collection of melanin: A rare entity - Report of a case with review of the literature.

    Science.gov (United States)

    Jayalakshmy, P S; Subitha, K; Priya, P V; Johnson, Gerald

    2012-05-01

    Epidermal cyst is a very common benign cystic lesion of the skin. It is usual to find ulceration of the lining epithelium, rupture of the cyst wall with chronic inflammation and foreign body giant cell reaction. But, it is very rare to see an epidermal cyst with marked accumulation of melanin pigment. Only a few cases of pigmented epidermal cyst with dense collection of melanin pigment have been published in the literature. Here, we are reporting a case of ruptured epidermal cyst with keratin granuloma formation and showing dense collection of melanin pigment.

  18. Congenital agenesis of unilateral parotid gland with ipsilateral type I first branchial cleft anomaly: A rare presentation

    OpenAIRE

    Tripti Maithani; Apoorva Pandey; Seema Acharya

    2014-01-01

    Aim: To report a rare case of unilateral parotid agenesis with ipsilateral type I first branchial cleft anomaly. Material and methods: A case study with special emphasis on the embryology, outlining the complex developmental process of parotid and branchial arches and highlighting the probable reason for development of such anomalies. Results: The literature states that unilateral parotid agenesis is a rare entity with few reported cases occurring solely or in conjunction with other hea...

  19. A rare presentation of extra nodal rosai-dorfman disease (case report

    Directory of Open Access Journals (Sweden)

    Abbas Al-Ramzi

    2017-06-01

    It is a very rare condition that Rosai-Dorfman disease might shoe a temporoparietal area involvement can be seen from a review of all literature. The presentation of the disease, differential diagnosis and treatment were discussed.

  20. Spontaneous Perforation of Common Bile Duct: A Rare Presentation of Gall Stones Disease

    Directory of Open Access Journals (Sweden)

    Duminda Subasinghe

    2016-01-01

    Full Text Available Background. Spontaneous perforation of the extrahepatic biliary system is a rare presentation of gall stones. Very few cases of bile duct perforation have been reported in adults. It is rarely suspected or correctly diagnosed preoperatively. Case Presentation. A 66-year-old female presented at the surgical emergency with 3 days’ history of severe upper abdominal pain with distension and repeated episodes of vomiting, as she had evidence of generalized peritonitis and underwent an exploratory laparotomy. A single 0.5 cm × 0.5 cm free perforation was present on the anterolateral surface of the common bile duct at the junction of cystic duct. A cholecystectomy and the CBD exploration were performed. Conclusion. Spontaneous perforation of the extrahepatic bile duct is a rare but important presentation of gall stones in adults. Therefore, awareness of the clinical presentation, expert ultrasound examination, and surgery are important aspects in the management.

  1. Pancoast syndrome: A rare presentation of non-Hodgkin′s lymphoma

    Directory of Open Access Journals (Sweden)

    Anirban Sarkar

    2013-01-01

    Full Text Available Pancoast syndrome is a common presentation of bronchogenic carcinoma, but other malignancies are rarely cited as its cause. Pancoast syndrome due to non-Hodgkin′s lymphoma is rarely described in the literature. Here, we report a case of Pancoast syndrome due to non-Hodgkin′s lymphoma to increase the awareness of the clinicians regarding essentiality of tissue diagnosis of Pancoast tumor before starting the treatment.

  2. Primary Peritoneal Hydatid Cyst Presenting as Ovarian Cyst Torsion: A Rare Case Report

    OpenAIRE

    Gandhiraman, Kavitha; Balakrishnan, Renukadevi; Ramamoorthy, Rathna; Rajeshwari, Raja

    2015-01-01

    Hydatid cyst disease is a zoonotic disease caused by Echinococcus granulosus, E.multilocularis or E.Vogli. The most common primary site is liver (75%) followed by lungs (5-15%) and other organs constitute 10-20%. Peritoneal hydatid cysts are very rare especially primary peritoneal hydatid. Secondary peritoneal hydatid cysts are relatively common, which usually occurs due to rupture of primary hepatic hydatid cyst. We present a rare case of large primary peritoneal hydatid cyst misdiagnosed as...

  3. Congenital tracheoesophageal fistula: A rare and late presentation in adult patient

    Directory of Open Access Journals (Sweden)

    Waseem M Hajjar

    2012-01-01

    Full Text Available Congenital H-type tracheoesophageal fistula (TEF in adults is a rare presentation and can test the diagnostic acumen of a surgeon, endoscopist, and the radiologist. These undetected fistulas may present as chronic lung disease of unknown origin because repeated aspirations can lead to recurrent lung infections and bronchiectasis. Congenital TEFs should be considered in the diagnosis of infants and young adults with recurrent respiratory distress and/or infections. Here, we present the successful management of this rare case in an adult patient.

  4. Iris abscess a rare presentation of intravenous drug abuse associated Candida endophthalmitis

    Directory of Open Access Journals (Sweden)

    Jonathan Pierce

    2016-12-01

    Conclusions and importance: An iris abscess is a rare clinical presentation of intravenous drug use-associated endogenous endophthalmitis and as a result may present a diagnostic challenge as it requires a high level of clinical suspicion and a detailed social history to elicit the drug abuse. Early diagnosis and aggressive therapy is the key to better visual outcomes in these patients.

  5. Calcifying epithelial odontogenic tumor, a rare presentation in children: Two case reports

    Directory of Open Access Journals (Sweden)

    Susant Mohanty

    2014-01-01

    Full Text Available Calcifying epithelial odontogenic tumor (CEOT is a rare and benign odontogenic neoplasm that affects the jaws. It is certainly an atypical instance to find this tumor in children. Here, we present two case reports of CEOT presenting in mandible of a 12- and 13-year-old female child, respectively. CEOT have been reported to show features of malignant transformation also.

  6. An Unusual Site of Adenomatoid Odontogenic Tumor Presenting as Periapical (Radicular Cyst: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    C Anand Kumar

    2010-01-01

    Here we are presenting a rare case report of an unusual site of extrafollicular adenomatoid odontogenic tumor in the mandible w.r.t 32, 33, 34 and 35 mimicking periapical disease clinical and radiographically. However, diagnosis of adenomatoid odontogenic tumor should be considered when the clinician is presented with a corticated radiolucency in the anterior lower jaw, especially in teens and young adults.

  7. Case of a rare type of non-neoplastic mucinous pancreatic cyst – likely new pathological entity?

    International Nuclear Information System (INIS)

    Hilendarov, A.; Nedeva, M.; Belovejdov, V.; Aleksieva, D.; Sirakov, N.

    2013-01-01

    Full text:Introduction: The cystic lesions of the pancreas consists of a range of pathologies which may be broadly divided into neoplastic, non- neoplastic and cysts. Recently a new non-neoplastic cystic lesions, called mucinous non-neoplastic cysts, have been described. Materials and Methods: The imaging methods (ultrasound and CT ) were used as well as invasive imaging methods under image control with a view of the histological verification of the diagnosis. A case of pancreatic cystic lesion is described, accidentally detected by ultrasound and CT scan made for different purpose. Results : The finding was a 28/32 mm cyst in the body of the pancreas, apparently communicating with the pancreatic duct . The Endoscopic Retrograde Cholangiopancreatography and laboratory tests of liver function, serum CEA and carbohydrate antigen C19 -9 were within normal limits. After the distal pancreatectomy and splenectomy the lasting histological specimen showed a simple cyst, lined with mucinous epithelium. Conclusion: The presented case guides the imaging diagnosticians and surgeons towards seeking a thorough preoperative clarification of pancreatic cystic lesions. It is recommended that patients diagnosed with 'benign' mucinous neoplasm are closely monitored due to the inability to completely confirm the benign nature of the lesion

  8. Causes of chronic non-infectious diarrhoea in infants less than 6 months of age: rarely recognized entities

    International Nuclear Information System (INIS)

    Mushtaq, I.; Cheema, H.A.; Malik, H.S.; Waheed, N.; Hashmi, M.A.

    2017-01-01

    Non-infectious causes of chronic diarrhoea are important and easily missed. The study was done with the objectives to identify different causes of chronic non-infectious diarrhoea in infants less than 6 months of age. Methods: All patients less than 6 months of age presenting for the first time to a Paediatric Gastroenterology tertiary care centre with a history of chronic diarrhoea and negative stool cultures, were enrolled over a period of 8 months. Demographical profile and various factors under observation were recorded in this observational study. Collected data was analysed using SPSS version 20. Chi square test was applied as a test of significance for any qualitative variable, p value (p<0.05) was taken as significant. Results: Among 72 enrolled patients, female to male ratio was 1.05:1. Age at onset of symptoms was between 15 days to 06 months. Aetiology found was Cow's milk protein allergy (CMPA) in 58 (80.6%), Primary intestinal lymphangiectasia (PIL) 6 (8.3%), Cystic fibrosis (CF) 3 (4.2%), Immunodeficiency (SCID) 2 (2.8%), 1 (1.4%) for each Abetalipoproteinemia (ABL), Glucose galactose malabsorption (GGM) and Congenital chloride diarrhoea (CCD). Conclusions: Among non-infectious causes of chronic diarrhoea in early infancy, cow's milk protein allergy is most common followed by Primary intestinal lymphangiectasia and Cystic fibrosis. (author)

  9. Male breast cancer is rare: an initial presentation may be as an abscess

    OpenAIRE

    Ventham, N T; Hussien, M I

    2010-01-01

    Breast cancer in men is rare. Breast cancer presenting initially as an abscess has been described only a handful of times in the literature. We present the first described case of invasive adenocarcinoma presenting as an abscess in a man. An 80-year-old diabetic man presented with symptoms typical of a breast abscess. The abscess failed to respond to percutaneous therapy and excision of breast abscess was performed. Histology revealed an invasive carcinoma. He went on to have a mastectomy. Hi...

  10. Jejunal Perforation: A Rare Presentation of Burkitt’s Lymphoma—Successful Management

    Directory of Open Access Journals (Sweden)

    Samir Ranjan Nayak

    2014-01-01

    Full Text Available Malignant tumors of the small bowel presenting as acute abdomen are a rare occurrence. Burkitt’s lymphoma presenting as a surgical emergency needing emergency laparotomy is an uncommon presentation of this tumor. We present an interesting case of jejunal perforation as a first manifestation of Burkitt’s lymphoma which was successfully managed with surgical resection, high dose chemotherapy, and good supportive care.

  11. Erythema multiforme like allergic contact dermatitis associated with laurel oil: a rare presentation.

    Science.gov (United States)

    Uzuncakmak, Tugba Kevser; Karadag, Ayse Serap; Izol, Belcin; Akdeniz, Necmettin; Cobanoglu, Bengu; Taskin, Secil

    2015-04-16

    Allergic contact dermatitis is a common skin disease, which affects approximately 20% of the population. This reaction may present with several clinical manifestations. Erythema multiforme-like allergic contact dermatitis is a rare type of non-eczematous contact dermatitis, which may lead to difficulty in diagnosis.Essential oil of Laurus nobilis is widely used in massage therapy for antiinflammatory and analgesic effects. Laurus nobilis induced contact dermatitis has been reported in the literature but an erythema multiforme-like presentation is rare.

  12. A rare nonsyndromic presentation of bilateral doughnut shaped lip pits in an Indian child

    Directory of Open Access Journals (Sweden)

    Senthil Balasubramani

    2016-01-01

    Full Text Available Lip pits are a rare congenital anomaly that presents on the upper or lower lip or the commissure of the lips. Lip pits are an autosomal dominant trait occurring almost always in association with cleft lip or palate. They most commonly occur in association with developmental disturbances such as Van der Woude's syndrome, popliteal pterygium syndrome, oro-facial-digital syndrome, Marres-Cremers syndrome, and Hirschsprung disease. Its occurrence in nonsyndromic individuals is extremely rare with only a handful of cases reported. The identification of lip pits with other associated anomalies is crucial for genetic counseling; we report a case of nonsyndromic presentation of bilateral lip pits.

  13. Adenoid Cystic Carcinoma of Nasal Cavity in an Adolescent Male- A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Harshi Dhingra

    2018-04-01

    Full Text Available Adenoid Cystic Carcinoma (ACC is uncommonly found outside the major or minor salivary glands and is especially rare when located in nasal cavity. A case report of 13 year old boy who presented with mass in nasal cavity and epistaxis is presented here. Endoscopic removal of mass was done and histopathology revealed ACC. ACCs are slow growing tumours with a propensity for frequent local recurrence and early perineural and haematogenous spread. Early diagnosis requires a high index of suspicion for this rare pathology.

  14. Guyon's canal syndrome due to tortuous ulnar artery with DeQuervain stenosing tenosynovitis, ligamentous injuries and dorsal intercalated segmental instability syndrome, a rare presentation: a case report.

    Science.gov (United States)

    Zeeshan, Muhammad; Ahmed, Farhan; Kanwal, Darakhshan; Khalid, Qazi Saad Bin; Ahmed, Muhammad Nadeem

    2009-12-23

    The Guyon's canal syndrome is a well known clinical entity and may have significant impact on patient's quality of life. We report a case of 43-year-old male who presented with complaints of pain and numbness in right hand and difficulty in writing for past one month. On imaging diagnosis of Guyon's canal syndrome because of tortuous ulnar artery was made with additional findings of DeQuervain's stenosing tenosynovitis and dorsal intercalated segmental instability syndrome with ligamentous injury and subsequently these were confirmed on surgery.Although it is a rare syndrome, early diagnosis and treatment prevents permanent neurological deficits and improve patient's quality of life.

  15. Rasmussen's Aneurysm: A Forgotten Entity?

    International Nuclear Information System (INIS)

    Keeling, A. N.; Costello, R.; Lee, M. J.

    2008-01-01

    We present the case of a rare entity which is a complication of a disease process that had almost disappeared from the Western World. With the recent resurgence in reported cases of Mycobacterium tuberculosis (TB) in Western communities, it is important to recognize complications and sequelae. A young alcoholic male with confirmed active TB suffered a cardiac arrest following massive haemoptysis. Multidetector computed tomography angiography diagnosed a Rasmussen's aneurysm, confirmed by digital subtraction angiography and then successfully embolized with glue. We outline this rare case and the embolization technique and review previously documented reports

  16. Rare Presentation of Genitourinary Tuberculosis Masquerading as Renal Cell Carcinoma: A Histopathological Surprise

    Directory of Open Access Journals (Sweden)

    Santosh Kumar

    2014-01-01

    Full Text Available Genitourinary tuberculosis (GUTB is a rare extrapulmonary manifestation of tuberculosis (TB. Various forms of presentation are described and in most cases the disease results in calcification, atrophy, or necrosis of the renal parenchyma. The kidney is not generally palpable except in cases of hydronephrosis due to an upper ureteric stricture. We present a case of GUTB presenting as inflammatory pseudotumor. This case was initially diagnosed as renal malignancy and managed accordingly. Histopathology confirmed the diagnosis of pseudotumoral renal TB.

  17. Unusual clinical presentation of rare case of vaginal leiomyoma: a case report

    OpenAIRE

    Ishrat Zuber; Purnima K. Nadkarni; Aditi A. Nadkarni; Akshay Nadkarni

    2016-01-01

    Primary vaginal leiomyoma are rare and usually arise from anterior vaginal wall, approximately 330 cases of vaginal fibroid reported in world literature. Vaginal myoma usually presented as discharge per vaginum, abnormal bleeding, pain lower abdomen, dyspareunia etc. We report a case of primary vaginal leiomyoma arising from lateral vaginal wall which is presented clinically as pain in hip joint and radiating to ipsilateral leg which is unusual clinical presentation creating diagnostic dilemm...

  18. Verrucous carcinoma of the hand: a rare presentation evaluated by magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Lopes, Jailson Rodrigues; Rosemberg, Laercio Alberto; Lourenco, Rafael Burgomeister; Cerri, Giovanni Guido [Universidade de Sao Paulo (InRad/HC/USP), SP (Brazil). Hospital das Clinicas. Inst. de Radiologia; Rodrigues, Marcelo Bordalo [Universidade de Sao Paulo (IOT/HC/USP), SP (Brazil). Hospital das Clinicas. Instituto de Ortopedia e Traumatologia

    2011-07-15

    Verrucous carcinoma is a variant of squamous cell carcinoma seen in mucous membranes and skin, and rarely found in the hand. The present report describes a case of two lesions on the dorsum of the hand, with no contact to each other, which underwent en-block resection and were confirmed as verrucous carcinoma. (author)

  19. Verrucous carcinoma of the hand: a rare presentation evaluated by magnetic resonance imaging

    International Nuclear Information System (INIS)

    Lopes, Jailson Rodrigues; Rosemberg, Laercio Alberto; Lourenco, Rafael Burgomeister; Cerri, Giovanni Guido; Rodrigues, Marcelo Bordalo

    2011-01-01

    Verrucous carcinoma is a variant of squamous cell carcinoma seen in mucous membranes and skin, and rarely found in the hand. The present report describes a case of two lesions on the dorsum of the hand, with no contact to each other, which underwent en-block resection and were confirmed as verrucous carcinoma. (author)

  20. Urethral triplication with meatuses terminating on the penis: a rare case presenting with bifid urinary stream

    International Nuclear Information System (INIS)

    Hirselj, Daniel A.; Jayanthi, Venkata R.; Lowe, Gregory K.

    2009-01-01

    Urethral duplication is an uncommon clinical finding, and classification systems have been described to explain the clinical findings. Urethral triplication is an extremely uncommon diagnosis, with few published case reports. We present the rare case of an infant noted to have a bifid urinary stream and found to have urethral triplication on voiding cystourethrography. (orig.)

  1. Urethral triplication with meatuses terminating on the penis: a rare case presenting with bifid urinary stream

    Energy Technology Data Exchange (ETDEWEB)

    Hirselj, Daniel A.; Jayanthi, Venkata R. [Nationwide Children' s Hospital, Department of Pediatric Urology, Columbus, OH (United States); Lowe, Gregory K. [The Ohio State University Medical Center, Department of Urology, Columbus, OH (United States)

    2009-11-15

    Urethral duplication is an uncommon clinical finding, and classification systems have been described to explain the clinical findings. Urethral triplication is an extremely uncommon diagnosis, with few published case reports. We present the rare case of an infant noted to have a bifid urinary stream and found to have urethral triplication on voiding cystourethrography. (orig.)

  2. Acute upper arm ischaemia: a rare presentation of non-Hodgkin's lymphoma.

    LENUS (Irish Health Repository)

    Daruwalla, Z J

    2010-12-01

    Digital ischaemia has been sparsely reported in current literature. Its association with lymphomatous conditions has been described in even more exceptional occurrences. We present the first case of upper arm ischaemia associated with non-Hodgkin\\'s lymphoma. A brief literature review of this rare phenomenon is also accompanied with it.

  3. Spontaneous acute subdural hematoma: A rare presentation of a dural intracranial fistula.

    Science.gov (United States)

    de Aguiar, Guilherme Brasileiro; Veiga, José Carlos Esteves; Silva, João Miguel de Almeida; Conti, Mario Luiz Marques

    2016-03-01

    Dural arteriovenous fistulas are acquired lesions between the meningeal arteries and their associated draining veins. They may have highly variable clinical presentations and evolution, from severe neurological deficit to no or trivial symptoms. Intracranial hemorrhage occurs in less than 24% of all dural fistulas, and the bleeding is usually subarachnoid, more infrequently intracerebral, and rarely in the subdural space. Here, we present a rare case of a patient who presented with a subdural spontaneous hemorrhage. After investigation by cerebral angiography, the diagnosis of a dural arteriovenous fistula was made. The patient underwent uneventful endovascular treatment. As there are with only a few reports in the literature of such a presentation, we present this patient and perform a brief review of the literature. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. A Type-II First Branchial Cleft Anomaly Presenting as a Post-Auricular Salivary Fistula: A Rare Presentation.

    Science.gov (United States)

    Jain, S; Deshmukh, Pt; Gupta, M; Shukla, S

    2014-01-01

    First branchial cleft anomalies are rare with the average age of presentation as 19 years. There is an average delay of 3.5 years between initial presentation and adequate treatment due to diagnostic dilemma. A very rare variant of first branchial cleft anomaly presenting as a post-auricular salivary fistula is reported. A 12-year-old girl presented with a history of intermittent watery discharge, more so at the time of meals from a right post-auricular opening for last 3 years. Computed tomography sialography revealed a fistulous tract connecting the sub segmental duct of the parotid gland extending along the pre-tragus region in subcutaneous plane up to mastoid tip after passing inferior to external auditory canal. Superficial parotidectomy with identification of facial nerve branches was carried out for excision of the tract. Histopathology revealed sinus tract comprising of ectodermal components and acini of the parotid gland. We classified our case into work's type-2 based on anatomical location at an angle of mandible, its relationship to parotid gland and facial nerve and previous history of ear discharge. To the best of our knowledge, this is the first case of its type to be reported.

  5. A Type-II First Branchial Cleft Anomaly Presenting as a Post-Auricular Salivary Fistula: A Rare Presentation

    Science.gov (United States)

    Jain, S; Deshmukh, PT; Gupta, M; Shukla, S

    2014-01-01

    First branchial cleft anomalies are rare with the average age of presentation as 19 years. There is an average delay of 3.5 years between initial presentation and adequate treatment due to diagnostic dilemma. A very rare variant of first branchial cleft anomaly presenting as a post-auricular salivary fistula is reported. A 12-year-old girl presented with a history of intermittent watery discharge, more so at the time of meals from a right post-auricular opening for last 3 years. Computed tomography sialography revealed a fistulous tract connecting the sub segmental duct of the parotid gland extending along the pre-tragus region in subcutaneous plane up to mastoid tip after passing inferior to external auditory canal. Superficial parotidectomy with identification of facial nerve branches was carried out for excision of the tract. Histopathology revealed sinus tract comprising of ectodermal components and acini of the parotid gland. We classified our case into work's type-2 based on anatomical location at an angle of mandible, its relationship to parotid gland and facial nerve and previous history of ear discharge. To the best of our knowledge, this is the first case of its type to be reported. PMID:24669347

  6. Perianal Ulcer and Diabetes Insipidus: A Rare Presentation of Langerhans Cell Histiocytosis in an Adult Male

    Directory of Open Access Journals (Sweden)

    Ng Chong Beng

    2017-07-01

    Full Text Available Langerhans cell histiocytosis (LCH, previously known as Histiocystosis X, is a rare idiopathic disease caused by a build-up of langerhan cells in the body. LCH has a heterogenous form of clinical presentation and presents with varying degree of severity. The severity often depends on the disease burden. LCH can manifest as a unifocal disease, multifocal-unisystem or multifocal-multisystem disease with the latter being more fatal than the rest. Risk organ involvement confers a grim prognosis. Perianal lesions are extremely rare skin manifestation of LCH with only few cases reported in the past. Vinca-alkaloids and steroid therapy have been shown to be effective in a large percentage of those presenting with LCH. This case report highlights the diagnosis and management of LCH presenting as a painful perianal ulcer in the background of newly diagnosed diabetes insipidus.

