Follicular Lymphoma With a Burkitt Translocation—Predictor of an Aggressive Clinical Course: A Case Report and Review of the Literature

2004 ◽  
Vol 128 (2) ◽  
pp. 210-213 ◽  
Author(s):  
Peter M. Voorhees ◽  
Kathryn A. Carder ◽  
Scott V. Smith ◽  
Lanier H. Ayscue ◽  
Kathleen W. Rao ◽  
...  

Abstract Follicular lymphoma is an indolent lymphoma characterized by the (14;18) translocation, which leads to aberrant expression of Bcl-2. Translocations involving 8q24 are most commonly associated with Burkitt lymphoma and result in c-Myc overexpression. We report a case of follicular lymphoma of predominant small cleaved-cell type (grade 1) associated with both a t(14;18)(q32;q21) and a t(8;22)(q24;q11). The 8q24 translocation predicted an aggressive clinical course, as the lymphoma transformed into acute lymphoblastic leukemia within a year of initial diagnosis. Routine cytogenetic analysis is recommended at initial diagnosis of follicular lymphoma to better identify abnormalities that may predict prognosis and influence therapy.

2018 ◽  
Vol 27 (3) ◽  
pp. 328-335
Author(s):  
Hongxing Gui ◽  
Elliott Lhospital ◽  
Arthur P. Staddon ◽  
Suneel N. Nagda ◽  
Eric L. Zager ◽  
...  

Sclerosing rhabdomyosarcoma (RMS) is a rare subtype of RMS with unique prominent stromal hyalinization and a pseudovascular architecture. It overlaps morphologically with spindle cell RMS and poses both diagnostic and therapeutic challenges because of its rarity and aggressive clinical course. In this article, we report a case of sclerosing RMS arising from a prior craniotomy site, which demonstrated both sclerosing and spindle cell components. A literature review of RMS with sclerosing morphology identified 122 cases. Our review documents the following: sclerosing RMS occurs in both childhood and adult populations, has a predilection for the head and neck areas, and has a worse prognosis in adults. Sclerosing RMS harbors a high frequency of MYOD1 mutations, conferring a poor clinical outcome. Sclerosing RMS and spindle RMS likely represent a morphologic spectrum of one entity.


2018 ◽  
Vol 27 (3) ◽  
pp. 322-327 ◽  
Author(s):  
Nicholas J. Olson ◽  
Julianna M. Czum ◽  
Francine B. de Abreu ◽  
Konstantinos Linos ◽  
Candice C. Black

Pulmonary adenofibroma (PAF) is a rare neoplasm that may be related to solitary fibrous tumor (SFT). A subset of PAFs harbor the NAB2-STAT6 fusion that is typical of SFT, but a significant proportion do not. Their distinction is clinically important as SFTs can potentially have an aggressive clinical course, while there has been no report of a PAF behaving in a malignant fashion. We report a case of a 60-year-old male who developed a SFT and PAF in the same lung. The SFT harbored a NAB2-STAT6 fusion, while the PAF did not have any identifiable fusion. This case represents the first instance of a single patient with both of these tumors occurring simultaneously in the same lung.


2017 ◽  
Vol 10 (1) ◽  
pp. 1-7 ◽  
Author(s):  
Madhumati R. Kalavar ◽  
Sami Ali ◽  
Damoun Safarpour ◽  
Saroj Davi Kunnakkat

Renal medullary carcinoma (RMC) is a rare, yet aggressive malignancy of the kidney that is found predominantly in young patients with African descent and sickle cell hemoglobinopathies and most specifically sickle cell trait. Due to its aggressive nature, most cases have metastasis or local invasion at the time of diagnosis. Prognosis is extremely poor with survival less than 1 year after diagnosis. Here we present a case of metastatic RMC in a 29-year-old African female. Despite chemotherapy with cisplatin, gemcitabine, and paclitaxel, and initial shrinkage of the tumor, the patient died 5 months after diagnosis.


2005 ◽  
Vol 38 (1) ◽  
pp. 19-24 ◽  
Author(s):  
Shin Saito ◽  
Yoshinori Hosoya ◽  
Wataru Arai ◽  
Masanobu Hyodo ◽  
Taku Yokoyama ◽  
...  

2003 ◽  
Vol 127 (5) ◽  
pp. 610-613
Author(s):  
Cherie H. Dunphy ◽  
Hendrik W. van Deventer ◽  
Kathryn J. Carder ◽  
Kathleen W. Rao ◽  
Georgette A. Dent

Abstract The translocation t(14;18)(q32;q21) is most commonly associated with follicular lymphoma but has also been described in acute lymphoblastic leukemia (ALL) of B-cell origin. Although these ALL cases have had a pre-pre-B, pre-B, or mature B-cell immunophenotype and L2 or L3 morphology, all have been associated with an abnormality of 8q24. In fact, 91% (10 of 11) have been associated with t(8;22) or t(8;14), marker chromosomes for Burkitt-type ALL. The other case was associated with del(8)(q24). Thus, Burkitt-type ALL may have various immunophenotypes and morphology when associated with t(14;18). We describe a case of mature B-cell ALL associated with t(14;18) and t(8;9)(q24;p13). The morphology was suggestive but not entirely characteristic of the L3 subtype. However, on the basis of the cytogenetic findings and the review of the literature, perhaps this case represents a variant of Burkitt-type ALL, which would be important to recognize for prognostic and therapeutic purposes. We describe our findings and review the literature to heighten awareness of this group of ALLs associated with t(14;18). Additional cases need to be accrued and documented to determine the significance of an associated abnormality of 8q24 in this setting.