  7. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

    OpenAIRE

    Konca Degertekin, Ceyla; Özkurt, Zübeyde Nur; Akyürek, Nalan; Yağcı, Münci

    2012-01-01

    Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic m...

  8. A Rare Clinical Presentation of Giant Bilateral Labial Fibroepithelial Stromal Polyps in Patient with Psoriasis Disease

    Directory of Open Access Journals (Sweden)

    Ayse Filiz Avsar

    2016-01-01

    Full Text Available Fibroepithelial polyps (FEPs are rarely seen lesions of the lower female genital tract with polypoid proliferations of stroma. These tumors usually present in the vulvovaginal region of the reproductive aged women. In this presentation, we report a case of a psoriatic woman who developed unusual multiple polypoid lesions approximately 15 cm in size arising from both left and right labia minora and unique connection of FEPs with psoriasis disease.

  9. Macular edema might be a rare presentation of hydroxychloroquine-induced retinal toxicity

    Directory of Open Access Journals (Sweden)

    Chih-Yao Chang

    2017-01-01

    Full Text Available The aim of this study is to report a rare case of macular edema as a presentation of hydroxychloroquine-related retinal toxicity. We presented a case of a 46-year-old female patient using hydroxychloroquine for underlying rheumatoid arthritis (RA with blurred vision over the left eye. Uveitis and macular edema were found initially. Systemic survey did not reveal any other etiology. Topical corticosteroid was given under the impression of RA-related uveitis. The uveitis resolved 1 week later, but macular edema persisted in spite of treatment. Under the suspicion of drug-related complication, we try to discontinue hydroxychloroquine. Her symptoms improved gradually after cessation of hydroxychloroquine, and further serial image study confirmed subsiding of the macular edema without any further treatment. Except the well-known signs of the retinal toxicity, macular edema might be a rare presentation of hydroxychloroquine-related retinal toxicity.

  10. A young diabetic with suicidal risk: Rare disease with a rarer presentation

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    Rajeev Philip

    2013-01-01

    Full Text Available Rare genetic or inherited forms of diabetes can mimic immune mediated type 1 diabetes. Early age of onset and associated features help to differentiate these diseases from type 1 diabetes. Wolfram syndrome, an inherited neuro degenerative disorder, presents as insulin dependent diabetes mellitus, diabetes insipidus, optic atrophy and deafness. But less well described features like psychiatric manifestations can be the presentation of this disease. We present such a case. Wolfram syndrome should be considered as a differential diagnosis in insulin dependent diabetic children who present with neuropsychiatric problems.

  11. Cryptococcoma mimicking a brain tumor in an immunocompetent patient: case report of an extremely rare presentation.

    Science.gov (United States)

    Paiva, Aline Lariessy Campos; Aguiar, Guilherme Brasileiro de; Lovato, Renan Maximilian; Zanetti, Arthus Vilar Deolindo; Panagopoulos, Alexandros Theodoros; Veiga, José Carlos Esteves

    2017-11-06

    Central nervous system (CNS) infectious diseases have high prevalence in developing countries and their proper diagnosis and treatment are very important for public health planning. Cryptococcus neoformans is a fungus that may cause several CNS manifestations, especially in immunocompromised patients. Cryptococcal meningitis is the most common type of involvement. Mass-effect lesions are uncommon: they are described as cryptococcomas and their prevalence is even lower among immunocompetent patients. The aim here was to report an extremely rare case of cryptococcoma causing a mass effect and mimicking a brain tumor in an immunocompetent patient. The literature on CNS cryptococcal infections was reviewed with emphasis on cryptococcomas. Clinical, surgical and radiological data on a female patient with this rare presentation of cryptococcoma mimicking a brain tumor are described. A 54-year-old female patient presented to the emergency department with a rapid-onset progressive history of confusion and completely dependency for basic activities. Neuroimaging showed a left occipital lesion and neurosurgical treatment was proposed. From histopathological evaluation, a diagnosis of cryptococcoma was established. She received clinical support with antifungals, but despite optimal clinical treatment, her condition evolved to death. Cryptococcal infections have several forms of presentation and, in immunocompetent patients, their manifestation may be even more different. Cryptococcoma is an extremely rare presentation in which proper surgical and clinical treatment should be instituted as quickly as possible, but even so, there is a high mortality rate.

  12. A rare and an unusually delayed presentation of orbital actinomycosis following avulsion injury of the scalp

    Directory of Open Access Journals (Sweden)

    Hegde Vidya

    2010-01-01

    Full Text Available We report a rare case of orbital swelling presenting one year after head trauma. An initial fine needle aspiration cytology revealed it to be an infected organizing hematoma. However, broad-spectrum antibiotics did not resolve the infection and the orbital lesion continued to grow in size, as evaluated by magnetic resonance imaging. Incisional biopsies were done, which were reported as orbital actinomycosis. Patient has responded well to treatment with penicillin. This case is of interest due to the delayed presentation of an orbital complication of head trauma and the rare infection with actinomyces. It also highlights the importance of using appropriate antibiotics, as well as the need for long-term treatment.

  13. A rare ultrasound presentation of splenic lesion in a patient with disseminated Penicillium marneffei infection

    International Nuclear Information System (INIS)

    Tan, Rong; Xiao, Ying; Tang, Qi; Zhang, Ying

    2013-01-01

    Focal hypoechoic lesions in the spleen often represent malignant disease in patients with acquired immune deficiency syndrome (AIDS). However, some infection can produce similar images. The abdominal ultrasound imaging about disseminated Penicillium marneffei (P. marneffei) infection has been rarely described in the medical literature. This case report presents a 47-year-old Chinese man with newly diagnosed AIDS who was infected by P. marneffei. An isolated splenic lesion was detected by ultrasound scan before, and assessed following, diagnostic treatment.

  14. Aggressive natural killer-cell leukemia: Classical presentation of a rare disease

    Directory of Open Access Journals (Sweden)

    Priya M Jacob

    2014-01-01

    Full Text Available Aggressive natural killer-cell leukaemia is a rare aggressive form of natural killer-cell neoplasm. We report a case of a 40-year-old male who presented with jaundice, raised blood counts,generalised lymphadenopathy and hepatosplenomegaly. The diagnosis was established by flow cytometric analysis of bone marrow aspirate. The patient, however, succumbed to his illness within 2 weeks of starting chemotherapy. To the best of our knowledge, this is the third reported case from India.

  15. A rare presentation of a child with osteogenesis imperfecta and congenital laryngomalacia for herniotomy

    Directory of Open Access Journals (Sweden)

    Roshith Chandran

    2011-01-01

    Full Text Available Sometimes anaesthesiologists come across rare congenital anomalies in their practice. The inherent complications associated with the disorder necessitate tailor-made approaches for providing anaesthesia to even seemingly simple surgical interventions. Here, we share our experience of anaesthesia management of an infant with congenital laryngomalacia and recently diagnosed osteogenesis imperfecta type 1 who had presented to us with an acute abdomen for a semi-emergency herniotomy.

  16. A rare case of quadrigeminal plate lipoma presenting with the sixth cranial nerve palsy

    Institute of Scientific and Technical Information of China (English)

    Bipin Kumar Chaurasia; Tolga Dundar; Narendra Shalike; Silak Ram Chaudhary; Shamsul Alam; Dhiman Chowdhory; Kanak Kanti Barua; Ranjit Kumar Chaurasiya; Raushan Kumar Chaurasia; Ramesh Kumar Chaurasia

    2017-01-01

    Intracranial lipomas are rare benign tumour that is slow growing, generally asymptomatic, most frequently located in the midline areas and are usually an incidental finding on imaging and therefore cases are not frequently reported. This study reports a case of a patient with quadrigeminal plate lipoma presenting with obstructive hydrocephalous and the 6th cranial nerve palsy that was successfully treated with ventriculo-peritoneal shunting without addressing the lesion.

  17. Primitive neuroectodermal tumor presenting as a presacral mass: A rare case report with review of literature

    Directory of Open Access Journals (Sweden)

    Pradnya S Bhadarge

    2017-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are a group of highly malignant small round cell tumor (SRCT of neuroectodermal origin. They exhibit a great diversity in their clinical manifestations and pathologic similarities with other SRCTs. PNET commonly occurs in the central nervous system, head and neck region, paravertebral region, pelvis, and lower extremities. PNET presenting as a presacral mass is very rare. We present a case of 65-year-old female patient presented with a mass in the abdomen. Exploratory laparotomy with excision of mass was carried out. Histopathology revealed the diagnosis of PNET. The rarity of PNET at presacral region prompted the description of this case.

  18. Presentation of idiopathic retroperitoneal fibrosis at a young age: A rare case report.

    Science.gov (United States)

    Minocha, Priyanka; Setia, Ankur

    2016-11-01

    Abdominal pain is a very common symptom in all age groups but retroperitoneal fibrosis is a rare differential diagnosis suspected in young patients presenting with nonspecific abdominal pain and symptoms of obstructive uropathy. Presented here is a case of a 16-year-old boy who presented with symptoms of persistent abdominal pain and a previous history of swelling in the left leg. A computed tomography (CT) scan suggested retroperitoneal fibrosis and an exploratory laparotomy and histopathological examination were performed for definitive diagnosis. This case report is intended to promote awareness of retroperitoneal fibrosis in young patients among health care providers.

  19. Beta-hemolytic streptococcus group a endocarditis: a rare clinical presentation

    International Nuclear Information System (INIS)

    Alam, A.; Tariq, M.

    2008-01-01

    A case report of an elderly gentleman is reported herein, who presented with one week history of fever, drowsiness and left lower limb weakness. Examination revealed left lower limb weakness with power of grade 3/5. His workup showed evidence of infection and multiple cerebral infarcts on the right side. Blood culture grew Streptococcus pyogens. Echocardiogram showed two vegetations on the aortic valve. Fever was the main presenting feature in this case but it was the presentation of patient with multiple cerebral infarcts that lead to the diagnosis of infective endocarditis. The organism causing Infective Endocarditis (IE) in this patient was a rare one. (author)

  20. Primary Non-Hodgkin's lymphoma of the tongue: A rare presentation

    Directory of Open Access Journals (Sweden)

    Lavanya Karanam

    2016-01-01

    Full Text Available The head and neck is the second most common region for extranodal lymphomas. The most common site is the Waldeyer's ring, and involvement of the base of tongue is extremely rare. We present a rare case of a young female with primary non-Hodgkin's lymphoma (NHL of the base of tongue. A 23-year-old female presented with a history of foreign body sensation in her throat for a month. Oral examination revealed a lobulated smooth mass at the base of tongue. Contrast-enhanced computed tomography neck shows polypoidal homogeneously enhancing soft tissue lesion in the base of tongue extending till the lateral pharyngeal wall. The biopsy of the lesion was reported as NHL. Hodgkin's lymphoma should be kept in the differential diagnosis of swelling arising from the base of tongue. We report a rare and varied presentation of extranodal lymphoma. A careful clinical evaluation supported by histopathological and radiologic investigations will help in identifying the disease at an early stage, resulting in a better prognosis.

  1. Carcinosarcoma of the Ureter with a Small Cell Component: Report of a Rare Pathologic Entity and Potential for Diagnostic Error on Biopsy

    Directory of Open Access Journals (Sweden)

    Kent Newsom

    2014-01-01

    Full Text Available Carcinosarcomas of the ureter are rare biphasic neoplasms, composed of both malignant epithelial (carcinomatous and malignant mesenchymal (sarcomatous components. Carcinosarcomas of the urinary tract are exceedingly rare. We report a unique case of a carcinosarcoma of the ureter with a chondrosarcoma and small cell tumor component arising in a 68-year-old male who presented with microscopic hematuria. CT intravenous pyelogram revealed right-sided hydroureter and hydronephrosis with thickening and narrowing of the right ureter. The patient underwent robot-assisted ureterectomy with bladder cuff excision and subsequent adjuvant chemotherapy. The patient is disease-free at 32 months after treatment. We provide a brief synoptic review of carcinosarcoma of the ureter and bladder with utilization of immunohistochemical (IHC stains and potential diagnostic pitfalls.

  2. Pancreatic Tail Cancer with Sole Manifestation of Left Flank Pain: A Very Rare Presentation

    Directory of Open Access Journals (Sweden)

    Hsing-Lin Lin

    2008-06-01

    Full Text Available Pancreatic cancer is sometimes called a “silent disease” because it often causes no symptoms in the early stage. The symptoms can be quite vague and various depending on the location of cancer in the pancreas. The anatomic site distribution is 78% in the head of the pancreas, 11% in the body, and 11% in the tail. Pancreatic cancer is rarely detected in the early stage, and it is very uncommon to diagnose pancreatic tail cancer during an emergency department visit. The manifestation of pancreatic tail cancer as left flank pain is very rare and has seldom been identified in the literature. We present a case of pancreatic tail cancer with the sole manifestation of dull left flank pain. Having negative findings on an ultrasound study initially, this female patient was misdiagnosed as having possible acute gastritis, urolithiasis or muscle strain after she received gastroendoscopy and colonofiberscopy. Her symptoms persisted for several months and she visited our emergency department due to an acute exacerbation of a persistent dull pain in the left flank area. Radiographic evaluation with computed tomography was performed, and pancreatic tail tumor with multiple metastases was found unexpectedly. We review the literature and discuss this rare presentation of pancreatic tail cancer.

  3. Chlorpyrifos-Induced Delayed Neurotoxicity with A Rare Presentation of Flaccid Quadriplegia: A Diagnostic Challenge

    Directory of Open Access Journals (Sweden)

    Patrícia Afonso Mendes

    2017-10-01

    Full Text Available Chlorpyrifos is an organophosphate compound recognized as causing acute toxicity. However, organophosphate-induced delayed polyneuropathy (OPIDP, although rare, has also been described. We describe an unusual presentation of OPIDP with flaccid quadriplegia progressing to a locked-in-like syndrome, 30 days after a 60-year-old man voluntarily ingested chlorpyrifos. In the absence of specific treatment, the patient only recovered partial motor responses and the ability to communicate. The authors present this report in order to highlight a form of OPIDP which can hinder diagnosis due to its atypia and the delay in the onset of symptoms from initial contact with the toxicant.

  4. BILATERAL ANTERIOR DISLOCATION OF SHOULDER WITH GREATER TUBEROSITY FRACTURE DUE TO HYPONATREMIA : A RARE PRESENTATION

    Directory of Open Access Journals (Sweden)

    Sivananda

    2015-01-01

    Full Text Available We here report a rare presentation of bilateral anterior dislocation of shoulder with associated fracture of greater tuberosity in a 38 year old male due to minor trauma which he sustained secondary to hyponatremia induced irritability. There was no associ ated rotator cuff tear which is often associated with BADS which makes this presentation unique. Unilateral dislocation of shoulder is a common condition which is frequently encountered in emergency trauma department. Anterior dislocation is more common th an posterior dislocation. However, simultaneous bilateral shoulder dislocations are usually posterior. Bilateral anterior dislocations with fractures of the greater tuberosity are even rarer and are usually associated with trauma or seizures

  5. Rare Presentation of Prostate Cancer Mimicking Malignant Lymphoma with Generalized Lymphadenopathy

    Directory of Open Access Journals (Sweden)

    Yu-Fen Tsai

    2014-06-01

    Full Text Available Prostate cancer typically metastasizes to bones and regional lymph nodes. Generalized lymphadenopathy is a rare manifestation of metastatic prostate cancer. We report a case of prostate cancer in a 65-year-old male with initial presentation of generalized lymphadenopathy and no urinary symptoms. Lymph node biopsy revealed metastatic adenocarcinoma, and immunohistochemical staining was positive for prostate-specific antigen (PSA compatible with a prostatic origin. Directed biopsy confirmed that the tumor originated in the prostate. Therefore, the prostate should be considered a possible origin of metastatic adenocarcinoma in men, and presentations consistent with generalized lymphadenopathy cannot exclude a diagnosis of prostate cancer.

  6. A rare benign renal tumour presenting as polycythaemia in a teenage girl.

    LENUS (Irish Health Repository)

    Geoghegan, S

    2010-04-01

    We present the case of a 15-year-old girl who presented with polycythemia. CT abdomen revealed an enhancing mass in the upper pole of her left kidney with features suggestive of renal cell carcinoma. She underwent a laparoscopic radical nephrectomy. Histology demonstrated a well circumscribed, focally encapsulated, round blue cell tumour showing areas of microcalcifications and numerous psammoma bodies. Imunostaining showed diffuse positive staining for CD 57. This was consistent with a diagnosis of metanephric adenoma a rare benign epithelial renal tumour.

  7. Bell’s Palsy As a Rare First Presentation of Breast Cancer

    Directory of Open Access Journals (Sweden)

    Mostafa Hosseini

    2016-06-01

    Full Text Available Background: Otalgia and Bell’s palsy are rare manifestations of metastasis and the most common presentation of an inflammatory process in the temporal bone.Case presentation: This article explains a 34-year-old woman with breast cancer who presented with cranial nerve palsy symptoms. The 7th and 8th cranial nerves were involved in the metastatic phase and then hoarseness was added to her symptoms. Brain MRI showed a petrous lesion in the temporal bone due to metastasis, which was the first clue to cancer. Her metastatic workup showed multiple bone lesions. On chest CT scan, multiple lung lesions were noted. Also, a breast mass was discovered on her chest CT scan. On breast examination an irregular mass fixed to the pectoralis muscle was found. Pathologic evaluation of samples obtained through ultrasound-guided core needle biopsy confirmed the diagnosis of invasive ductal carcinoma.Conclusion: Temporal bone metastases are rare and may be asymptomatic, or with mild symptoms mimicking mastoid infections. Physicians should consider metastatic cancer on the list of differential diagnoses in patients presenting with prolonged otologic symptoms or facial nerve disorders.

  8. Male breast cancer is rare: an initial presentation may be as an abscess.

    Science.gov (United States)

    Ventham, N T; Hussien, M I

    2010-08-06

    Breast cancer in men is rare. Breast cancer presenting initially as an abscess has been described only a handful of times in the literature. We present the first described case of invasive adenocarcinoma presenting as an abscess in a man. An 80-year-old diabetic man presented with symptoms typical of a breast abscess. The abscess failed to respond to percutaneous therapy and excision of breast abscess was performed. Histology revealed an invasive carcinoma. He went on to have a mastectomy. Histology should be obtained from breast abscesses not resolving within 2 months of initial percutaneous therapy. Histology could be obtained by ultrasound-guided fine-needle aspiration (FNA), core or vacuum assisted biopsy, or by formal incision and drainage.

  9. Reversible thyrotroph hyperplasia with hyperprolactinemia: A rare presenting manifestation of primary hypothyroidism

    Directory of Open Access Journals (Sweden)

    Rajesh Rajput

    2012-01-01

    Full Text Available Pituitary thyrotroph hyperplasia with hyperprolactinemia has been described as a rare presentation of primary hypothyroidism. Premenopausal females with this disorder can present with features of hypothyroidism, menstrual disturbances, galactorrhea, and visual field defects because of enlarged pituitary. Here we describe a 32-year-old female presenting to her gynecologist primarily with galactorrhea and secondary amenorrhea. She was found to have raised serum prolactin, and MRI brain showed enlarged pituitary. She was referred for pituitary surgery when she came to us. Clinical examination and biochemistry were suggestive of primary hypothyroidism. She was prescribed levothyroxine replacement. At 6 weeks follow-up, serum prolactin came down to normal, galactorrhea subsided, and spontaneous menstrual cycles resumed. In 12 weeks, pituitary enlargement completely regressed and in another month after that, she conceived. Hence, primary hypothyroidism can present with thyrotroph hyperplasia, where correct diagnosis and levothyroxine therapy can prevent unnecessary pituitary surgery. Hyperprolactinemia in this setting is of no clinical significance.

  10. Arterial tortuosity syndrome: An extremely rare disease presenting as a mimic of pulmonary sling

    Directory of Open Access Journals (Sweden)

    Amy Farkas, MD

    2018-02-01

    Full Text Available Pulmonary sling is the anatomic variant defined by the aberrant origin of the left pulmonary artery from the right pulmonary artery. This patient presented with a mimic of pulmonary sling as a result of an extremely rare condition, arterial tortuosity syndrome (ATS. The patient was first diagnosed with pulmonary sling on prenatal echocardiogram performed by cardiology. Computed tomography angiography of the chest obtained at birth to evaluate respiratory depression demonstrated ATS. The early detection of ATS has been demonstrated to improve patient outcome. This case provides an overview of the typical imaging features of ATS to aid radiologists in making this uncommon diagnosis.

  11. Biliary Dyskinesia as a Rare Presentation of Metastatic Breast Carcinoma of the Gallbladder: A Case Report

    Directory of Open Access Journals (Sweden)

    A. Markelov

    2011-01-01

    Full Text Available Background. Breast carcinoma is the most common malignancy in women worldwide. It is most commonly associated with metastases to the liver, lung, bone, and the brain. Invasive lobular carcinoma is a less common pathology with slightly higher metastases to the upper gastrointestinal tract. Invasive lobular carcinoma metastasis to the gallbladder is extremely rare. Method. In this paper we are presenting a case of a 67-year-old female with metastases of invasive lobular breast cancer to the gallbladder six years after her therapy. Conclusion. This case clearly signifies the nature of the micrometastatic foci of the invasive lobular carcinoma even many years after a successful treatment.

  12. Aggressive Digital Papillary Adenocarcinoma in a Young Female-a Rare Presentation.

    Science.gov (United States)

    Gole, Gautam Nandkumar; Tati, Shekhar Y; Deshpande, Ashok Kumar; Gole, Sheetal G

    2011-06-01

    A 20 year old female presented with a recurrent soft tissue swelling over the medial aspect of proximal phalanx of left little finger. It had recurred one year after excision. There was no lymphadenopathy or bony involvement. Previous histopathology reports were not available. After excision histopathological diagnosis was aggressive digital papillary adenocarcinoma. Later Ray's amputation of little finger was planned. Aggressive digital papillary adenocarcinomas are rare sweat gland tumors which occur on hands, fingers, and toes. They have high local recurrence rate and may metastasize to lungs and lymph nodes.

  13. The Patient Educator Presentation in Dental Education: Reinforcing the Importance of Learning About Rare Conditions.

    Science.gov (United States)

    Edwards, Paul C; Graham, Jasmine; Oling, Rebecca; Frantz, Kate E

    2016-05-01

    The aim of this study was to determine whether a patient educator presentation (PEP) on pemphigus vulgaris would increase second-year dental students' awareness of the importance of learning about rare conditions and improve their retention of rare disease knowledge. The study involved students' subjective assessments of a PEP experience at two U.S. dental schools. In this mixed methods study, cross-sectional data were obtained by surveys and in-depth interviews. Questions focused on students' assessment of the messages acquired from the PEP and its likely impact on their future clinical care. At University 1, students completed paper surveys with open-ended questions and participated in a focus group. At University 2, students completed an online survey consisting of rating scale and open-ended questions. Responses to open-ended questions were categorized into themes. At University 1, 79 students (out of a possible 102; response rate 77.5%) completed the survey, and an additional ten students participated in a focus group. At University 2, 30 students (out of a possible 104; response rate 28.8%) completed the survey. At Universities 1 and 2, 88% and 100%, respectively, of respondents stated the PEP would influence their future clinical decision making. The vast majority of respondents (94% and 100% at University 1 and University 2, respectively) were of the opinion that the personal testimonial from a patient would help them recall information about pemphigus vulgaris in five years' time. Respondents from both universities commented that the PEP emphasized the importance of not dismissing a patient's concerns. These results suggest that a presentation by a patient with a rare condition can be an effective educational tool for preclinical dental students.

  14. Acute abdominal pain presenting as a rare appendiceal duplication: a case report

    Directory of Open Access Journals (Sweden)

    Mahmood Ali

    2012-03-01

    Full Text Available Abstract Introduction Appendiceal duplication is a rare anomaly that can manifest as right lower quadrant pain. There are several variations described for this condition. We recommend aggressive operative management should this anatomical variation present in the presence of acute appendicitis. Case presentation We report the case of a 15-year-old African American girl who presented to our hospital with right lower quadrant pain and was subsequently found to have appendiceal duplication. Conclusion There are two categorical systems that have described and stratified appendiceal duplication. Both classification systems have been outlined and referenced in this case report. A computed tomography scan has been included to provide a visual aid to help identify true vermiform appendiceal duplication. The presence of this anatomical abnormality is not a reason for surgical intervention; however, should this be found in the setting of acute appendicitis, aggressive resection of both appendices is mandatory.

  15. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis.

    Science.gov (United States)

    Konca Degertekin, Ceyla; Ozkurt, Zübeyde Nur; Akyürek, Nalan; Yağcı, Münci

    2014-09-01

    Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic myelofibrosis refractory to treatment, developing EMH after splenectomy in various organs, exceptionally the skin and the heart. Along with the case, the clinical presentations, treatment options, prognostic significance of EMH and the role of cytogenetics is discussed in the light of the literature.

  16. A rare presentation of atypical demyelination: tumefactive multiple sclerosis causing Gerstmann’s syndrome

    Science.gov (United States)

    2014-01-01

    Background Tumefactive demyelinating lesions are a rare manifestation of multiple sclerosis (MS). Differential diagnosis of such space occupying lesions may not be straightforward and sometimes necessitate brain biopsy. Impaired cognition is the second most common clinical manifestation of tumefactive MS; however complex cognitive syndromes are unusual. Case presentation We report the case of a 30 year old woman who presented with Gerstmann’s syndrome. MRI revealed a large heterogeneous contrast enhancing lesion in the left cerebral hemisphere. Intravenous corticosteroids did not stop disease progression. A tumour or cerebral lymphoma was suspected, however brain biopsy confirmed inflammatory demyelination. Following diagnosis of tumefactive MS treatment with natalizumab effectively suppressed disease activity. Conclusions The case highlights the need for clinicians, radiologists and surgeons to appreciate the heterogeneous presentation of tumefactive MS. Early brain biopsy facilitates rapid diagnosis and management. Treatment with natalizumab may be useful in cases of tumefactive demyelination where additional evidence supports a diagnosis of relapsing MS. PMID:24694183

  17. Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

    Science.gov (United States)

    Morais, Barbara Albuquerque; Cardeal, Daniel Dante; Ribeiro E Ribeiro, Renan; Frassetto, Fernando Pereira; Andrade, Fernanda Goncalves; Matushita, Hamilton; Teixeira, Manoel Jacobsen

    2017-08-01

    Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

  18. Osteosarcoma of the Maxilla: A Rare Case with Unusual Clinical Presentation

    Directory of Open Access Journals (Sweden)

    Pouyan Amini Shakib

    2013-08-01

    Full Text Available Osteosarcoma (OS is a malignant mesenchymal tumor, which rarely occurs in the maxilla. Although variable histologic and radiographic features of OS have been reported previously, in the majority of the cases painful swelling of the jaw is mentioned as the first clinical presentation. Furthermore, early diagnosis and wide surgical resection of the tumor are the most important determinant factors of prognosis. Therefore, the unusual clinical presentations of OS should be considered meticulously to expedite the diagnosis process. We describe a case of OS of the maxilla with extremely unusual presentation in a 42-year-old female, that was initially designated as “epulis fissuratum”. Here, we highlight the importance of combining the clinical, radiographic and histopathologic examination to obtain a definitive diagnosis and also the significance of early effective surgical intervention in evaluation of pathologic lesions.

  19. A rare pediatric case of grossly dilated ureter presenting as abdominal mass

    International Nuclear Information System (INIS)

    Srivastava, Madhur Kumar; Govindarajan, Krishna Kumar; Chakkalakkoombil, Sunitha Vellathussery; Halanaik, Dhanapathi

    2016-01-01

    Renal masses account for 55% of cases presenting as palpable abdominal mass in children.[1] An eight year male presented with palpable abdominal mass and pain. The patient underwent renal dynamic scan, which raised possibility of left duplex kidney with non-functioning moiety, as the size of left kidney was smaller than seen on Ultrasonography (USG). Magnetic resonance (MR)urography confirmed the findings with patient undergoing left hemi-nephrectomy and is doing well. In case of discrepancy in size of kidney on USG and renal scan, duplex kidney should be considered as differential, other causes being, renal cyst, benign/malignant mass and renal calculi. Gross hydro-ureter presenting as palpable abdominal mass is very rare with few reported cases.[234

  20. Avascular necrosis of femoral heads post-adrenal surgery for Cushing's syndrome: a rare presentation.

    LENUS (Irish Health Repository)

    2012-01-31

    Avascular necrosis (AVN) is a well-recognized complication of patients on high-dose steroids for a long time. Exogenous hypercortisolism is a well known cause of AVN and a number of cases have been reported. Cushing\\'s syndrome describes hypercortisolism of any cause endogenous or exogenous. A variety of traumatic and non-traumatic factors contribute to the aetiology of AVN although exogenous glucocorticoids administration and alcoholism are among the most common non-traumatic causes. AVN secondary to endogenous hypercortisolism is rare and very few case reports are available describing this complication. No literature is available on AVN presenting post-adrenal surgery. Here we present a young woman who presented with avascular necrosis of both hips 1 year after adrenalectomy for Cushing\\'s syndrome.

  1. "Laparoscopic excision of a large ovarian cyst herniating into the inguinal canal: a rare presentation".

    Science.gov (United States)

    Machado, Norman Oneil; Machado, Lovina S M; Al Ghafri, Wadha

    2011-08-01

    Inguinal hernia repair is one of the most common operation in surgical practice. Despite its common occurrence, hernia often poses a surgical dilemma even for a skilled surgeon. The unexpected hernial content constitutes one of these cases. Although the often-reported, unusual contents of a hernia sac include ovary, fallopian tube, vermiform appendix, Meckel diverticulum, and urinary bladder, the herniation of a large ovarian cyst into the inguinal canal has been hardly reported. Majority of the ovarian cysts are asymptomatic or present with vague lower abdominal pain, whereas the presentation of a large ovarian cyst as an inguinolabial swelling as in our patient is extremely rare. We present here one of the few reported cases of a laparoscopic excision of a large ovarian cyst herniating into the inguinal canal and discuss the pathogenesis of an ovarian cyst as hernial content, the advantages and concerns of a laparoscopic approach in resecting large ovarian cysts, and simultaneous management of the inguinal hernia.

  2. Cerebriform intradermal nevus presenting as cutis verticis gyrata with multiple cellular blue nevus over the body: A rare occurrence

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    Somenath Sarkar

    2014-01-01

    Full Text Available Cutis verticis gyrata is a rare skin condition characterized by swelling of scalp resembling the surface of the brain. Various conditions, like cerebriform intradermal nevus (CIN, may give rise to this clinical entity. Moreover, its association with cellular blue nevus is extremely rare and has not been reported so far. Here, we report a 28-year-old male with a huge cerebriform swelling covering the occipital lobe along with multiple nodules all over the body. Histology of the scalp swelling showed solitary or clusters of nevus cells in the dermis and from the body lesions showed features of cellular blue nevus. The diagnosis of CIN with cellular blue nevus was confirmed

  3. A rare presentation of ruptured interstitial ectopic pregnancy with broad ligament hematoma: A case report

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    Ahmed M. Abbas

    2017-03-01

    Full Text Available Ectopic pregnancy is a major cause of maternal morbidity and mortality in the first trimester. Interstitial type is the most dangerous variety with a high risk of life-threatening internal hemorrhage. Obstetricians need a high index of suspicion to diagnose such rare type. We are reporting a rare case of ruptured interstitial ectopic pregnancy presented with a large broad ligament hematoma early in the first trimester. A 25-year-old woman was presented with gradual onset of increasing abdominal pain after 6 weeks of amenorrhea. She had a positive urinary pregnancy test. Abdominal ultrasound revealed bulky empty uterus and ill-defined mass at the right side of the uterus. On exploration, incision and drainage of broad ligament hematoma were performed in addition to right salpingectomy. Interstitial ectopic pregnancy represents a diagnostic and therapeutic challenge and frequently constitutes an obstetrical emergency. Its rupture early in the first trimester should be expected. Early diagnosis and proper management are the most important issues to avoid its catastrophic consequences.

  4. Simultaneous meningioma and brain metastasis from renal cell carcinoma – a rare presentation. Case report

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    Aline Lariessy Campos Paiva

    2017-05-01

    Full Text Available ABSTRACT CONTEXT: Brain metastases are the most common tumors of the central nervous system. Because of their high frequency, they may be associated with rare situations. Among these are tumor-to-tumor metastasis and an even a rarer situation called simultaneous brain tumors, which are more related to primary tumors of the reproductive and endocrine systems. CASE REPORT: A 56-year-old male patient with a history of renal cell carcinoma (which had previously been resected presented with a ventricular lesion (suggestive of metastatic origin and simultaneous olfactory groove lesion (probably a meningioma. First, only the ventricular lesion was dealt with, but after a year, the meningothelial lesion increased and an occipital lesion appeared. Therefore, both of these were resected in a single operation. All the procedures were performed by the same neurosurgeon. The patient evolved without neurological deficits during the postoperative period. After these two interventions, the patient remained well and was referred for adjuvant treatment. CONCLUSIONS: This study provides the first description of an association between these two tumors. Brain metastases may be associated with several lesions, and rare presentations such as simultaneity with meningioma should alert neurosurgeons to provide the best oncological treatment.

  5. Ascites in a Young Woman: A Rare Presentation of Eosinophilic Gastroenteritis

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    Carina Santos

    2018-01-01

    Full Text Available Introduction. Eosinophilic gastroenteritis (EGE is a rare idiopathic disease that can affect one or more organs of the digestive tract. It has an estimated incidence of 1–20 cases per 100,000 patients. Klein et al. classified EGE into 3 subtypes: predominant mucosal, muscular, or subserosal. Clinical Case. We report a case of a 32-year-old woman, who presented with diffuse abdominal pain, nausea, postprandial infarction, diarrhea, and moderate ascites of three-week evolution. The rest of physical examination did not show alterations. The past medical history was unremarkable. Laboratory test results revealed peripheral blood eosinophilia. Abdominal CT scan revealed diffuse and concentric parietal thickening of the distal 2/3 of esophagus, moderate volume ascites, and small bowel wall thickening and distension on the left quadrants. The paracentesis revealed 93.3% of eosinophils. The colon biopsies evidenced an increase in the number of eosinophils. Secondary causes of eosinophilia were excluded. The patient was treated with oral prednisolone 40 mg/day with immediate clinical and analytical improvement. Conclusion. Eosinophilic gastroenteritis is a rare condition with a nonspecific and highly variable clinical presentation, which requires a high level of clinical suspicion. It is a diagnosis of exclusion. Secondary causes of eosinophilia such as intestinal tuberculosis, parasitosis, and malignant neoplasms should be excluded.

  6. Actinomycotic Osteomyelitis of Maxilla Presenting as Oroantral Fistula: A Rare Case Report

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    Ashalata Gannepalli

    2015-01-01

    Full Text Available Actinomycosis is a chronic granulomatous infection caused by Actinomyces species which may involve only soft tissue or bone or the two together. Actinomycotic osteomyelitis of maxilla is relatively rare when compared to mandible. These are normal commensals and become pathogens when they gain entry into tissue layers and bone where they establish and maintain an anaerobic environment with extensive sclerosis and fibrosis. This infection spreads contiguously, frequently ignoring tissue planes and surrounding tissues or organ. The portal of entry may be pulpal, periodontal infection, and so forth which may lead to involvement of adjacent structures as pharynx, larynx, tonsils, and paranasal sinuses and has the propensity to damage extensively. Diagnosis is often delayed and is usually based on histopathology as they are cultured in fewer cases. The chronic clinical course without regional lymphadenopathy may be essential in diagnosis. The management of actinomycotic osteomyelitis is surgical debridement of necrotic tissue combined with antibiotics for 3–6 months. The primary actinomycosis arising within the maxilla with contiguous involvement of paranasal sinus with formation of oroantral fistula is rare. Hence, we present a 50-year-old female patient with chronic sclerosing osteomyelitis of maxilla which presented as oroantral fistula with suppurative and sclerotic features.

  7. Tuberculous Dactylitis with Concomitant Thyroid Involvement: A Rare Presentation of Childhood Tuberculosis.

    Science.gov (United States)

    Qamar, Sobia; Naz, Farrah; Naz, Samia; Ejaz, Iftikhar

    2017-03-01

    Extrapulmonary tuberculosis rarely presents as thyroid involvement along with other manifestations, and poses a diagnostic challenge on account of paucibacillary nature of disease. In general, the diagnosis of tuberculosis is based on epidemiological risk factors, clinical features, imaging studies, in addition to a positive skin testing or Interferon Gamma Release Assay (IGRA). A 14-year boy presented with history of fever and weight loss for one year. On examination, he had painful swelling of fingers and toes along with a painless thyroid nodule and squint. Hand X-ray showed lytic-sclerotic lesions in phalanges. MRI of brian showed multiple ring enhancing lesions and radionuclide thyroid scan showed multinodular goitre. Histology showed epithelioid cell granulomas (thyroid and bone) and tuberculomas of brain confirmed tuberculosis. He responded well to four-drug anti-tuberculous therapy and his fever, squint, thyroid nodule, and dactylitis disappeared. Tuberculosis of thyroid, a rare phenomenon, can be diagnosed and treated well; if clinical index of suspicion is kept high, particularly in tuberculosis prevalent areas.

  8. Rare presentation of intralobar pulmonary sequestration associated with repeated episodes of ventricular tachycardia.

    Science.gov (United States)

    Rao, D Sheshagiri; Barik, Ramachandra

    2016-07-26

    Arterial supply of an intralobar pulmonary sequestration (IPS) from the coronary circulation is extremely rare. A significant coronary steal does not occur because of dual or triple sources of blood supply to sequestrated lung tissue. We present a 60-year-old woman who presented to us with repeated episodes of monomorphic ventricular tachycardia (VT) in last 3 mo. Radio frequency ablation was ineffective. On evaluation, she had right lower lobe IPS with dual arterial blood supply, i.e., right pulmonary artery and the systemic arterial supply from the right coronary artery (RCA). Stress myocardial perfusion scan revealed significant inducible ischemia in the RCA territory. Coronary angiogram revealed critical stenosis of proximal RCA just after the origin of the systemic artery supplying IPS. The critical stenosis in the RCA was stented. At 12 mo follow-up, she had no further episodes of VT or angina.

  9. Paediatric Stroke: A Rare Presentation of Iron Deficiency Anemia in a Four Year Old Child.

    Science.gov (United States)

    Subedi, K; Koirala, S; Basnet, R; Poudel, A

    Paediatric stroke is an uncommon syndrome with even lesser annual incidence rates of arterial ischemic stroke in infants and children. In children the diagnosis of stroke is frequently delayed or missed. This is due to subtle and nonspecific clinical presentations, a complicated differential diagnosis and a lack of awareness by physicians and also delay in the seeking of medical attention as in our case. We report you a rare case of a four year old child from remote Nepal who presented to our Out Patient Department after a long gap of around four months after the sudden onset of loss of consciousness and decreased movement of right limbs who after detailed history examination and lab investigations and imaging revealed ischemic stroke due to iron deficiency anemia.

  10. A rare case of Amyand’s hernia presenting as an enterocutaneous fistula

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    L Flood

    2010-09-01

    Full Text Available The finding of the vermiform appendix in an inguinal hernia has an incidence of approximately 1%. The condition is given the eponymous name Amyand’s hernia. However in just 0.08% the condition is complicated by an acute appendicitis. The clinical presentation varies, depending on the extent of inflammation of the appendix and is most often misdiagnosed as an incarcerated inguinal hernia. As such it is rarely recognised prior to surgical exploration. We report a case of Amyand’s hernia in an 85 year old woman, which presented as a right groin enterocutaneous fistula. CT scanning illustrated a fistulous tract in the right groin, which communicated with the caecum and the peritoneal cavity. She underwent laparotomy, which revealed that the appendix appeared inflamed, lay in the inguinal canal and was the origin of the enterocutaneous communication.

  11. Pancreatic Calculus Causing Biliary Obstruction: Endoscopic Therapy for a Rare Initial Presentation of Chronic Pancreatitis.

    Science.gov (United States)

    Shetty, Anurag J; Pai, C Ganesh; Shetty, Shiran; Balaraju, Girisha

    2015-09-01

    Biliary obstruction in chronic calcific pancreatitis (CCP) is often caused by inflammatory or fibrotic strictures of the bile duct, carcinoma of head of pancreas or less commonly by compression from pseudocysts. Pancreatic calculi causing ampullary obstruction and leading to obstructive jaundice is extremely rare. The medical records of all patients with CCP or biliary obstruction who underwent endoscopic retrograde cholangiopancreatography (ERCP) over 4 years between 2010-2014 at Kasturba Medical College, Manipal were analyzed. Five patients of CCP with impacted pancreatic calculi at the ampulla demonstrated during ERCP were identified. All 5 presented with biliary obstruction and were incidentally detected to have CCP when evaluated for the same; 3 patients had features of cholangitis. All the patients were managed successfully by endoscopic papillotomy and extraction of pancreatic calculi from the ampulla with resolution of biliary obstruction. Pancreatic calculus causing ampullary obstruction, though very rare, should be considered as a possibility in patients with CCP complicated by biliary obstruction. Endoscopic therapy is affective in the resolution of biliary obstruction in such patients.

  12. Cerebral venous sinus thrombosis: A diagnostic challenge in a rare presentation

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    Sultan AbdulWadoud Alshoabi

    2017-01-01

    Full Text Available Cerebral venous sinus thrombosis (CVST is an uncommon, life-threatening condition with a variable clinical presentation that makes it a challenge of diagnosis. A 39-year-old male patient presented to the hospital with complete loss of conscious and admitted to Medical Intensive Care Unit for investigation without any obvious history that was difficult for diagnosis. In this case, the patient presented with coma that is a rare presentation of CVST with no obvious clinical history and he was male patient that means he is free of all gender-specific risk factors of CVST. The brain computed tomography (CT scan showed hypodense lesion in the left upper parietal region with no hemorrhage. The lesion was low-signal intensity (SI on T1WIs and high SI on T2WIs and restricted on diffusion-weighted images like arterial infarctions, but magnetic resonance angiography (MRA was normal that excluded arterial infarction. Gadolinium-enhanced MR venography (MRV showed the filling defect of CVST. CVST can be present by a mysterious clinical presentation that makes it as a challenge of diagnosis even by medical imaging by CT and MR imaging (MRI. A combination of MRI and MRV is the best, noninvasive, and nonionizing imaging modality for the diagnosis of CVST.

  13. Isolated Juvenile Xanthogranuloma in Thoracic Spine: Intraoperative Cytological Diagnosis of a Rare Presentation

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    Shashi Singhvi

    2014-06-01

    Full Text Available Juvenile Xanthogranulomas (JXG are benign proliferative disorders of non-Langerhans histiocytes, which present in children as multiple, self-limited, cutaneous lesions. The extracutaneous manifestations of JXG are uncommon, and isolated JXG involving the spinal column is extremely rare. We report here a case of isolated juvenile xanthogranuloma in thoracic spine correctly diagnosed intraoperatively on crush smear cytology and later confirmed by histopathological and immunohistochemical studies. This case report draws attention to the fact that isolated xanthogranuloma should be considered among possible diagnoses of spinal tumor in children. Also, since the long term survival depends on complete surgical resection, a correct intraoperative diagnosis is extremely important for optimal management and prognosis of the patient. [J Interdiscipl Histopathol 2014; 2(3.000: 158-162

  14. Viable Intrauterine Pregnancy and Coexisting Molar Pregnancy in a Bicornuate Uterus: A Rare Presentation

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    Kavitha Krishnamoorthy

    2016-01-01

    Full Text Available A complete hydatidiform mole with a viable coexisting fetus (CMCF is a rare occurrence. Similarly, Mullerian anomalies such as a bicornuate uterus are uncommon variants of normal anatomy. We report a case of a 40-year-old female with a known bicornuate uterus presenting at 13 weeks gestation with vaginal bleeding. Ultrasound findings showed a healthy viable pregnancy in the right horn with complete molar pregnancy in the left horn. After extensive counseling, the patient desired conservative management, however, was unable to continue due to profuse vaginal bleeding. The patient underwent suction dilation and curettage under general anesthesia and evacuation of the uterine horns. Postoperatively, the patient was followed until serum beta-human chorionic gonadotropin (β-hCG level dropped to <5 mU. This is the first case of a CMCF reported in a bicornuate uterus, diagnosed with the use of ultrasound imaging.

  15. Metastatic hepatocellular carcinoma in the maxilla and mandible, an extremely rare presentation

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    Satya Ranjan Misra

    2015-01-01

    Full Text Available Malignancy is characterized by anaplasia, invasiveness, and metastasis. Primary oral squamous cell carcinoma is the most prevalent oral malignancy, but secondary malignancy from distant sites have also been reported. Hepatocellular carcinoma (HCC is a common primary liver malignancy that frequently metastasizes during the course of the disease, but < 1% of cases show oral involvement. Such secondary neoplasms do not have any pathognomonic clinical or radiologic findings, and therefore they pose a diagnostic challenge. Hence, in the differential diagnosis of malignant tumors of the oral cavity, it is essential to consider the occurrence of both primary as well as metastatic tumors despite the low incidence of the latter. A rare case of HCC metastasizing to both the maxilla and mandible is presented, in which the patient succumbed to the disease as a result of the delay in diagnosis.

  16. Isolated unilateral sixth cranial nerve palsy: A rare presentation of dengue fever

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    Yang Liang Boo

    2016-11-01

    Full Text Available Dengue fever is a common mosquito-borne viral infection endemic in tropical and subtropical countries. Neurological manifestations in dengue infection are relatively uncommon, and include encephalitis, encephalopathy, neuromuscular disorders and neuro-ocular disorders. Cranial mononeuropathy is a rare manifestation of dengue infection. A 40-year-old man was diagnosed with isolated, unilateral sixth cranial nerve palsy complicating dengue infection. The patient was managed accordingly, and full ocular recovery was observed. This was the first reported case of isolated sixth cranial nerve palsy associated with dengue fever in Malaysia. It is important for clinicians to consider dengue as a differential diagnosis in patients presenting with fever and sixth cranial nerve palsy.

  17. A rare ovarian tumor, leydig stromal cell tumor, presenting with virilization: a case report

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    Soheila Aminimoghaddam

    2012-11-01

    Full Text Available  Abstract Leydig stromal cell tumor is a rare ovarian tumor that belongs to the group of sex-cord stromal tumors. They produce testosterone leading to hyperandrogenism. We present a 41yr old woman with symptoms of virilization and a mass of right adenex via ultra Sonography, and a rise of total and free serum testosterone. An ovarian source of androgen was suspected and a surgery performed. A diagnosis of leydig-stromal cell tumor was confirmed. Our report is a reminder that although idiopathic hirsutism and other benign androgen excess disorder like Polycystic Ovarian Syndrome (PCOs are common, ovarian mass should be considered in differential diagnosis. 

  18. [The hidradenocarcinoma of the wrist - an extremely rare malignant carcinoma: case presentation and literature review].

    Science.gov (United States)

    Arsalan-Werner, A; Mentzel, T; Kempf, B; Sauerbier, M

    2013-10-01

    Hidradenocarcinomas are rare, yet highly malignant tumors of eccrine sweat gland origin. Due to its locally aggressive growth and likelihood for metastasis it should be considered as a differential diagnosis especially in case of suspicious intraoperative findings. We report the case of a 73-year-old female patient presenting with a hidradenocarcinoma of the wrist. Treatment of hidradenocarcinomas is similar to the treatment of sarcomas: The first step is an incisional biopsy and obtaining an expert second opinion on the histopathological diagnosis as well as staging. The second step is a clear margin resection of the tumor and the plastic-surgical reconstruction. A long-term follow-up is mandatory to detect potential recurrence or metastasis. © Georg Thieme Verlag KG Stuttgart · New York.

  19. Cor triatriatum dexter, a very rare congenital anomaly presented in a complex pathological context – case presentation

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    Gozar Liliana

    2014-08-01

    Full Text Available Background Cor triatriatum dexter is an extremely rare congenital anomaly, and in most cases, without hemodynamic significance. Congenital cytomegalovirus infection is the most common viral infection, the majority of infected infants being asymptomatic and only 5-15% being symptomatic in the neonatal period.

  20. Carcinomatous meningitis due to gastric adenocarcinoma:A rare presentation of relapse

    Institute of Scientific and Technical Information of China (English)

    Nibal; Saad; Ahmad; Alsibai; Tarik; H; Hadid

    2014-01-01

    While solid tumors are less commonly associated with meningeal involvement; lung, breast and melanoma are the ones most often reported. A few case reports have included gastric carcinoma but these are rare and most often associated with systemic disease at the time of diagnosis. Here we report a unique presentation of gastric carcinoma relapse with leptomeningeal carcinomatosis. An 81-year-old female was diagnosed with gastric cancer approximately one year before presentation. Following neoadjuvant chemotherapy, she had gastrectomy. Her periodic surveillance was stable. Thereafter she presented with a one week history of progressive fatigue lightheadedness, syncope. During hospitalization her mental status deteriorated. A repeat computed axial tomography scan of the head showed no changes to suggest an etiology. A lumbar puncture was performed and cerebral spinal fluid(CSF) cytopathology confirmed gastric signet cell adenocarcinoma. Encephalopathy was likely caused by increased intracranial pressure from communicating hydrocephalus.Leptomeningeal carcinomatosis is associated with short life expectancy. Therapeutic lumbar punctures and best supportive care or systemic therapy can be applied with guarded prognosis. Survival, however, may improve with cytologic negative conversion of the CSF if patient performance status allows treatment.

  1. Onychomycosis: A Rare Presentation of Fungal Urinary Tract Infection in an Extremely Preterm Neonate

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    Shilpa Kalane

    2015-06-01

    Full Text Available Onychomycosis refers to nail infections, caused by fungi including yeasts and non-dermatophyte moulds. One or several toenails or fingernails (seldom all may be involved in this condition. Many cases of fingernail onychomycosis are due to yeasts. Fungal infection has emerged as an important cause of neonatal infection, associated with significant morbidity and mortality, especially in very low birth weight (< 1500 g and extremely low birth weight (< 1000 g infants. Herein, we report a case of a 24-day-old male infant, who presented with onychomycosis on the left ring fingernail, associated with fungal urinary tract infection (UTI. The evaluation of nails helped us detect fungal UTI. To date, there have been no reports suggesting onychomycosis as a presentation of fungal UTI. We could not find the association between onychomycosis and neonatal fungal UTI. Hence, retrospectively, it can be said that onychomycosis was a presentation of fungal UTI. Further studies are required to evaluate the etiology and treatment of neonatal onychomycosis. Moreover, dermatologists should pay particular attention to this rare event.

  2. Brown′s syndrome with ocular albinism: Case report of a rare presentation and literature review

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    Soumya Nambiar

    2015-01-01

    Full Text Available We report a rare case of Brown′s syndrome associated with ocular albinism in a 10-year-old boy. This report highlights the importance of further analysis of cases of Brown′s syndrome and throws some light on the etiology and association of this rare condition.

  3. Sudden Onset, Rapidly Expansile, Cervical Cystic Hygroma in an Adult: A Rare Case with Unusual Presentation and Extensive Review of the Literature

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    Vivek Dokania

    2017-01-01

    Full Text Available Cystic hygroma (CH is a benign infiltrative malformation of the lymphatic channels. We report a case of a 28-year-old Indian female who presented with rapidly enlarging right sided neck swelling over the posterior triangle since 5 days. Complete resection of CH is sometimes not amenable because of its infiltrative nature and involvement of surrounding vital structures. However, in our patient successful complete surgical resection was undertaken. The MRI findings of our patient were consistent with brachial cleft cyst; this posed a challenge in the diagnosis of CH. The histopathological analysis of the resected mass confirmed CH. CH is rare in adults and such an acute presentation is exceptionally atypical. History of prior trauma and infection are known etiological factors for adult CH; these were conspicuously absent in our patient. CH should be considered in the differentials of rapidly enlarging cystic swelling of posterior region of neck in adults. Optimal and timely management is necessary to achieve a favorable prognosis. Therefore, we report a case of rapidly enlarging cervical CH in an adult along with extensive literature review to have a better understanding regarding epidemiology, etiopathogenesis, clinical presentation, optimal management, and prognosis of such a rare entity in adults.

  4. A rare presentation of adult onset Still’s disease in an elderly patient

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    Mariya Apostolova

    2011-10-01

    Full Text Available Adult onset Still’s disease (AOSD is a rare inflammatory disorder of unknown etiology that usually affects young adults. Very few patients older than 70-year-old have been reported. Clinical features include quotidian fevers, arthralgias, arthritis, pharyngitis, lymphadenopathy and an evanescent rash. AOSD should be considered in the differential diagnosis of fever of unknown origin. Early diagnosis is often difficult since it is a diagnosis of exclusion and the presence of infectious, neoplastic and autoimmune conditions needs to be ruled out before the diagnosis is made. No specific laboratory tests are available to aid in the diagnosis of AOSD. As a result, a set of diagnostic criteria that define the clinical features of this condition, termed the Yamaguchi criteria, have been most commonly used to establish the diagnosis. We describe the case of a 72-year-old Caucasian male with past medical history significant for generalized anxiety disorder, depression, BPH, and hypertriglyceridemia, who presented to a tertiary institution complaining of profound generalized weakness and weight loss that started three weeks prior to presentation. Initial laboratory studies showed leukocytosis, elevated ESR, CRP, ferritin and liver dysfunction. Cultures, ANA and rheumatoid factor studies were negative. The patient underwent further extensive workup that excluded the presence of infectious, neoplastic and autoimmune disorders and was subsequently diagnosed with AOSD and new onset diabetes mellitus. For the management of AOSD he was started on prednisone with significant improvement in markers of inflammation, symptoms and level of function.

  5. Isolated Medial Rectus Nuclear Palsy as a Rare Presentation of Midbrain Infarction.

    Science.gov (United States)

    Al-Sofiani, Mohammed; Lee Kwen, Peterkin

    2015-10-08

    Diplopia is a common subjective complaint that can be the first manifestation of a serious pathology. Here, we report a rare case of midbrain infarction involving the lateral subnucleus of the oculomotor nuclear complex presenting as diplopia, with no other stroke manifestations. An 83-year-old right-handed white man with past medical history of diabetes mellitus, hypertension, dyslipidemia, and coronary artery disease presented to the emergency department (ED) with diplopia and unsteadiness. Two days prior to admission, the patient woke up with constant horizontal diplopia and unsteadiness, which limited his daily activities and led to a fall at home. He denied any weakness, clumsiness, nausea, vomiting, photophobia, fever, or chills. Ocular exam showed a disconjugate gaze at rest, weakness of the left medial rectus muscle, impaired convergence test, and bilateral 3-mm reactive pupils. The diplopia resolved by closing either eye. The remaining extraocular muscles and other cranial nerves were normal. There was no nystagmus, ptosis, or visual field deficit. Sensation, muscle tone, and strength were normal in all extremities. Magnetic resonance imaging (MRI) of the brain revealed a tiny focus of restricted diffusion in the left posterior lateral midbrain. A thorough history and physical examination is essential to diagnose and manage diplopia. Isolated extraocular palsy is usually thought to be caused by orbital lesions or muscular diseases. Here, we report a case of midbrain infarction manifested as isolated medial rectus palsy.

  6. A rare case of cervical epidural extramedullary plasmacytoma presenting with monoparesis

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    Turk Okan

    2017-03-01

    Full Text Available Multiple myeloma and other plasma cell disorders are characterized by production of a large number of plasma cells in the bone marrow. On the other hand, plasmacytoma results from proliferation of abnormal plasma cells in the soft tissue or skeletal system. Neurological complications are frequently observed in these diseases. The most commonly known complications among those complications are spine fractures, spinal cord compressions, and peripheral neuropathies. Although neurological involvements are common in plasmacytomas, extramedullary spinal epidural localizations have been reported very rarely. In this case report, we aimed to present a plasmacytoma case that presented with acute onset of upper extremity monoparesis. A 40-year-old woman was admitted to our clinic with complaints of sudden weakness and numbness in her left arm following neck and left arm pain. Emergency cervical magnetic resonance imaging (MRI revealed an epidural mass and the patient underwent emergency surgery. The patient showed improvement post-operatively and the pathology was reported as plasmacytoma. Following hematology consultation, systemic chemotherapy was initiated and radiotherapy was planned after wound healing.

  7. Occipital lobe infarction: a rare presentation of bilateral giant cavernous carotid aneurysms: a case report.

    Science.gov (United States)

    Vanikieti, Kavin; Poonyathalang, Anuchit; Jindahra, Panitha; Cheecharoen, Piyaphon; Chokthaweesak, Wimonwan

    2018-02-02

    Cavernous carotid aneurysm (CCA) represents 2-9% of all intracranial aneurysms and 15% of internal carotid artery (ICA) aneurysms; additionally, giant aneurysms are those aneurysms that are > 25 mm in size. Bilateral CCAs account for 11-29% of patients and are commonly associated with structural weaknesses in the ICA wall, secondary to systemic hypertension. CCAs are considered benign lesions, given the low risk for developing major neurologic morbidities (i.e., subarachnoid hemorrhage, cerebral infarction, or carotid cavernous fistula). Moreover, concurrent presentation with posterior circulation cerebral infarction is even rarer, given different circulation territory from CCA. Here, we report on a patient with bilateral giant CCAs who presented with both typical and atypical symptoms. An 88-year-old hypertensive woman presented with acute vertical oblique binocular diplopia, followed by complete ptosis of the right eye. Ophthalmic examination showed dysfunction of the right third, fourth, and sixth cranial nerves. Further examination revealed hypesthesia of the areas supplied by the ophthalmic (V1) and maxillary (V2) branches of the right trigeminal nerve. Bilateral giant cavernous carotid aneurysms, with a concurrent subacute right occipital lobe infarction, were discovered on brain imaging and angiogram. Additionally, a prominent right posterior communicating artery (PCOM) was revealed. Seven months later, clinical improvement with stable radiographic findings was documented without any intervention. Dysfunction of the third, fourth, and sixth cranial nerves, and the ophthalmic (V 1 ) and maxillary (V 2 ) branches of the trigeminal nerves, should necessitate brain imaging, with special attention given to the cavernous sinus. Despite unilateral symptomatic presentation, bilateral lesions cannot be excluded solely on the basis of clinical findings. CCA should be included in the differential diagnosis of cavernous sinus lesions. Although rare, ipsilateral

  8. A rare case of cleidocranial dysplasia presenting with failure to thrive

    Science.gov (United States)

    Mahajan, Parag Suresh; Mahajan, Anuradha Parag; Mahajan, Prashant Suresh

    2015-01-01

    Cleidocranial dysplasia (CCD) is a rare (1:1,000,000) congenital condition secondary to spontaneous mutation (40%) or autosomal dominant inheritance (60%) affecting skeletal and dental systems. Hypomineralization of the hypoplastic clavicles and/or cranium is the major feature observed by prenatal ultrasound. Radiologically clavicles are hypoplastic or absent in chest X-ray. Delayed closure of the fontanelle and the skull sutures in pediatric and adolescent population and increased mobility of shoulders in all age groups (exhibited by the ability to bring shoulders close to each other) are prominent clinical diagnostic features of CCD. The diagnosis of CCD is often missed or significantly delayed. The management of CCD involves a multidisciplinary approach and its early diagnosis is essential to select an optimum plan and therapeutic benefit. We present here a case of CCD in a 17-month-old girl referred to us for investigation of below average weight and height gain; we stress on the usefulness of early diagnosis in the management of CCD and discuss current management concepts. PMID:25810671

  9. Bilateral Non-Hodgkin’s Lymphoma of the Temporal Bone: A Rare and Unusual Presentation

    Directory of Open Access Journals (Sweden)

    Sanjay Vaid

    2016-01-01

    Full Text Available Primary lymphoma of the temporal bone is an unusual finding in clinical practice and bilateral affection is even more rare. To the best of our knowledge, there are no reports of bilateral primary temporal bone lymphoma without middle ear involvement in the English medical literature so far. We report, for the first time, a case of primary lymphoma involving both temporal bones which presented with left-sided infranuclear facial palsy. A combination of contrast enhanced magnetic resonance imaging (MRI and high resolution computed tomography (HRCT was used to characterize and to map the extent of the lesion, as well as to identify the exact site of facial nerve affection. An excision biopsy and immunohistochemistry revealed diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL. Whole body fluorodeoxyglucose (FDG positron emission tomography-computed tomography study (PET-CT was performed to stage the disease. The patient was treated with chemotherapy and radiation therapy and is now on regular follow-up. The patient is alive and asymptomatic without disease progression for the last twenty months after initial diagnosis.

  10. A Hispanic female patient with heartburn: A rare presentation of Paroxysmal Nocturnal Hemoglobinuria.

    Science.gov (United States)

    Figueroa-Jiménez, Luis A; González-Márquez, Amy Lee; Alicea-Guevara, Ricardo; Santiago-Casiano, Mónica; de la Paz-López, Maryknoll; Negrón-Garcia, Luis; Báez-Diaz, Luis; Cáceres-Pérkins, William

    2015-01-01

    Paroxysmal nocturnal hemoglobinuria (PNH) is a non-malignant, acquired clonal hematopoietic stem cell disease that can present with bone marrow failure, hemolytic anemia, smooth muscle dystonias, and thrombosis. We present a case of a 32 year-old-female, G2P2A0 with no past medical history of any systemic illnesses who refers approximately 2 months of progressively worsening constant heartburn with associated abdominal discomfort. CBC showed leukopenia (WBC 2.9 x 103 /µL) with neutropenia (segmented neutrophils 48%), macrocytic anemia (Hgb 6.1 g/dL, hematocrit 20%, MCV,113 fL) and thrombocytopenia (platelet count 59 x 109/L). Abdomino-pelvic CT scan revealed a superior mesenterc vein thrombosis, which was treated initially with low-molecular-weight heparih for full anticoagulation. Peripheral blood flow cytometry assays revealed diminished expression of CD55 and CD59 on the erythrocytes, granulocytes and monocytes.' Paroxysmal nocturnal hemoglobinuria is a rare, clonal, hematopoietic stem-cell disorder whose manifestations are almost entirely explained by complement-mediated intravascular hemolysis. The natural history of PNH is highly variable, ranging from indolent to life-threatening. The median survival is 10 to 15 years, but with a wide range. Thrombosis is the leading cause of death, but others may die of complications of bone marrow failure, renal failure, myelodysplastic syndrome, and leukemia. Anticoagulation is only partially effective in preventing thrombosis in PNH; thus, thrombosis is an absolute indication for initiating treatment with Eculizumab. Nevertheless, bone marrow transplantation (BMT) is still the only curative therapy for PNH but is associated with significant morbidity and mortality.

  11. Antro-duodenal tuberculosis causing gastric outlet obstruction--a rare presentation of a protean disease.

    Science.gov (United States)

    Gheorghe, Liana; Băncilă, Ion; Gheorghe, Cristian; Herlea, Vlad; Vasilescu, Cătălin; Aposteanu, Gabriela

    2002-06-01

    Gastroduodenal tuberculosis is a rare location of abdominal tuberculosis. It usually occurs secondary to pulmonary tuberculosis. We report a case of a 63-year-old woman admitted to the referral center for symptoms of upper gastrointestinal obstruction caused by ulcerohypertrophic antroduodenal tuberculosis. The lesion was misdiagnosed as malignancy at endoscopy. Even at surgery, the lesion was considered gastric cancer and imposed an oncologic resection. The diagnosis was established in the presence of giant-cell granulomas with caseating necrosis in the surgical resected specimens. In our case, the rare gastroduodenal location of abdominal tuberculosis occurred as primary tuberculosis, in the absence of other identifiable locations.

  12. Megalourethra with Y-Type Duplication of Urethra Presented as Perianal Fistula: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Shashi Verma

    2015-01-01

    Full Text Available Megalourethra with Y-type duplication is an extremely rare anomaly. We report here one such case, diagnosed with retrograde urethrogram, which was done from both penile meatus and perianal opening simultaneously. Patient was successfully treated by laser optical internal urethrotomy (OIU, excision of duplicated urethra, and reduction urethroplasty in a single stage.

  13. A rare presentation of childhood Pompe disease : Cardiac involvement provoked by Epstein-Barr virus infection

    NARCIS (Netherlands)

    Talsma, Melle; Kroos, MA; Visser, G; Kimpen, JLL; Niezen, KE

    Myocarditis attributed to Epstein-Barr virus (EBV) as the sole cause is a rare manifestation. Myocarditis ascribed to EBV infection in combination with other factors has been reported in a few more cases. We report a child who experienced active EBV infection and later, at 19 months of age, received

  14. Bilateral Wyburn-Mason Syndrome presenting as acute subarachnoid haemorrhage - a very rare congenital neurocutaneuos disorder

    DEFF Research Database (Denmark)

    Cortnum, Søren Ole Stigaard; Sørensen, Preben; Andresen, J

    2008-01-01

    . Wyburn-Mason syndrome is a very rare congenital neurocutaneuos disorder comprising of vascular malformations of the retina, ipsilateral cerebral AVMs and occasionally lesions in the oronasopharyngeal area. Subarachnoid haemorrhage associated with Wyburn-Mason syndrome has been described in only 5...

  15. A rare case of extracranial meningioma in parapharyngeal space presented as a neck mass

    Directory of Open Access Journals (Sweden)

    Nader Albsoul

    2015-01-01

    Conclusion: Extracranial meningiomas are quite rare. The diagnosis of these types of tumors is challenging due to the non specific nature of the symptoms. The anatomic complexity of the region of parapharyngeal space also makes their detection difficult. Imaging modalities can aid in the diagnosis, but pathological examinations are essential in confirming a definite diagnosis.

  16. Doença de Marchiafava-Bignami: uma rara entidade com prognóstico sombrio Marchiafava-Bignami disease: a rare entity with a poor outcome

    Directory of Open Access Journals (Sweden)

    Paulo Eduardo Mestrinelli Carrilho

    2013-03-01

    Full Text Available A doença de Marchiafava-Bignami é uma entidade rara, caracterizada por uma degeneração primária do corpo caloso, associada com o consumo crônico do etanol. A doença pode, ocasionalmente, ocorrer em pacientes não etilistas cronicamente desnutridos. Uma deficiência de vitaminas do complexo B é considerada como a hipótese etiopatogênica principal, uma vez que muitos pacientes obtiveram uma melhora após a administração desses compostos. Algumas vezes, entretanto, tal resposta terapêutica não foi observada. O diagnóstico definitivo da doença de Marchiafava-Bignami pode ser problemático e depende das características de estudos de neuroimagem, especialmente a ressonância magnética. Seu tratamento, dessa forma, é ainda controverso, com resultados variáveis. Como estão implicados fatores nutricionais, analogamente à encefalopatia de Wernicke, alguns autores recomendam a reposição de vitaminas do complexo B, particularmente da B1. O presente artigo relata a forma aguda da doença de Marchiafava-Bignami em um paciente masculino dependente do álcool, que apresentou discreta melhora após a administração parenteral das vitaminas do complexo B. Como consequência de suas más condições neurológicas e imunológicas, ele desenvolveu infecções pulmonares múltiplas e permaneceu, por longo tempo, na unidade de terapia intensiva. Seu óbito ocorreu por sepse causada por um fungo raro, o Rhodotorula mucilaginosa. O artigo é um relato clínico da evolução desse paciente, com a apresentação de seus dados de neuroimagem, acompanhada por uma revisão sobre doença de Marchiafava-Bignami e sobre as infecções por Rhodotorula dentro da perspectiva da unidade de cuidado intensivo.Marchiafava-Bignami disease is a rare affliction characterized by primary degeneration of the corpus callosum associated with chronic consumption of ethanol. The disease may occasionally occur in patients who are not alcoholics but are chronically

  17. Hypohidrotic ectodermal dysplasia with ankylosis of temporomandibular joint and cleft palate: A rare presentation

    Science.gov (United States)

    Goyal, Manisha; Pradhan, Gaurav; Gupta, Sunita; Kapoor, Seema

    2015-01-01

    The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED) is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate. PMID:25684924

  18. Hypohidrotic ectodermal dysplasia with ankylosis of temporomandibular joint and cleft palate: A rare presentation

    Directory of Open Access Journals (Sweden)

    Manisha Goyal

    2015-01-01

    Full Text Available The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate.

  19. Parotid lipoblastoma in a child: Rare presentation as huge infratemporal mass with cervical extension

    Directory of Open Access Journals (Sweden)

    Anantharajan Natarajan

    2010-01-01

    Full Text Available Lipoblastomas arising within the parotid gland and extending into the infratemporal fossa are very rare. They are common in children < 3 years of age. Access to the tumour requires careful planning and interpretation of imaging studies. Lipoblastomas tend to recur. Meticulous dissection of the tumour is needed to prevent recurrence. By adopting the appropriate approach, complete removal is possible with minimal morbidity.

  20. Testing the Application for Analyzing Structured Entities

    OpenAIRE

    Ion IVAN; Bogdan VINTILA

    2011-01-01

    The paper presents the testing process of the application for the analysis of structured text entities. The structured entities are presented. Quality characteristics of structured entities are identified and analyzed. The design and building processes are presented. Rules for building structured entities are described. The steps of building the application for the analysis of structured text entities are presented. The objective of the testing process is defined. Ways of testing the applicat...

  1. The rare presentations of a large polyp and an esophageal carcinoma in heterotropic gastric mucosa: a case series

    Directory of Open Access Journals (Sweden)

    Alagozlu Hakan

    2007-11-01

    Full Text Available Abstract Background Heterotopic gastric mucosa (HGM is commonly seen in the upper esophagus during endoscopyand is generally considered a benign disease. A hyperplastic polyp and an adenocarcinoma arising in heterotopic gastric mucosa are quite rare occurences. Case presentations We present two cases: The first is a patient who suffered from dysphagia because of a large hyperplastic polyp that arose from HGM; the polyp was excised endoscopically. Secondly, we report a rare case of adenocarcinoma arising in HGM of the cervical esophagus. Conclusion Morphologic changes or malignant transformation can develop in the inlet patch. Therefore, gastroenterologists should be aware of the possibility of HGM just distal to the upper esophageal sphincter.

  2. The prune belly syndrome in a female foetus with urorectal septum malformation sequence: a case report on a rare entity with an unusual association.

    Science.gov (United States)

    Goswami, Dibyajyoti; Kusre, Giriraj; Dutta, Hemonta Kumar; Sarma, Adity

    2013-08-01

    The prune belly syndrome is a rare congenital anomaly which is characterized by the triad of an absent or a deficient development of the abdominal muscle, bilateral cryptorchidism and an anomalous urinary tract. In its full form, this condition occurs only in males. However, a similar condition occurs in females in the absence of cryptorchidism. On the other hand, the urorectal septum malformation sequence is a lethal congenital malformation which is characterized by the development of a phallus like structure, a smooth perineum and the absence of urethral, vaginal and anal openings. We are reporting a case of a female foetus with the prune belly syndrome, which was associated with a urorectal septum malformation sequence. A dead foetus with a protruded abdomen and ambiguous genitalia, was born at 32 weeks of pregnancy. On autopsy, it was found to have female internal genital organs. The left kidney, the urinary bladder and the rectum were absent. The sigmoid colon, the ureters and the fallopian tubes opened into a common cloacal sac. The histopathological examination of the ovary showed the presence of Leydig's cells. The occurrence of the female counterpart of the prune belly syndrome is extremely rare and only few of such cases were found to be discussed in the details in the indexed English literature so far. Hence, we hope that this case report will contribute to the existing knowledge on the prune belly syndrome.

  3. Rare Presentation of Metastatic Cystic Trophoblastic Tumor in a Patient Without Prior Chemotherapy

    Directory of Open Access Journals (Sweden)

    Michael L. Wang

    2017-07-01

    Full Text Available Cystic trophoblastic tumor (CTT is a rare testicular germ cell tumor (GCT predominantly seen in post-chemotherapy patients. It is prognostically similar to teratoma and requires no additional chemotherapy in the absence of a nonteratomatous GCT component. We report a case of metastatic CTT in a patient with primary testicular teratoma without prior chemotherapy. Retroperitoneal lymph node metastases contained teratoma, embryonal carcinoma, and CTT. The CTT was β-hCG positive and SALL4 negative by immunohistochemistry (IHC. CTT can arise in metastatic testicular GCT in treatment naïve patients. An important differential diagnosis is choriocarcinoma due to treatment implications, and SALL4 IHC may help.

  4. Hypertriglyceridemia-induced acute pancreatitis with diabetic ketoacidosis: A rare presentation of type 1 diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Prabhat Kumar

    2017-01-01

    Full Text Available Diabetic ketoacidosis (DKA is a frequently encountered complication of diabetes mellitus. DKA is an insulin deficit state and results in moderate to severe hypertriglyceridemia (HTG. HTG is the third leading cause of acute pancreatitis (AP and often goes unnoticed. The triad of DKA, HTG, and AP is rarely seen, and literature on the same is sparse. We report a case of AP which was due to DKA-induced secondary HTG in an adult with previously undiagnosed type 1 diabetes. His HbA1c was significantly raised, and C-peptide level was low, confirming chronic hyperglycemia. He was treated successfully with insulin infusion, intravenous crystalloid, and analgesics.

  5. Stiff Person Syndrome: A Rare Neurological Disorder, Heterogeneous in Clinical Presentation and Not Easy to Treat

    Directory of Open Access Journals (Sweden)

    Susanne Buechner

    2015-01-01

    Full Text Available Background. Stiff person syndrome (SPS is a rare neurological disorder characterized by progressive rigidity of axial and limb muscles associated with painful spasms. SPS can be classified into classic SPS, paraneoplastic SPS, and SPS variants. Its underlying pathogenesis is probably autoimmune, as in most cases antibodies against glutamic acid decarboxylase (GAD are observed. Similarly, paraneoplastic SPS is usually linked to anti-amphiphysin antibodies. Treatment is based on drugs enhancing gamma-aminobutyric acid (GABA transmission and immunomodulatory agents. Case Series. Patient 1 is a 45-year-old male affected by the classic SPS, Patient 2 is a 73-year-old male affected by paraneoplastic SPS, and Patient 3 is a 68-year-old male affected by the stiff limb syndrome, a SPS variant where symptoms are confined to the limbs. Symptoms, diagnostic findings, and clinical course were extremely variable in the three patients, and treatment was often unsatisfactory and not well tolerated, thus reducing patient compliance. Clinical manifestations also included some unusual features such as recurrent vomiting and progressive dysarthria. Conclusions. SPS is a rare disorder that causes significant disability. Because of its extensive clinical variability, a multitask and personalized treatment is indicated. A clearer understanding of uncommon clinical features and better-tolerated therapeutic strategies are still needed.

  6. Rare acquired hemostatic disorders as a cause of prolonged bleeding – presentation of two case reports

    Directory of Open Access Journals (Sweden)

    Polona Novak

    2011-10-01

    Full Text Available BACKGROUNDPatient’s anamnesis is of primary importance in determining hemostatic disorders. Based on anamnestic data, a clinician may decide for further laboratory tests. We must consider an acquired bleeding disorder in a patient with unusual, unexpected and prolonged bleeding episodes. In this article we will describe two rare acquired hemostatic disordes.TWO CASE REPORTSOur first patient had prolonged bleeding after a pacemaker implantation. We diagnosed him with acquired von Willebrand syndrome. Further on, the patient required a planned surgical procedure. In our second case we describe a patient with unusual and excessive skin bruising and prolonged bleeding after teeth extractions. He was diagnosed with acquired hemophilia.CONCLUSIONIn the assessment of a patient with a potential acquired bleeding disorder we must first rule out the most common causes, such as iatrogenic ones. But, because of high morbidity and mortality rates, we must also be aware of some rare acquired bleeding disorders. In case of uncertainty, we should consult with a hematologist.

  7. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

    OpenAIRE

    Foray, Nathalie; Hudali, Tamer; Papireddy, Muralidhar; Gao, John

    2016-01-01

    Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical hist...

  8. A Rare Presentation of the Syndrome of Inappropriate Antidiuretic Hormone in a 12-Year-Old Girl as the Initial Presentation of an Immature Ovarian Teratoma.

    Science.gov (United States)

    Iqbal, Anoop Mohamed; Schwenk, W Frederick

    2018-02-01

    Immature ovarian teratoma is very rare in childhood. We report on a 12-year-old girl with immature ovarian teratoma who presented initially with syndrome of inappropriate antidiuretic hormone. A 12-year-old girl presented with acute abdomen and distention. Initial laboratory tests showed hyponatremia (sodium, 123 mmol/L), that did not respond to fluid management. Computed tomography imaging showed a 15 cm × 9 cm × 20 cm mass in the right ovary with multifocal internal fat, and dystrophic calcifications. She underwent exploratory laparotomy with a right salpingo-oophorectomy, omentectomy, and peritoneal stripping. The pathology revealed metastatic immature teratoma. Hyponatremia resolved soon after the surgery. Although a rare diagnosis, immature ovarian teratoma must be considered in a girl who presents with abdominal mass and hyponatremia. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  9. Intracystic papillary carcinoma in a male as a rare presentation of breast cancer: a case report and literature review.

    LENUS (Irish Health Repository)

    Romics, Laszlo

    2009-01-01

    The term "intracystic papillary ductal carcinoma in situ" has recently changed and is now more appropriately referred to "intracystic papillary carcinoma". Intracystic papillary carcinoma in men is an extremely rare disease with only a few case presentations published in the literature so far.

  10. Rare presentation of a testicular angiofibroma treated with testis sparing surgery.

    Science.gov (United States)

    Leone, Luca; Fulvi, Paola; Sbrollini, Giulia; Filosa, Alessandra; Caraceni, Enrico; Marronaro, Angelo; Galosi, Andrea B

    2016-12-30

    Testicular benign tumors are very rare (< 5%). Testicular Angiofibroma (AF) is one of those, however the gold standard of treatment and follow-up is still unclear. A 47 years-old man with only one functioning testis was referred to our clinic for a palpable right testicular mass and atrophic contralateral testis. Patient underwent testis-sparing surgery with inguinal approach and intraoperative frozen sections examination with diagnosis of AF. Final histology confirmed AF. Post-operative follow-up was uneventful. Clinical and ultrasonographic follow-up was negative after 8 months. We report a conservative surgery in a patient with AF of the solitary testis. AF is a benign para-testicular fibrous neoplasm that could be misinterpreted as malignant tumor and treated with orchiectomy. Testis-sparing surgery is recommended in this case with intraoperative pathological examination. The excision of the mass is enough but in front of a possible recurrence a long follow-up is advisable.

  11. A rare case of Turner′s syndrome presenting with Mullerian agenesis

    Directory of Open Access Journals (Sweden)

    Suresh Vaddadi

    2013-01-01

    Full Text Available Turner′s syndrome also called as Ullrich Turner′s syndrome, is a disease of unclear pathogenesis characterized by complete or partial absence of one sex chromosome, with or without cell line mosaicism in a phenotypic female with short stature. Various anomalies result in a constellation of features, of which the most disturbing is primary amenorrhea due to gonadal dysgenesis. Hormone therapy in these patients can often result in successful menstruation, and scope for subsequent pregnancy because of anatomically normal uterus and vagina. Coexisting Mullerian agenesis in these patients can jeopardize the chances of future pregnancy as they have associated structural abnormalities of the uterus and vagina. We report a rare case of middle-aged female with Turner′s syndrome and Mullerian agenesis having absent secondary sexual characters and missing uterus with incompletely formed vagina.

  12. Mucoepidermoid carcinoma of the posterior-lateral border of tongue: a rare presentation.

    Science.gov (United States)

    Mathew, Lisha; Janardhanan, Mahija; Suresh, Rakesh; Savithri, Vindhya

    2017-10-24

    Mucoepidermoid carcinoma (MEC) is the most common malignant tumour of the major and minor salivary glands. Minor salivary glands are scattered in different areas of the oral cavity such as palate, retromolar area, floor of the mouth, buccal mucosa, lips and tongue, but so far, only a few lingual MEC cases have been documented in the literature and most of the studies have shown a predilection for base and dorsum of the tongue. We report a rare case of MEC involving the posterior-lateral border of the tongue. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  13. The irreducible floating hip: a unique presentation of a rare injury.

    Science.gov (United States)

    Tiedeken, Nathan C; Saldanha, Vilas; Handal, John; Raphael, James

    2013-10-04

    A floating hip injury occurs in the setting of poly-trauma and is a rare and difficult problem to manage. Floating hip injuries require vigilant attention not only to the osseous injuries but also the surrounding compartments and soft tissue envelope. We report the case of a 35-year-old male with a lower extremity posterior wall acetabular fracture, ipsilateral femoral shaft fracture and a postero-superior hip dislocation. Closed reduction failed, necessitating an open reduction internal fixation of his hip dislocation and acetabular fracture. The patient then developed a thigh compartment syndrome requiring a fasciotomy. Despite the obvious bony injuries, orthopedic surgeons must be vigilant of the neurovascular structures and soft tissues that have absorbed a great amount of force. A treatment plan should be formulated based on the status of the overlying soft tissue, fracture pattern and the patient's physiologic stability. Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2013.

  14. Hidradenocarcinoma: A Rare Sweat Gland Neoplasm Presenting as Small Turban Tumor of the Scalp.

    Science.gov (United States)

    Asati, Dinesh P; Brahmachari, Swagata; Kudligi, Chandramohan; Gupta, Chandramohan

    2015-01-01

    Hidradenocarcinomas are very rare malignant sweat gland tumors that possess an infiltrative and/or low metastatic potential. Here we describe an interesting case of hidradenoma on the fronto-parietal region of the scalp of an elderly female, part of which had developed carcinomatous changes, infiltrating up to the pericranium. She developed intense itching, pain, spontaneous ulceration and rapid increase in the size of the tumor correlating with the expression of malignant behavior of the neoplasm. An initial incision biopsy suggested features of benign poroid hidradenoma, while the histology from the excised tumor exhibited a fairly well circumscribed epithelial neoplasm in dermis consisting of interconnected nodules as well as differentiated ducts, the neoplastic cells showing mild pleomorphism of nuclei, mitotic figures and abundant pale cytoplasm. Clefts, sclerotic stroma and foci of necrosis en mass were also seen. The final diagnosis was a well differentiated and slow growing hidradenocarcinoma. The tumor recurred locally despite total excision.

  15. Hidradenocarcinoma: A rare sweat gland neoplasm presenting as small turban tumor of the scalp

    Directory of Open Access Journals (Sweden)

    Dinesh P Asati

    2015-01-01

    Full Text Available Hidradenocarcinomas are very rare malignant sweat gland tumors that possess an infiltrative and/or low metastatic potential. Here we describe an interesting case of hidradenoma on the fronto-parietal region of the scalp of an elderly female, part of which had developed carcinomatous changes, infiltrating up to the pericranium. She developed intense itching, pain, spontaneous ulceration and rapid increase in the size of the tumor correlating with the expression of malignant behavior of the neoplasm. An initial incision biopsy suggested features of benign poroid hidradenoma, while the histology from the excised tumor exhibited a fairly well circumscribed epithelial neoplasm in dermis consisting of interconnected nodules as well as differentiated ducts, the neoplastic cells showing mild pleomorphism of nuclei, mitotic figures and abundant pale cytoplasm. Clefts, sclerotic stroma and foci of necrosis en mass were also seen. The final diagnosis was a well differentiated and slow growing hidradenocarcinoma. The tumor recurred locally despite total excision.

  16. Dynamic collective entity representations for entity ranking

    NARCIS (Netherlands)

    Graus, D.; Tsagkias, M.; Weerkamp, W.; Meij, E.; de Rijke, M.

    2016-01-01

    Entity ranking, i.e., successfully positioning a relevant entity at the top of the ranking for a given query, is inherently difficult due to the potential mismatch between the entity's description in a knowledge base, and the way people refer to the entity when searching for it. To counter this

  17. Entity associations for search

    NARCIS (Netherlands)

    Reinanda, R.

    2017-01-01

    In this thesis, we investigate the broad problem of computing entity associations for search. Specifically, we consider three types of entity association: entity-entity, entity-document, and entity-aspect associations. We touch upon various domains, starting with specific domains such as the

  18. Entity ranking using Wikipedia as a pivot

    NARCIS (Netherlands)

    Kaptein, R.; Serdyukov, P.; de Vries, A.; Kamps, J.; Huang, X.J.; Jones, G.; Koudas, N.; Wu, X.; Collins-Thompson, K.

    2010-01-01

    In this paper we investigate the task of Entity Ranking on the Web. Searchers looking for entities are arguably better served by presenting a ranked list of entities directly, rather than a list of web pages with relevant but also potentially redundant information about these entities. Since

  19. Can pneumocephalus present as flashes of light? A rare case report

    Directory of Open Access Journals (Sweden)

    Santanu Ghosh

    2017-01-01

    Full Text Available Orbital emphysema and associated pneumocephalus usually result from trauma to orbital bones or due to sinus disease, allowing air to travel from paranasal sinus into orbit and brain. However, it is extremely rare to have orbital emphysema and pneumocephalus in the absence of orbital wall fracture. In our case, a young male was admitted with severe eye pain, diminution of vision due to sudden exposure of compressed air gun. Examination revealed proptosed, emphysematous right eye with conjunctival laceration. Computed tomography scan of the head and orbit revealed multiple radiolucencies with air in the right orbit and brain extending up to the spinal canal without any evidence of orbital sinus or cranial bone fracture. Visual acuity recovered completely on follow–up; however, 2 weeks following injury, the patient developed disabling flashes of light which the patient perceived as central in location and resolved finally over a period of 3 months. Flashes of light, in our case, could be attributable to unnoticed damage to cerebral vasculature or connective tissue surrounding the optic nerve due to pneumocephalus.

  20. Rare presentation of a testicular angiofibroma treated with testis sparing surgery

    Directory of Open Access Journals (Sweden)

    Luca Leone

    2016-12-01

    Full Text Available Introduction: Testicular benign tumors are very rare (< 5%. Testicular Angiofibroma (AF is one of those, however the gold standard of treatment and follow-up is still unclear. Case report: A 47 years-old man with only one functioning testis was referred to our clinic for a palpable right testicular mass and atrophic contralateral testis. Patient underwent testis-sparing surgery with inguinal approach and intraoperative frozen sections examination with diagnosis of AF. Final histology confirmed AF. Post-operative follow-up was uneventful. Clinical and ultrasonographic follow-up was negative after 8 months. Conclusion: We report a conservative surgery in a patient with AF of the solitary testis. AF is a benign para-testicular fibrous neoplasm that could be misinterpreted as malignant tumor and treated with orchiectomy. Testis-sparing surgery is recommended in this case with intraoperative pathological examination. The excision of the mass is enough but in front of a possible recurrence a long follow-up is advisable.

  1. Rare adrenal gland incidentaloma: an unusual Ewing's sarcoma family of tumor presentation and literature review.

    Science.gov (United States)

    Guo, Hui; Chen, Shuaiqi; Liu, Shukun; Wang, Kaixuan; Liu, Erpeng; Li, Faping; Hou, Yuchuan

    2017-04-04

    Members of the Ewing's sarcoma family of tumor (ESFT) are malignant neoplasms and rarely observed in the adrenal gland. We report an extremely exceptional case of ESFT rising from the adrenal gland in a 57-year-old Chinese man. The patient was hospitalized with abdominal swelling for 2 months. Computed tomography (CT) scan revealed a nearly-circular mass measuring about 8.1 × 10.6 cm in the right adrenal region. The patient underwent right adrenal resection. Histopathologic examination found the tumor was composed of small round blue cells forming typical Homer-Wright rosettes in focal area. The immunohistochemical analysis confirmed the case to be ESFT, which was positive for membranous CD99 and nuclear FLI-1. The patient was scheduled for four courses of large doses of chemotherapy and died for cancer metastasis one year later after surgery. Histopathological evidence of Homer-Wright rosettes and immunohistochemical markers positivity, such as CD99 and FLI-1, are valuable factors for ESFT diagnosis, although cytogenetic analysis is considered as the gold standard. Complete surgery is the treatment of choice for ESFT and adjuvant radiotherapy and combination chemotherapy can significantly improve the survival rate of postoperative patients.

  2. A paradoxical presentation of rickets and secondary osteomyelitis of the jaw in Type II autosomal dominant osteopetrosis: Rare case reports.

    Science.gov (United States)

    Jayachandran, S; Kumar, M Suresh

    2016-01-01

    Osteopetrosis is a rare genetic bone disorder arising due to a defect in the differentiation or function of osteoclast which results in a generalized increase in bone mass. Osteomyelitis is one of the most common complications because of decreased bone marrow function and compromised blood supply. Radiologist plays a vital role in diagnosing osteopetrosis. Here, we present two cases of autosomal dominant osteopetrosis Type II (ADO II) with secondary osteomyelitis changes which were reported to our department. One of these two cases presented with secondary osteomyelitis in both maxilla and mandible and features of rickets, which is very rarely seen in ADO II. To the best of our knowledge, the presentation of rickets with ADO is the first of its kind to be reported. In this paper, we describe the clinical and radiological features leading to the diagnosis of ADO in these two patients. Further, a review of the literature regarding ADO is discussed.

  3. Primary Amoebic (Naegleria fowleri Meningoencephalitis Presenting as Status Epilepticus

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    A Sharma

    2011-04-01

    Full Text Available Primary amebic meningoencephalitis (PAM is a rare entity. Usual presenting features are fever, headache and seizures with meningeal signs and this disease carries high mortality rate. We present a case report of PAM presenting as status epilepticus.

  4. Immunoglobulin G4-related disease: a rare disease with an unusual presentation.

    Science.gov (United States)

    Khan, Muhammad Waqas; Hadley, Terrance; Kesler, Melissa; Gul, Zartash

    2016-07-01

    IgG4-RD can also present in the skeletal muscle, mimicking several other diseases. It is unusual for this relatively new classification of diseases to present in the muscles and can be mistakenly diagnosed as other autoimmune diseases rendering a delay in the appropriate management and progression of the disease.

  5. A Rare Cause of Acute Kidney Injury in a Female Patient with Breast Cancer Presenting as Renal Colic

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    Roxana Jurubita

    2016-01-01

    Full Text Available Renal infarction is a rare cause of acute kidney injury which could lead to permanent loss of renal function. A prompt diagnosis is necessary in order to achieve a successful revascularization of the occluded artery. Given the rarity of the disease and the paucity of the reported cases in the previous literature a high index of suspicion must be maintained not only in the classical cardiac sources of systemic emboli (atrial fibrillation, dilated cardiomyopathy, or endocarditis, but also in the situations when a hypercoagulable state is presumed. The unspecific presenting symptoms often mask the true etiology of the patient’s complaints. We present here a rare case of renal infarction that occurred in the setting of a hypercoagulable state, in a female patient with a history of breast cancer and documented hepatic metastases.

  6. Hemi chorea hemiballism syndrome: the first presentation of type 2 diabetes mellitus as a rare cause of chorea

    International Nuclear Information System (INIS)

    Mittal, P.

    2011-01-01

    Hemi chorea-hemiballism syndrome, which is most commonly related to non-ketotic hyperglycemia, is a rare type of chorea. Here, we present an unusual case of Hemi chorea-hemiballism syndrome who was not a known case of diabetes. This case highlights the importance of recognising underlying non-ketotic hyperglycemia, as control of hyperglycemia is helpful in the quick relief of symptoms.

  7. Interphalangeal joint involvement of the big toe in gout: a rare presentation.

    Science.gov (United States)

    Dobson, Michael; Alwahab, Yasir; Fazal, Muhammad A

    2012-01-01

    Atypical presentation of gout can cause diagnostic dilemmas. We report a case of gout that presented with an expansile lytic lesion involving the interphalangeal joint of the hallux, lack of a history of gout, and an associated solitary lung nodule. Magnetic resonance imaging showed an expansile destructive bony lesion with soft-tissue involvement suggestive of a possible bony metastasis. A needle biopsy was performed, and histopathologic features were diagnostic of chronic tophaceous gout.

  8. A rare presentation of patent ductus arteriosus in an adult patient with normal pulmonary hypertension and limb edema

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    Bahram Pishgoo

    2014-09-01

    Full Text Available BACKGROUND: Patent ductus arteriosus (PDA at childhood is one of the five major and frequent congenital abnormalities, but it can be rarely seen in adults. Pulmonary hypertension (PHTN and other presentations such as heart failure and edema are the identified complications of longstanding PDA, but adult case with no permanent heart symptoms and PHTN was rare. We reported a rare case of with an obvious PDA and normal pulmonary pressure. CASE REPORT: A 61-year-old woman presented with dyspnea (New York Heart Association class 2, chest pain, and lower limb edema. Echocardiogram showed; normal left ventricular chamber size and function, normal size of both atria. Furthermore, an obvious PDA (diameter = 6-7 mm connecting the aortic arch to the pulmonary artery was reported in echocardiography. No lung congestion and evidence for PHTN was reported by computed tomographic angiography [Pulmonary capillary wedge pressure (PCWP = 30 mmHg]. The patient was treated with antihypertensive drugs and after 1 and 3 months follow-up, edema and other symptoms were resolved. CONCLUSION: Finally, we conclude that PDA in adulthood can present with nonspecific cardiovascular symptoms, and it seems that PHTN is not a fixed echocardiographic finding in these patients.   Keywords: Adults, Edema, Patent Ductus Arteriosus eri, Pulmonary Hypertension  Normal 0 false false false EN-US X-NONE AR-SA

  9. Solitary phalangeal brown tumour in primary hyperparathyroidism: Report of a rare presentation

    International Nuclear Information System (INIS)

    Nagaraj, Chandana; Oommen, Regi; Jacob, Paul M; Irodi, Aparna

    2012-01-01

    Parathyroid adenoma is the most common cause of primary hyperparathyroidism. Severe cases of primary hyperparathyroidism manifest as osteitis fibrosa cystica generalisata, characterized by generalized bone loss with increased bone resorption, including both subperiosteal and endosteal surfaces. The most common sites for formation of fibrotic cystic lesions (brown tumors) are in the long bones and jaw which present as swelling, pathological fracture, and/or bone pain, usually involving multiple sites. Here, we describe an unusual presentation of a solitary brown tumor in a young male who initially presented to the hand surgeon with a history of right thumb swelling following trivial trauma. Further detailed clinical, biochemical, scintigraphic (Tc 99m methylene diphosphonate scintigraphy and Tc 99m Sestamibi scintigraphy), and radiological investigations aided definitive diagnosis and treatment. The causative parathyroid adenoma was excised curing hyperparathyroidism and the lesion regressed substantially

  10. Infantile holocord cellular ependymoma with communicating hydrocephalus: unusual presentation of a rare case.

    Science.gov (United States)

    Aryan, Saritha; Ghosal, Nandita; Aziz, Zarina A; Hegde, A S; Dadlani, Ravi

    2014-01-01

    We present a case of infantile holocord ependymoma in a 4-month-old boy who presented with infection of ventriculoperitoneal shunt done elsewhere for a communicating hydrocephalus. On magnetic resonance imaging, a diffuse holocord T2-hyperintense, T1-hypointense intramedullary bulky lesion with syringomyelia in the cervical level was seen. To the best of our knowledge, this is the first case of infantile holocord ependymoma. As the extent of morbidity associated with a spinal cord tumor is high, an increased level of suspicion and the need for a complete spinal cord screening in a case of infantile hydrocephalus without obvious clinical and radiological evidence of intracranial abnormality is emphasized.

  11. Severe neutropenia revealing a rare presentation of dengue fever: a case report.

    Science.gov (United States)

    Shourick, J; Dinh, A; Matt, M; Salomon, J; Davido, B

    2017-08-17

    Arboviruses are a common cause of fever in the returned traveler often associated with leucopenia, especially lymphopenia and thrombocytopenia. Transient neutropenia has been described in a few cases of arboviruses. However, prolonged and severe neutropenia (dengue fever, especially in the returned traveler in Europe. A 26-year-old healthy female without any medical past history, flying back from Thailand, presented a transient fever with severe neutropenia (dengue fever. Outcome was favorable without any antimicrobial therapy. Physicians should be wary of possible unusual presentation of dengue fever with prolonged neutropenia. Although such biological sign is more often associated with malaria or severe bacterial infection, it may be a sign of arbovirus.

  12. Genitourinary tuberculosis - a rare presentation of a still frequent infection in renal transplant recipients

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    Natacha Jardim Rodrigues

    Full Text Available Abstract Mycobacterium tuberculosis infection in renal transplant recipients is associated with significant morbidity and mortality. Genitourinary tuberculosis is a less frequent presentation and a high level of suspicion is needed to avoid treatment delay. Management is challenging due to the interaction of calcineurin inhibitors with antituberculous medications and the known side effects of these drugs, with higher prevalence in this population. The authors present a case of a renal transplant recipient with urinary and constitutional symptoms whom is diagnosed with tuberculosis after a prostatic biopsy in an already disseminated stage and develops hepatotoxicity to antituberculous therapy.

  13. Human Listeriosis Presenting as Breast Abscess: Report of a Rare Case.

    Science.gov (United States)

    Kandi, Venkataramana

    2017-02-01

    An abscess is defined as a collection of pus in various tissues of the body including skin and other organs. Abscesses most commonly are formed on the skin under the armpits, groin areas, and rectal areas. Most abscesses involve microbial infections with few remaining sterile. The treatment of abscesses includes both medical and surgical intervention. In the era of multidrug resistance, isolation and identification of the causative microbe and testing for antimicrobial susceptible patterns assume greater significance for the better management of patients, thereby reducing the resultant morbidity and mortality. Listeria spp. are a group of aerobic and non-spore forming gram-positive bacilli. They are present in the environment, soil, and water. Listeria spp. have also been noted to be present as a normal intestinal flora of animals. They are known for their ability to thrive under both cold and hot environmental conditions. Human infections with Listeria spp. have not been frequently reported, mostly because of the difficulty in laboratory identification and complex clinical presentations. In humans, Listeria spp. have been frequently responsible for food poisoning and neonatal meningitis. Although not considered as a classic pathogen, Listeria spp. are associated with infections in elderly people, pregnant women, newborns, and persons with weakened immune systems. This report presents a case of breast abscess caused by Listeria spp. in a young lactating female belonging to rural India.

  14. Initial presentation with dilated cardiomyopathy in a patient of tuberous sclerosis: A rare case report

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    Dharmendra Jain

    2013-01-01

    Full Text Available A 35-year-old man presented with dilated cardiomyopathy, an unusual association with tuberous sclerosis. Clinical history and examination were consistent with tuberous sclerosis including major features of tuberous sclerosis complex (TSC like facial angiofibroma, shagreen patch, subependymal nodules, and angiomyolipoma of kidney. The clinical manifestations, pathogenesis and evaluation of tuberous sclerosis are discussed.

  15. The Power of a Soccer Ball: A Traumatic Open Finger Dislocation—A Rare Case Presentation

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    Turan Cihan Dülgeroğlu

    2015-01-01

    Full Text Available Proximal interphalangeal joint dislocations are injuries observed frequently and caused by axial loading on the finger in the extension. In this paper we present a traumatic open finger dislocation due to a ball hitting a wrestler. It was successfully treated with reduction and the volar plate and collateral bond fixation were applied with absorbable sutures.

  16. A rare presentation of multiple endocrine neoplasia (MEN type 2A syndrome

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    Elroy Patrick Weledji

    2016-02-01

    Full Text Available Peptic ulcer disease may be a manifestation of symptomatic primary hyperparathyroidism. A case of an intractable complicated peptic ulcer disease secondary to hypercalcaemia from multiple endocrine neoplasia type 2A is presented. Hypercalcaemia should always be excluded as a cause of recurrent, or complicated peptic ulcer disease.

  17. Management of Surgical Third Lower Molar Extraction and Postoperative Progress in Patients With Factor VII Deficiency: A Clinical Protocol and Focus on This Rare Pathologic Entity.

    Science.gov (United States)

    Passarelli, Pier Carmine; Pasquantonio, Guido; D'Addona, Antonio

    2017-10-01

    The purpose of the present study was to analyze the management of surgical third molar extraction and postoperative progress in patients with a diagnosis of factor VII deficiency. Close collaboration between the oral-maxillofacial surgeon and hematologist will allow the team to categorize the risk and operate safely, thereby minimizing the incidence and severity of intraoperative and postoperative complications. The present retrospective study included 7 patients with factor VII deficiency who had undergone third lower molar surgery. Their factor VII deficiency ranged from 10.5 to 21.0%. Recombinant activated factor VII (rFVIIa) (coagulation factor VIIa [recombinant]; NovoSeven RT; Novo Nordisk, Bagsvaerd, Denmark) was transfused intravenously in a single dose of 25 μg/kg body weight, 30 minutes before surgical extractions. After the surgery, betamethasone, an analgesic, and an ice pack were administered. Pretreatment with recombinant activated factor VII resulted in excellent hemostasis. No hemorrhagic complications and no postoperative major bleeding were observed. The extraction of the third lower molar appears to be a safe procedure for patients with factor VII deficiency when appropriate prophylaxis with rFVIIa is used. Copyright © 2017 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  18. A Rare Case of Mayer-Rokitansky-Kuster-Hauser Syndrome Presenting with Acute Psychosis.

    Science.gov (United States)

    Nath, Kamal; Boro, Bhanita; Naskar, Subrata

    2016-04-01

    The psychiatric co-morbidities in female population with mullerian agenesis is an area with limited research. This is probably due to the fact that when those patients are diagnosed not much attention or information is given for long term psychiatric follow-up. Owing to their inability to bear children, these subjects often become socially harassed. Thus these constant stressors may lead to development of psychopathology in future. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital abnormality with absence of uterus, cervix and vagina, but normal secondary sexual characteristics and external genitalia and occurs in every 1 out of 4000-10,000 females. There is also limited literature on the probable common chromosomal aetiology for both psychosis and MRKH patients. We, present here a case of MRKH syndrome, whose initial presentation was psychosis only. In this respect, we also highlight the much neglected need of appropriate psychiatric screening and provision of psychiatric care in this population.

  19. A Rare Case of Chronic Ectopic Pregnancy Presenting as Large Hematosalpinx

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    Madhavi Nacharaju

    2014-01-01

    Full Text Available Ectopic pregnancy is defined as implantation and subsequent development of an embryo outside the uterine lining. It has wide range of presentation from acute hemoperitoneum to chronic ectopic pregnancy. This is an unusual case of chronic ectopic pregnancy with large hematosalpinx without classical symptoms. A 22-year-old South Indian woman reported to the outpatient clinic with irregular spotting for a duration of 2 months which was not associated with pain. There was no preceding amenorrhea and previous menstrual cycles were regular. Clinically, the patient was hemodynamically stable but severely anemic. The abdomen was soft on palpation, cervical movements were not tender, and human chorionic gonadotropin was absent in the urine. Ultrasound revealed a complex adnexal mass. Magnetic resonance imaging (MRI revealed a large hematosalpinx. Laparoscopic left salpingectomy was conducted and histopathology confirmed ectopic pregnancy. Ectopic pregnancy presents diagnostic dilemmas in the absence of classical symptoms. MRI and laparoscopy are important tools in such a diagnostic dilemma.

  20. A Rare Case of Lateral Canthal Gouty Tophus Presenting as an Eyelid Mass

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    Austin S. Nakatsuka

    2016-01-01

    Full Text Available A 41-year-old man with a history of gout presented with an enlarging eyelid growth. Clinical examination revealed a mildly indurated nodule at the lateral canthus. Following resection, histopathological examination revealed needle-shaped, crystalline material surrounded by multinucleated giant cells, findings consistent with gouty tophus. This represents just the sixth case of gouty tophus of the eyelid reported in the literature.

  1. Concurrent presentation of appendicitis and acute cholecystitis: diagnosis of rare occurrence

    Science.gov (United States)

    Gandhi, Jamish; Tan, Jeffrey

    2015-01-01

    A 67-year-old woman presented with a 2-day history of central abdominal pain migrating to the right upper and lower abdomen. On examination she was normothermic but tachycardic. Inflammatory markers were noted to be elevated with a white cell count of 18.5×109/L and C reactive protein of 265 mg/L. A CT scan revealed dual pathology of appendicitis and acute cholecystitis, which was confirmed intraoperatively and histologically. PMID:26396122

  2. Salmonella pyomyositis with concurrent sacroiliac osteomyelitis presenting as piriformis syndrome: A rare case

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    P S Phadke

    2017-01-01

    Full Text Available A-21-year old male admitted with fever and piriformis syndrome, typically associated with gluteal region pain radiating down the thigh, was evaluated and found to have pyomyositis involving piriformis and osteomyelitis with sacroiliac joint affection on radiological imaging. Salmonella serotype typhi was isolated from blood culture. He was treated with intravenous Ceftriaxone for 6 weeks with signs of recovery documented clinically as well as on imaging studies. Salmonella pyomyositis with osteomyelitis in an immunocompetent patient with no previous hematological or endocrine disorder makes this case an unusual presentation.

  3. Chronic Neck Pain Associated with an Old Odontoid Fracture: A Rare Presentation

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    Mauricio Avila-Guerra

    2013-01-01

    Full Text Available Cervical spine injuries represent a minority of injury cases in motor vehicles accidents but are a real threat to a patient’s life. In the wide range of cervical spine injuries, odontoid (dens fractures represent the most common findings. These fractures are more usually found in the elderly population due to the changes associated with age. Neurological deficit is not frequently found in these injuries. The following is a case presentation of a chronic odontoid fracture with neurological deficit in a young man that was discovered 23 years after he sustained a motor vehicle accident.

  4. Sudden cardiac arrest as a rare presentation of myxedema coma: case report.

    Science.gov (United States)

    Salhan, Divya; Sapkota, Deepak; Verma, Prakash; Kandel, Saroj; Abdulfattah, Omar; Lixon, Antony; Zwenge, Deribe; Schmidt, Frances

    2017-01-01

    Myxedema coma is a decompensated hypothyroidism which occurs due to long-standing, undiagnosed, or untreated hypothyroidism. Untreated hypothyroidism is known to affect almost all organs including the heart. It is associated with a decrease in cardiac output, stroke volume due to decreased myocardial contractility, and an increase in systemic vascular resistance. It can cause cardiac arrhythmias and the most commonly seen conduction abnormalities are sinus bradycardia, heart block, ventricular tachycardia, and torsade de pointes. The authors report a case of an elderly man who presented with sudden cardiac arrest and myxedema coma and who was successfully revived.

  5. Defecation of a colon cast as a rare presentation of acute graft-versus-host disease

    International Nuclear Information System (INIS)

    Al Ashgar, Hamad; Peedikayil, Musthafa; Chaudhri, Naeem; AlGhamdi, Abdulmonem

    2009-01-01

    Diffuse involvement of the gastrointestinal tract by graft versus host disease (GVHD) is a common complication of allogeneic hematopoietic stem cell transplant (HSCT). Gastrointestinal GVHD usually presents 3 or more weeks after HSCT and is characterized by profuse diarrhea, anorexia, nausea, vomiting, abdominal pain and gastrointestinal bleeding. We report a case of a 23 year old male who had undergone allogeneic HSCT and presented with bloody diarrhea on the 90th day post-HSCT. On the fourth day of admission, the patient passed per rectum a 27-cm long pinkish colored fleshy material recognized as a colon cast. Sigmoidoscopy showed a congested and erythematous rectum with the remaining portion of the colon cast attached to the proximal part of the sigmoid colon. A biopsy from the rectal wall was suggestive of grade 4 GVHD. The patient was treated with methylprednisolone, cyclosporine and mycophenolate mofetil, with a partial response (diarrhea and abdominal pain improved), but then he developed multiple other medical complications and died after 3 months. (author)

  6. The evaluation and presentation of a rare case of gonadal dysgenesis

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    Ghafarnejad M

    1996-07-01

    Full Text Available A seventeen-years old woman was presented with infertility and primary amenorrhea. She had normal stature, femate phenotype and normal development of breasts, external genitalia, vagina and cervix. Pelvic examination showed a large lobulated mass. On sonography there was a mass with probable origin of ovary. Paraclinic tests were carried out. Gonadotropins were in postmeno pausal limits. Alpha Fetoprotein, Beta Subunit (B-HCG assay were normal Laparotomy revealed a gonadat mass on right side, normal uterus and left streak gonad. Pathologic report of tumor was dysgerminoma and teratoma. Due to pathology of tumor and Y chromosome, we advised the patient to remove the other streak gonad and have irradiatio

  7. Surfer's myelopathy: a rare presentation in a non-surfing setting and review of the literature.

    Science.gov (United States)

    Maharaj, Monish M; Phan, Kevin; Hariswamy, Soumya; Rao, Prashanth J

    2016-09-01

    Surfers myelopathy can be a rapidly devastating disease and little is known surrounding the pathophysiology of the condition. Although the classical pattern of illness has been well reported, it has never been observed in a non-surfing setting. A 51-year-old demolition worker presented with acute non-traumatic myelopathy. Clinical examination revealed sensory loss to the level of L2. T2-MRI and MRI-DWI revealed a hyperintense signal suggestive of an ischaemic event. A diagnosis of surfer's myelopathy was made and he was commenced on steroid therapy. Following steroid therapy and fluid management the patient was discharged after 6 days with minor anaethesia but significant overall neurological improvement. Diagnosis of SM requires a thorough history, clinical examination and imaging (MRI, MRI-DWI). The patient should be admitted early and investigated. The use of rehabilitation services may be useful if available.

  8. Myxedema Coma due to Hashimoto Thyroiditis: A Rare but Real Presentation of Failure to Thrive in Infancy.

    Science.gov (United States)

    Heksch, Ryan A; Henry, Rohan K

    2018-05-04

    Hashimoto thyroiditis (HT) is uncommon in infancy, and myxedema coma (MC) is even less common. While prior reports have documented these entities separately, to our knowledge, MC in combination with HT has not been reported before in this age group. A 10-month-old female presented with ptosis, lethargy, dysphagia, and failure to thrive (FTT). She developed hypotension, bradycardia, hypothermia, and apnea requiring intubation. Initial thyroid-stimulating hormone was 422 μIU/mL, and free thyroxine was < 0.5 ng/dL, despite the presence of a normal thyroid newborn screen (NBS). Of note, sepsis workup was unremarkable. With the diagnosis of MC, treatment with intravenous levothyroxine was initiated, although after hydrocortisone administration to avert the possibility of an adrenal crisis, despite a random cortisol of 16.4 μg/dL. Based on positive thyroid antibodies suggesting HT, autoimmune workup later revealed positive acetylcholinesterase antibodies consistent with a diagnosis of ocular myasthenia gravis. MC may be a cause of altered mental status in infancy and may simultaneously be associated with FTT on presentation. With the presence of a normal thyroid NBS, autoimmunity should be entertained as the etiology of profound hypothyroidism, as positive thyroid antibodies may prompt an exploration for coexisting diseases which may explain other presenting features. © 2018 S. Karger AG, Basel.

  9. A RARE CASE OF SINUS OF VALSALVA ANEURYSM PRESENTING WITH TRICUSPID STENOSIS AND RIGHT HEART FAILURE

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    P. V. R. S. Subrahmanya Sarma

    2017-12-01

    Full Text Available PRESENTATION OF CASE A female patient of age 48 years came with the complaints of dyspnoea on exertion, no history of orthopnoea or PND attacks. There is history of easy fatigability and mild abdominal distension, since past 3 months. On clinical examination, she is moderately built and nourished. There was no pallor, cyanosis, clubbing, lymphadenopathy, oedema and icterus. Family history was not significant. She was conscious and coherent. Vitals were within the normal limits. Her BP being 120/76 mmHg. She was found to have an elevated JVP up to angle of the mandibule with a prominent "A" wave, and on palpation, there are no thrills or sounds palpable and on auscultation first heart sound and a normal split second heart sounds were heard with no added sounds or murmurs being heard and the presence of free fluid in the abdomen was confirmed. Hepatomegaly was also noticed. Clinically, she was thought to have right heart failure. Her ECG showed that she was in atrial fibrillation with controlled ventricular rate.

  10. Extraskeletal myxoid chondrosarcoma presenting as an intradural spinal mass: report of a rare clinical presentation with an emphasis on differential diagnostic considerations

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    Priya Rao

    2014-12-01

    Full Text Available Extraskeletal myxoid chondrosarcoma is a rare soft tissue neoplasm that occurs predominantly in the soft tissues of the lower extremities. Herein we present a case of a 29 year old male who presented with bilateral femoral numbness believed to be the result of prior injury to his back. A magnetic resonance imaging revealed a mass in the T4-T5 epidural space compressing the spinal cord. Laminectomy was performed and the lesion removed piecemeal. The pathology specimen consisted of multiple fragments of dura involved by a myxoid neoplasm with a nodular growth pattern. The tumor cells were arranged in anastomosing cords and strands. Individual tumor cells were small, of uniform size and shape, with small hyperchromatic nuclei and scant eosinophilic cytoplasm. Immunohistochemical stains were performed which showed the tumor cells were diffusely positive for vimentin and focally positive for EMA, S-100 protein and cytokeratin, whereas they were negative for CD34 and CD99. Fluorescence in situ hybridization (FISH studies showed a clonal population of cells with re-arrangement of the EWSR1 locus, confirming the histologic impression of extraskeletal myxoid chondrosarcoma. This is the first report of a case of an extraskeletal myxoid chondrosarcoma arising from the dura, confirmed to have rearrangement of the EWSR1 gene by FISH. There have only been two other cases of dural based extraskeletal myxoid chondrosarcoma reported prior to our case. We also briefly review the published literature and discuss differential diagnostic considerations for this rare tumor.

  11. Cerebral Venous Thrombosis and Venous Infarction: Case Report of a Rare Initial Presentation of Smoker’s Polycythemia

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    Mihir Raval

    2010-11-01

    Full Text Available Introduction: Cerebral venous thrombosis is a rare initial presentation of polycythemia. If diagnosed early, treatment can reduce mortality and morbidity significantly. Often it may present with headache as the only complaint, and thus the diagnosis is likely to be missed. Case Presentation: A medically stable 31-year-old male, a chronic smoker with a ∼17 pack-year history of smoking, was admitted to the emergency room with a 2-week history of gradually worsening, severe, throbbing headache in the occipital region sensitive to light. Initial neurological examination was positive only for some involuntary motor tics of the left leg. Initial laboratory workup showed hemoglobin of 20 g/dl and hematocrit of 56.5%. The carboxyhemoglobin level was normal, but the oxygen dissociation curve was shifted to the left. Further evaluation by MRI and MRA of the brain suggested extensive and complete thrombosis of the superior sagittal sinus, right transverse sinus and right sigmoid sinus with a small venous infarct in the right parafrontal region. Given that the patient first presented with a thrombotic event, workup for primary polycythemia and hypercoagulable disorders was carried out, including JAK2 mutation evaluation, which was negative. This left us with smoking as the only risk factor and possible cause for secondary polycythemia. He improved significantly with phlebotomy and anticoagulation treatment. Conclusion: This case illustrates a rare but severe complication of secondary polycythemia stressing the importance of being aware of the risk of developing cerebral thrombosis in patients with chronic smoking exposure.

  12. A rare case of multiple pituitary adenomas in an adolescent Cushing disease presenting as a vertebral compression fracture

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    Ji-Yeon Song

    2017-09-01

    Full Text Available Cushing disease in children and adolescents, especially with multiple pituitary adenomas (MPAs, is very rare. We report 17-year-old boy with MPAs. He presented with a vertebral compression fracture, weight gain, short stature, headache, and hypertension. On magnetic resonance imaging (MRI, only a left pituitary microadenoma was found. After surgery, transient clinical improvement was observed but headache and hypertension were observed again after 3 months later. Follow-up MRI showed a newly developed right pituitary microadenoma 6 months after the surgery. The need for careful clinical and radiographic follow-up should be emphasized in the search for potential MPAs in patients with persistent Cushing disease.

  13. A Rare Bilateral Presentation of Multiple Dens Invaginatus, Shovel-Shaped Incisor and Talon Cusp With Mesiodens.

    Science.gov (United States)

    Hegde, S; Jain, M; Shubha, A B

    2014-01-01

    The aim of this paper is to describe a unique and unusual case of concomitant appearance of morphological dental anomalies in the maxillary anterior region, along with its management in a patient with no systemic abnormality. This case report describes the clinical and radiographic features of talon cusp, dens invaginatus, shovel-shaped incisors and a supernumerary tooth occurring in a single patient, which is a rare presentation. All 4 permanent maxillary incisors had dens invaginatus, the permanent maxillary canines showed the presence of talon cusps, the permanent maxillary central incisors were shovel-shaped and an erupted mesiodens was also observed. Treatment included restorative, surgical and orthodontic approaches.

  14. A rare case of multiple pituitary adenomas in an adolescent Cushing disease presenting as a vertebral compression fracture.

    Science.gov (United States)

    Song, Ji-Yeon; Mun, Sue-Jean; Sung, Soon-Ki; Hwang, Jae-Yeon; Baik, Seung-Kug; Kim, Jee Yeon; Cheon, Chong-Kun; Kim, Su-Young; Kim, Yoo-Mi

    2017-09-01

    Cushing disease in children and adolescents, especially with multiple pituitary adenomas (MPAs), is very rare. We report 17-year-old boy with MPAs. He presented with a vertebral compression fracture, weight gain, short stature, headache, and hypertension. On magnetic resonance imaging (MRI), only a left pituitary microadenoma was found. After surgery, transient clinical improvement was observed but headache and hypertension were observed again after 3 months later. Follow-up MRI showed a newly developed right pituitary microadenoma 6 months after the surgery. The need for careful clinical and radiographic follow-up should be emphasized in the search for potential MPAs in patients with persistent Cushing disease.

  15. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis.

    Science.gov (United States)

    Foray, Nathalie; Hudali, Tamer; Papireddy, Muralidhar; Gao, John

    2016-01-01

    Background . Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation . A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Renal biopsy revealed crescentic glomerulonephritis (GN) pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV). Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion . Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment.

  16. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

    Directory of Open Access Journals (Sweden)

    Nathalie Foray

    2016-01-01

    Full Text Available Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA. Renal biopsy revealed crescentic glomerulonephritis (GN pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV. Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion. Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment.

  17. A rare case of lateral ovotesticular disorder with Klinefelter syndrome mosaicism 46, XX/47, XXY: An unusual presentation.

    Science.gov (United States)

    Talreja, Shyam M; Banerjee, Indraneel; Yadav, Sher Singh; Tomar, Vinay

    2015-01-01

    Ovotesticular disorder of sex development (OT-DSD) is a rare disorder of sexual differentiation characterized by the presence of both ovarian and testicular tissues in the same individual. It's incidence ranges from 3% to 10% of all disorder of DSD's, and the most common presentation is 46, XX followed by 46, XX/46, XY mosaicism and 46, XY. Klinefelter syndrome (KS) mosaicism 46, XX/47, XXY is extremely rare, and its association with the ovotesticular disorder is even rarer. We report an unusual case of 16-year-old with male habitus who presented with complains of cyclic hematuria. On examination, he had bilateral gynecomastia, unilateral left cryptorchidism, absent facial hair, sparse axillary hair growth, and pubic hair distribution of feminine type. The right testis was of normal size located normally in hemiscrotum and was confirmed by radio imaging. Ultrasonography and magnetic resonance imaging revealed a cystic area behind posterior half of urinary bladder. Chromosomal analysis revealed 46, XX/47, XXY mosaicism of female karyotype and KS. Histopathological report of this left side excised specimen confirmed the structures to be ovary, uterus, and fallopian tube, thus confirming our diagnosis of the lateral ovotesticular disorder. Meticulous workup combined interdisciplinary approach will lead to early diagnosis and resolve timely sex reassignment issues and also prevent consequences arising due to gonadal insufficiency.

  18. Hepatogastric fistula: A rare complication of liver abscess

    Directory of Open Access Journals (Sweden)

    Shrihari Anikhindi

    2015-01-01

    Full Text Available Rupture of amebic liver abscess into stomach is a rare complication. We report a case of a young male presenting with haematemesis due to a rupture of left lobe amebic liver abscess into stomach. We discuss the diagnosis and management of this rare clinical entity.

  19. Tumour lysis syndrome: A rare acute presentation of locally advanced testicular cancer – Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Marcus Chow

    2016-01-01

    Full Text Available Tumour lysis syndrome (TLS is a potentially fatal complication of malignancy or its treatment. This uncommon syndrome comprises laboratory findings of hyperuricaemia, hypocalcaemia, hyperkalaemia and hyperphosphataemia. A literature search revealed a total of eight patients, with testicular cancer, who had TLS. All these patients had metastatic disease. We present a unique case of a 47-year-old gentleman we saw in clinic, who presented with a rapidly growing right groin mass and acute breathlessness, and discuss the diagnosis and management of TLS. TLS is extremely rare in testicular cancer but necessitates the awareness of urologists. TLS can occur spontaneously in testicular malignancy. Cell lysis in a rapidly proliferating germ cell tumour is a possible mechanism. The prompt identification and institution of management for TLS is crucial to improve clinical outcomes.

  20. A rare diagnosis of abdominal pain presentation in the emergency department: Idiopathic omental bleeding: A case report.

    Science.gov (United States)

    Wu, Yen-Hung; Liu, Kuan-Ting; Wen, Chun-Kai

    2017-12-01

    Idiopathic omental bleeding is a rare cause of acute abdomen, with only a few reported cases. It usually presents with abdominal pain and may be life-threatening. As it rarely occurs, it may not be considered initially during patient presentation. A 35-year-old male came to our emergency department with abdominal pain present for around 5 to 6 hours. The patient complained of left upper quadrant abdominal pain after eating breakfast. The only associated symptom was 3 episodes of vomiting up food. Physical examination revealed mild left upper quadrant abdominal tenderness without muscle guarding or rebounding pain. Blood examination showed leukocytosis with neutrophil predominance and C reactive protein elevation. The pain was persistent and relief was not obtained by medication. Computed tomography showed a large lobular-contour homogenous slightly hyperdense lesion without enhancement along the greater curvature of the stomach in the lesser sac. A surgeon was consulted and laparotomy was suggested. Hematoma was found at Morrison pouch, subsplenic fossa, and lesser sac under operation. Laparotomy and ligation for hemostasis. The patient was discharged with stable condition after 7 days of hospitalization. This diagnosis should be considered in patients presenting with epigastric pain and vomiting after eating while in the emergency department because this disease might be life-threatening. This case highlights 2 important learning points. First, idiopathic omental bleeding could occur after eating in patients without underlying disease or trauma history, and this disease should be taken into consideration when acute abdomen occurs. Second, emergent laparotomy is indicated if the cause of acute abdomen is not clear. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  1. Primary tubercular abscess of the breast – an unusual entity

    Science.gov (United States)

    Gupta, R; Singal, RP; Gupta, A; Singal, S; Shahi, SR; Singal, R

    2012-01-01

    Primary breast tuberculosis manifested as abscess is a rare entity. We are reporting a case of primary breast tuberculosis, which presented as breast abscess. Abscess was drained and tissue sent for histopathology. To our surprise, diagnosis came as breast tuberculosis. Aspiration cytology was not done, as it is not a routine test for abscess cases. Patient was put on anti- tubercular drugs. In the follow-up of 6 months, she was asymptomatic and advised to continue medicine. PMID:22574095

  2. Primary tubercular abscess of the breast--an unusual entity.

    Science.gov (United States)

    Gupta, R; Singal, R P; Gupta, A; Singal, S; Shahi, S R; Singal, R

    2012-02-22

    Primary breast tuberculosis manifested as abscess is a rare entity. We are reporting a case of primary breast tuberculosis, which presented as breast abscess. Abscess was drained and tissue sent for histopathology. To our surprise, diagnosis came as breast tuberculosis. Aspiration cytology was not done, as it is not a routine test for abscess cases. Patient was put on anti- tubercular drugs. In the follow-up of 6 months, she was asymptomatic and advised to continue medicine.

  3. Entity Ranking using Wikipedia as a Pivot

    NARCIS (Netherlands)

    R. Kaptein; P. Serdyukov; A.P. de Vries (Arjen); J. Kamps

    2010-01-01

    htmlabstractIn this paper we investigate the task of Entity Ranking on the Web. Searchers looking for entities are arguably better served by presenting a ranked list of entities directly, rather than a list of web pages with relevant but also potentially redundant information about

  4. Ischemic neuropathy and rhabdomyolysis as presenting symptoms of postpartum cardiomyopathy

    NARCIS (Netherlands)

    Helmich, Rick C. G.; van Laarhoven, Hanneke W. M.; Schoonderwaldt, Hennie C.; Janssen, Mirian C. H.

    2009-01-01

    Rhabdomyolysis and peripheral neuropathy are two distinct disease entities which are rarely encountered in combination. We present a woman with rhabdomyolysis and peripheral neuropathy 3 weeks postpartum. Her symptoms were caused by bilateral femoral artery thrombosis due to postpartum

  5. Meningoradiculitis due to borreliosis presenting as low back pain only

    International Nuclear Information System (INIS)

    Demaerel, P.; Crevits, I.; Baert, A.L.; Casteels-Van Daele, M.

    1998-01-01

    We report a child with Borrelia burgdorferi meningoradiculitis. This entity, also known as Bannwarth syndrome, is rare and its presentation with low back pain only is even more unusual. The MRI findings can suggest the diagnosis. (orig.)

  6. Pancreatitis and myocarditis followed by pulmonary hemorrhage, a rare presentation of leptospirosis- A case report and literature survey

    Directory of Open Access Journals (Sweden)

    Ranawaka Nuwan

    2013-01-01

    Full Text Available Abstract Background Leptospirosis is a potentially fatal disease which can cause multi-organ dysfunction. It can rarely present as acute pancreatitis. This is the first ever report of leptospirosis presenting with acute pancreatitis and myocarditis followed by diffuse pulmonary hemorrhages to the best of our knowledge. Case presentation A 15-year-old South Asian boy presented with high grade fever, epigastric discomfort and was anicteric on admission. He developed tachycardia, transient hypotension, changes of electro-cardiogram and positive troponin I suggestive of myocarditis. Acute pancreatitis was diagnosed with 12 fold high serum amylase and with the evidence of computerized tomography. Then he developed diffuse pulmonary hemorrhages and later acute renal failure. Leptospirosis was confirmed by positive leptospira IgM, negative IgG and strongly positive Microscopic Agglutination Test. Other possible infective and autoimmune causes were excluded. Patient recovered completely with antibiotics and the supportive care. Conclusion This case illustrates diagnostic difficulties especially in resource poor settings where leptospirosis is common. Additionally it highlights the fact that leptospirosis should be considered in patients presenting with pancreatitis which can be complicated with myocarditis and diffuse pulmonary hemorrhages. We hypothesize that Toll like receptors may play a role in such systemic involvement.

  7. Severe Anemia with Hemoperitoneum as a First Presentation for Multinodular Hepatocellular Carcinoma: A Rare Event in Western Countries

    Directory of Open Access Journals (Sweden)

    Thein Swe

    2016-01-01

    Full Text Available Hemoperitoneum due to spontaneous rupture of hepatocellular carcinoma is a life-threatening and rare condition in western countries with an incidence of less than 3% because of early detection of cirrhosis and neoplasm. Here, we describe a case of a 66-year-old male patient with altered mental status with hemorrhagic shock. Computed tomography scan of abdomen revealed hemoperitoneum and mass in liver. Patient underwent resection of liver tumor and biopsy revealed multinodular hepatocellular carcinoma. A high degree of suspicion is required where severe anemia and hemoperitoneum can be a first presentation for hepatocellular carcinoma especially in patients with chronic hepatitis C infection. Early diagnosis is crucial since mortality rates remain high for untreated cases.

  8. Case report: A rare case of focal myositis presenting as Sartorius muscle contracture: A case report and review of literature.

    Science.gov (United States)

    Wang, Jin; Jiao, Juyang; Zhao, Guanglei; Shi, Jingsheng; Xia, Jun

    2018-05-01

    Focal myositis (FM) is a very rare myopathy of unknown etiology characterized by focal enlargement within one single skeletal muscle. In particular, it occurs only involving the Sartorius muscle has never been reported. A 25-year-old man was admitted to the hospital with progressive restricted left hip joint extension, left thigh discomfort and gait disturbance for 6 years. Combining clinical manifestations with results of radiological and pathological examinations, it was consistent with the diagnosis of FM INTERVENTIONS:: The patient received a surgery under general anesthesia to release the contracted Sartorius tendon. The range of motion of the patient's left hip and ipsilateral knee has significantly improved as well as the discomfort of his left thigh relieved obviously after the surgery. This case report is the first to report FM presenting as sartorius muscle contracture and the surgery is an alternative therapy for these patients.

  9. Unusual Presentation Of Idiopathic Retroperitoneal Fibrosis: Case ...

    African Journals Online (AJOL)

    Idiopathic retroperitoneal fibrosis (IRF) is an uncommon entity described as progressive proliferation of connective tissues leading to a fibrous plaque-like lesions that encases the aorta and inferior vena cava inferior to the level of the renal arteries. Mass forming retroperitoneal fibrosis is rare. We present a rare case of a ...

  10. Epistaxis as a Common Presenting Symptom of Glanzmann’s Thrombasthenia, a Rare Qualitative Platelet Disorder: Illustrative Case Examples

    Directory of Open Access Journals (Sweden)

    Michael Recht

    2017-01-01

    Full Text Available Children often present to emergency departments (EDs with uncontrollable nose bleeding. Although usually due to benign etiologies, epistaxis may be the presenting symptom of an inherited bleeding disorder. Whereas most bleeding disorders are detected through standard hematologic assessments, diagnosing rare platelet function disorders may be challenging. Here we present two case reports and review diagnostic and management challenges of platelet function disorders with a focus on Glanzmann’s thrombasthenia (GT. Patient 1 was a 4-year-old boy with uncontrolled epistaxis. His medical history included frequent and easy bruising. Previous laboratory evaluation revealed only mild microcytic anemia. An otolaryngologist stopped the bleeding, and referral to a pediatric hematologist led to the definitive diagnosis of GT. Patient 2 was a 2.5-year-old girl with severe epistaxis and a history of milder recurrent epistaxis. She had a bruise on her abdomen with a palpable hematoma and many scattered petechiae. Previous assessments revealed no demonstrable hemostatic anomalies. Platelet aggregation studies were performed following referral to a pediatric hematologist, leading to the diagnosis of GT. As evidenced by these cases, the ED physician may often be the first to evaluate severe or recurrent epistaxis and should recognize indications for coagulation testing and hematology consultation/referral for advanced hematologic assessments.

  11. Periocular xanthogranuloma: A forgotten entity?

    Directory of Open Access Journals (Sweden)

    Charalampos Papagoras

    2010-03-01

    Full Text Available Charalampos Papagoras1, George Kitsos2, Paraskevi V Voulgari1, Anastasia K Zikou3, Maria I Argyropoulou3, Aikaterini Zioga4, Alexandros A Drosos11Rheumatology Clinic, Department of Internal Medicine; 2Department of Ophthalmology; 3Department of Clinical Imaging and Radiology, 4Department of Pathology, Medical School, University of Ioannina, Ioannina, GreeceAbstract: Periocular xanthogranulomatous diseases are a rare group of disorders which are characterized by a predilection to affect the orbit and ocular adnexa and special histopathological features, in particular infiltrates comprising non-Langerhans-derived foamy histiocytes and Touton giant cells. The differential diagnosis is difficult and occasionally definite diagnosis cannot be established even after clinical and histopathological findings are taken together. We describe a case of a middle-aged man who presented with a 10-year history of voluminous eyelid swelling with concomitant late-onset atopic manifestations, namely bronchial asthma and allergic rhinitis with nasal polyps. After thorough clinical and laboratory investigation, including a biopsy of the eyelid, we classified the patient’s disease to a rare entity that has been relatively recently described: periocular xanthogranuloma associated with adult-onset asthma. In a review of the literature, no prospective trials concerning the treatment of this disease were found. The literature mainly contained case reports and case series in which corticosteroids and chemotherapy with alkylating agents have been reported to be beneficial. We treated our patient with a combination of oral corticosteroids and cyclophosphamide pulses and we observed substantial regression of the eyelid masses together with a normalization of systemic immunologic abnormalities.Keywords: periocular xanthogranuloma, adult-onset asthma, non-Langerhans histiocytoses, cyclophosphamide, methylprednisolone

  12. Unusual Clinical Presentation of a Rare Type of Breast Malignancy: A Case Report and a Short Review of Literature

    Directory of Open Access Journals (Sweden)

    Nadeesha J Nawarathna

    2015-01-01

    Full Text Available Primary extra osseous osteogenic sarcoma is one of the rarest forms of malignant tumor of the breast. It can arise as a result of osseous metaplasia of a preexisting neoplasm or from a none-phyllodes sarcoma of a previously normal breast. Due to its rarity, natural history and optimal treatment methods remain unclear. A 60-year-old patient presented to the surgical casualty ward with large breast abscess. Abscess wall histology revealed an osteosarcoma of the breast. Left total mastectomy with axillary clearance was performed. Histology and subsequent imunohistochemical studies confirmed the diagnosis of osteogenic sarcoma without lymph nodal metastasis. The patient was referred to the oncologist for further management. Rare types of breast tumors could present as breast abscess. Incision and drainage, together with wall biopsy, helped to exclude associated sinister pathologies. Diagnosis of primary osteosarcoma of the breast was made using histological and immunohistochemical findings once the possible primary from the sternum and ribs were excluded. Treatment was similar to sarcomas affecting other locations and should comprise a multidisciplinary approach.

  13. Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Irappa Madabhavi

    2015-01-01

    Full Text Available Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH demonstrated the t(X:18 translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

  14. Guyon's canal syndrome due to tortuous ulnar artery with DeQuervain stenosing tenosynovitis, ligamentous injuries and dorsal intercalated segmental instability syndrome, a rare presentation: a case report

    OpenAIRE

    Zeeshan, Muhammad; Ahmed, Farhan; Kanwal, Darakhshan; Khalid, Qazi Saad Bin; Ahmed, Muhammad Nadeem

    2009-01-01

    The Guyon's canal syndrome is a well known clinical entity and may have significant impact on patient's quality of life. We report a case of 43-year-old male who presented with complaints of pain and numbness in right hand and difficulty in writing for past one month. On imaging diagnosis of Guyon's canal syndrome because of tortuous ulnar artery was made with additional findings of DeQuervain's stenosing tenosynovitis and dorsal intercalated segmental instability syndrome with ligamentous in...

  15. THREE PORTS LAPAROSCOPIC REPAIR OF ADULT MORGAGNI HERNIA AND RARE SIMULTANEOUS PRESENTATION OF PARA-ESOPHAGEAL HERNIA WITH MORGAGNI HERNIA

    Directory of Open Access Journals (Sweden)

    Salman Assad

    2018-05-01

    Full Text Available We report two case reports of Morgagni hernia repair. Our first case was on 65 year old white male who presented with abdominal pain in right upper quadrant and right side of chest for last 3 days. He was having 3 episodes of dark appearing vomiting associated with pain. He also had two episodes of hematemesis. Patient had gastric outlet obstruction with severe distension of stomach because of incarcerated small bowel and colon in the right sided anterior diaphragmatic Morgagni hernia. Laparoscopic repair of incarcerated Morgagni hernia under general anesthesia was planned. We report our second case on rare simultaneous presentation of Morgagni Hernia with type 3 Para esophageal hernia. 60 years old female patient presented in clinic with a follow up of chest discomfort which was progressively increasing with shortness of breath and a chronic gastric reflux. Her vitals were within normal limits and had body mass index (BMI= 29.52kg/m2 (overweight category. Previous past medical history included multiple episodes of gastric regurgitation and cardiovascular intervention for coronary stenting. CT scan showed type 3 paraesophageal hernia (gastro esophageal junction with fundus of stomach displaced above diaphragm. The patient had more than 30% of her stomach incarcerated in the chest as a paraesophageal hernia. The gastro esophageal junction was intra-abdominal after lysis of adhesion. Mesh was placed after posterior crural repair, followed by Nissen fundoplication over a 54 French bougie patient also had an incidental finding of a reducible Morgagni hernia through an anterior defect, followed by a repair without mesh. Esophago-gastro-duodenoscopy showed there was no evidence of any air leak with good valve creation on retroflexion through a fundoplication.

  16. An Atypical Presentation of Congenital Pulmonary Airway Malformation (CPAM): A Rare Case with Antenatal Ultrasound Findings and Review of Literature.

    Science.gov (United States)

    Gautam, Munnangi Satya; Naren Satya, Srinivas M; Prathyusha, Ivvala Sai; Reddy, K Hema Chandra; Mayilvaganan, Kamala Retnam; Raidu, Deepthi

    2017-01-01

    Congenital pulmonary airway malformation (CPAM) is a relatively rare congenital anomaly with a wide spectrum of ultrasound features depending on the specific variety of CPAM. Antenatal ultrasound is a valuable, safe, nonionizing, cost-effective, widely available and easily reproducible imaging tool and is indispensable in the diagnosis of CPAM. In this paper, we aimed to report an atypical imaging presentation of CPAM type II in the second trimester, extensively involving all lobes of the left lung. A 25-year-old G1P0A0 woman with a gestational age of around 22 weeks was referred for an anomaly scan. The antenatal ultrasound scan showed a single, live, intrauterine foetus corresponding to a gestational age of around 22 weeks and 4 days. There were multiple, anechoic structures noted within the pulmonary tissue in the left hemithorax, each measuring around 3 to 4 mm in diameter. The lesion was extending from the left lower lobe up to the apical (apicoposterior) segment of the left upper lobe. The ultrasound diagnosis of congenital pulmonary airway malformation type II was made. After explaining the condition and the poor prognosis to the patient, an informed consent was obtained after she opted for medical termination of pregnancy. Congenital pulmonary airway malformation (CPAM) is an uncommon foetal anomaly with a very wide range of ultrasound appearances depending on the specific type of CPAM. CPAM also has a wide spectrum of differential diagnoses and a variable prognosis. Antenatal ultrasound should always be the primary mode of diagnosis in CPAM.

  17. A Rare Presentation of a Morel-Lavallee Lesion of the Lower Leg Successfully Treated With Ultrasound-Guided Aspiration.

    Science.gov (United States)

    Falconi, Audrey; Crellin, Holly; Tagawa, Chelsea

    2017-07-21

    A Morel-Lavallee lesion (MLL) is a relatively rare condition that is caused by a traumatic shearing force. This force leads to a closed degloving injury of the subcutaneous tissue and fascia that creates a potential space that can fill with lymph, blood, and necrotic fat. The MLLs are traditionally seen after high impact trauma and typically located at the greater trochanter and pelvis, although recent reports have found them to be located at the knee, thigh, and lower leg. The MLLs typically present as swelling at the site of injury, which can be difficult to differentiate from several other diagnoses. This case report discusses an MLL in the lower extremity that occurred during a rugby game. A lack of familiarity with MLLs often leads to delayed diagnosis and treatment. The diagnosis was eventually made with an magnetic resonance imaging, and the lesion was successfully treated with ultrasound-guided aspiration and compression. The athlete was able to return to play without recurrence of the lesion.

  18. Programming Entity Framework

    CERN Document Server

    Lerman, Julia

    2009-01-01

    Programming Entity Framework is a thorough introduction to Microsoft's new core framework for modeling and interacting with data in .NET applications. This highly-acclaimed book not only gives experienced developers a hands-on tour of the Entity Framework and explains its use in a variety of applications, it also provides a deep understanding of its architecture and APIs -- knowledge that will be extremely valuable as you shift to the Entity Framework version in .NET Framework 4.0 and Visual Studio 2010. From the Entity Data Model (EDM) and Object Services to EntityClient and the Metadata Work

  19. Intraosseous verrucous carcinoma arising from an orthokeratinized odontogenic keratocyst: A report of a rarest entity

    Directory of Open Access Journals (Sweden)

    Nagaraju Kamarthi

    2016-01-01

    Full Text Available Intraosseous verrucous carcinomas (VCs arising from odontogenic cysts are a rare entity. An unusual case of a VC arising from the orthokeratinized odontogenic cyst is described for the first time. The microscopic features of the lesion are presented.

  20. THIRD BRANCHIAL CLEFT CYST PRESENTATION IN ADULTHOOD: A CASE REPORT

    OpenAIRE

    Srinjeeta Garg; Yuvraj Patil; Karan Vayangankar; Adip Shetty; Haritosh Kamalakar Velankar

    2014-01-01

    Third branchial cleft cysts (BCCs) are rare entities that represent abnormal persistence of the branchial apparatus. Most cases of third branchial cleft cysts (BCCs) are diagnosed in childhood and show a marked preference for the left side. However, here we present this rare anomaly in a 40 year old female which presented as a fast growing swelling in adulthood.

  1. Gastric Volvulus and Wandering Spleen: A Rare Surgical Emergency

    Directory of Open Access Journals (Sweden)

    Georgios Lianos

    2013-01-01

    Full Text Available Gastric volvulus is a rare but potentially life-threatening clinical entity due to possible gastric necrosis. A wandering spleen may also be associated with gastric volvulus. Patients presenting with the triad epigastralgia, vomiting followed by retching, and difficulty or inability to pass a nasogastric tube into the stomach are likely to have gastric volvulus. The operating surgeon should include this rare entity in the differential diagnosis when dealing with a patient with such a clinical profile. Herein, we present a case of gastric volvulus associated with a wandering spleen in a 28-year-old Caucasian woman and we provide a brief review of the literature on this issue.

  2. Programming Drupal 7 entities

    CERN Document Server

    Michael, Sammy

    2013-01-01

    The book follows a standard tutorial-based approach to create, retrieve, update, and delete Drupal 7 entities, their properties and fields.Programming Drupal 7 Entities is perfect for intermediate or advanced developers new to Drupal entity development who are looking to get a good grounding in how to code using the new paradigm. It's assumed that you will have some experience in PHP development already, and being vaguely familiar with Drupal, GIT, and Drush will also help.

  3. Rare presenting features of carcinoma of stomach – Leptomeningeal carcinomatosis, breast metastasis, malignant pleural effusion, and dermatoses: A report of two cases and review of literature

    Directory of Open Access Journals (Sweden)

    Dhruv Pankaj Mehta

    2017-01-01

    Full Text Available Gastric cancer can have protean manifestations, usual symptoms mimicking those of peptic ulcer disease. Gastric adenocarcinomas have rarely been reported with leptomeningeal carcinomatosis (LMC and dermatoses as initial presenting features. It is also difficult to diagnose breast metastasis of gastric carcinoma due to its rarity. We report two such cases with rare initial presentation. Our first case was a 46-year-old male who presented with LMC. Our second case was a 24-year-old male whose initial complaints were shortness of breath, bilateral breast lumps, and skin discoloration. Both cases on further investigations were found to have primary tumors in the stomach.

  4. Rare presentation of subcapsular hepatic steatosis in a woman with uncontrolled diabetes without peritoneal dialysis: a case report

    OpenAIRE

    Chowdhary, Varun; Pernicka, Jennifer S. Golia; Sharma, Richa

    2016-01-01

    Background Subcapsular hepatic steatosis is a rare atypical pattern of fatty deposition of the liver reported in patients with diabetic nephropathy receiving peritoneal dialysis with intraperitoneal insulin. To date, there has been only one pediatric and zero adult cases of subcapsular hepatic steatosis with no history of continuous ambulatory peritoneal dialysis. We report the first published case of subcapsular hepatic steatosis in an adult diabetic patient without any history of peritoneal...

  5. Logical Entity Level Sentiment Analysis

    DEFF Research Database (Denmark)

    Petersen, Niklas Christoffer; Villadsen, Jørgen

    2017-01-01

    We present a formal logical approach using a combinatory categorial grammar for entity level sentiment analysis that utilizes machine learning techniques for efficient syntactical tagging and performs a deep structural analysis of the syntactical properties of texts in order to yield precise resu...

  6. Bilateral ossification of the auricles: an unusual entity and review of the literature

    Directory of Open Access Journals (Sweden)

    Siabi Vassiliki

    2009-10-01

    Full Text Available Abstract Background True ossification of the auricle with cartilage replacement by bone, is a very rare clinical entity and can result in an entirely rigid auricle. Case presentation We present a rare case of bilateral ossification of the auricles in a 75-years old man with profound progressive rigidity of both auricles. His main complaint was a mild discomfort during resting making sleeping unpleasant without any other serious symptoms. His medical history was significant for predisposing factors for this condition such as, Addison's disease and diabetes mellitus. Excisional biopsy was performed confirming the ossified nature of the auricles. Further treatment deemed unnecessary in our case due to his mild clinical picture. Conclusion True auricular ossification is a quite rare clinical entity with unclear pathogenesis and one should have in mind that there is always the possibility of a serious co-existed disease like endocrinopathy.

  7. Total Pulmonary Artery Atresia Associated with Abnormal Pulmonary Venous Drainage as a Rare Presentation of Scimitar Syndrome

    Directory of Open Access Journals (Sweden)

    Reza Javadrashid

    2013-09-01

    Full Text Available Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex, and variable malformation of the right lung characterized by an abnormal right sided pulmonary drainage into the inferior vena cava, malformation of the right lung, abnormal arterial supply, and sometimes cardiac malformation. Despite the varying degrees of pulmonary hypoplasia and pulmonary artery hypertension, about half of the patients with scimitar syndrome are asymptomatic or mildly symptomatic when the diagnosis is made. Neonates have severe symptoms and worse prognosis while older children come to light because of recurrent respiratory infections, heart murmur, or an abnormal chest radiograph.

  8. Testing the Application for Analyzing Structured Entities

    Directory of Open Access Journals (Sweden)

    Ion IVAN

    2011-01-01

    Full Text Available The paper presents the testing process of the application for the analysis of structured text entities. The structured entities are presented. Quality characteristics of structured entities are identified and analyzed. The design and building processes are presented. Rules for building structured entities are described. The steps of building the application for the analysis of structured text entities are presented. The objective of the testing process is defined. Ways of testing the application on components and as a whole are established. A testing strategy for different objectives is proposed. The behavior of users during the testing period is analyzed. Statistical analysis regarding the behavior of users in processes of infinite resources access are realized.

  9. Double Trouble: A Rare Case of Bilateral Upper Pole Ureteropelvic Junction Obstruction

    Directory of Open Access Journals (Sweden)

    Craig A. Peters

    2014-09-01

    Full Text Available A 16-year-old girl presented with bilateral back pain caused by bilateral upper pole ureteropelvic junction obstructions; an extremely rare phenomenon. Bilateral robotically assisted upper pole pyeloplasties were preformed at the same setting with an excellent clinical response. Although rare, upper pole ureteropelvic junction obstruction is a defined entity that urologists should be aware of.

  10. Primary Tuberculosis of the Appendix: A Rare Cause of a Common ...

    African Journals Online (AJOL)

    Tuberculosis is still a common infection in India. Although the ileocecal region is the most affected part in intestinal tuberculosis, acute tuberculous appendicitis is quite a rare entity. Our case report highlights a rare presentation of tuberculosis and a brief review of literature. Key words: Appendix, granuloma, tuberculosis ...

  11. Programming Entity Framework

    CERN Document Server

    Lerman, Julia

    2010-01-01

    Get a thorough introduction to ADO.NET Entity Framework 4 -- Microsoft's core framework for modeling and interacting with data in .NET applications. The second edition of this acclaimed guide provides a hands-on tour of the framework latest version in Visual Studio 2010 and .NET Framework 4. Not only will you learn how to use EF4 in a variety of applications, you'll also gain a deep understanding of its architecture and APIs. Written by Julia Lerman, the leading independent authority on the framework, Programming Entity Framework covers it all -- from the Entity Data Model and Object Service

  12. Presentations

    International Nuclear Information System (INIS)

    2007-01-01

    The presented materials consist of presentations of international workshop which held in Warsaw from 4 to 5 October 2007. Main subject of the meeting was progress in manufacturing as well as research program development for neutron detector which is planned to be placed at GANIL laboratory and will be used in nuclear spectroscopy research

  13. CNN-based ranking for biomedical entity normalization.

    Science.gov (United States)

    Li, Haodi; Chen, Qingcai; Tang, Buzhou; Wang, Xiaolong; Xu, Hua; Wang, Baohua; Huang, Dong

    2017-10-03

    Most state-of-the-art biomedical entity normalization systems, such as rule-based systems, merely rely on morphological information of entity mentions, but rarely consider their semantic information. In this paper, we introduce a novel convolutional neural network (CNN) architecture that regards biomedical entity normalization as a ranking problem and benefits from semantic information of biomedical entities. The CNN-based ranking method first generates candidates using handcrafted rules, and then ranks the candidates according to their semantic information modeled by CNN as well as their morphological information. Experiments on two benchmark datasets for biomedical entity normalization show that our proposed CNN-based ranking method outperforms traditional rule-based method with state-of-the-art performance. We propose a CNN architecture that regards biomedical entity normalization as a ranking problem. Comparison results show that semantic information is beneficial to biomedical entity normalization and can be well combined with morphological information in our CNN architecture for further improvement.

  14. Primary Ovarian Leiomyoma: An Uncommon Entity ‑ Case Report ...

    African Journals Online (AJOL)

    Journal of Basic and Clinical Reproductive Sciences · January - June 2013 · Vol 2 · Issue 1. 54 ... case report of primary ovarian leiomyoma, which is a rare entity, in a postmenopausal female with ... There were extensive areas of edema.

  15. Uncommon presentation of a rare tumour - incidental finding in an asymptomatic patient: case report and comprehensive review of the literature on intrapericardial solitary fibrous tumours

    OpenAIRE

    Czimbalmos, Csilla; Csecs, Ibolya; Polos, Miklos; Bartha, Elektra; Szucs, Nikolette; Toth, Attila; Maurovich-Horvat, Pal; Becker, David; Sapi, Zoltan; Szabolcs, Zoltan; Merkely, Bela; Vago, Hajnalka

    2017-01-01

    Background A solitary fibrous tumour is a rare, mainly benign spindle cell mesenchymal tumour most commonly originating from the pleura. An intrapericardial location of a solitary fibrous tumour is extremely unusual. We present a case of an asymptomatic patient with a slow-growing massive benign cardiac solitary fibrous tumour. Case presentation A 37-year-old asymptomatic female patient was referred to our hospital with an enlarged cardiac silhouette found on her screening chest X-ray. The ec...

  16. Monilethrix – Case report of a rare disease

    Directory of Open Access Journals (Sweden)

    Tasleem Arif

    2015-01-01

    Full Text Available Monilethrix is a rare genetic disorder of hair characterized by beaded appearance of the hair shaft leading to hair fragility and patchy dystrophic alopecia. In this disorder, the hair shaft has alternate widenings (nodes and constrictions (internodes that lead to fracture of hair shaft and varying degree of alopecia. We report an eight year old Kashmiri boy who presented with diffuse hair loss since infancy. As monilethrix is a rare disease entity which prompted us to report this case.

  17. Aneurysmal bone cyst of maxillary alveolus: A rare case report

    Directory of Open Access Journals (Sweden)

    Subhas Chandra Debnath

    2016-01-01

    Full Text Available Aneurysmal bone cyst (ABC is a nonneoplastic rare pathologic entity of the jaws. Its locally aggressive nature and high recurrence rate after curettage make surgical resection a better treatment option. Here, we present a case of ABC of maxillary alveolus and its management by alveolectomy followed by white head varnish pack application in the surgical defect.

  18. Aneurysmal bone cyst of maxillary alveolus: A rare case report

    Science.gov (United States)

    Debnath, Subhas Chandra; Adhyapok, Apurba Kumar; Hazarika, Kriti; Malik, Kapil; Vatsyayan, Ashutosh

    2016-01-01

    Aneurysmal bone cyst (ABC) is a nonneoplastic rare pathologic entity of the jaws. Its locally aggressive nature and high recurrence rate after curettage make surgical resection a better treatment option. Here, we present a case of ABC of maxillary alveolus and its management by alveolectomy followed by white head varnish pack application in the surgical defect. PMID:27041915

  19. Uterine arteriovenous malformation as a rare cause of genital bleeding

    International Nuclear Information System (INIS)

    Rodriguez, A.; Escartin, I.; Riazuelo, G.; Zaragozano, R.

    2002-01-01

    Uterine arteriovenous malformation is a rarely described entity, the presenting sign of which is usually genital bleeding. We report a case of this malformation in a woman of child-bearing age with a history of traumatic delivery and repeated subsequent curettage, describing the ultrasound, computed tomography and magnetic resonance findings, as well as the results of arteriography. (Author) 5 refs

  20. Congenital Cystic Adenomatoid Malformation of Lung-Rare Case Report

    Directory of Open Access Journals (Sweden)

    N. S. Kamakeri

    2016-10-01

    Full Text Available Congenital cystic adenomatoid malformation of lung associated with Cystic dysplasia of kidney, cystic disease of liver with mixed gonadal dysgenesis is rare and is not reported in literature so far. Hence an attempt is made to present this rarest entity.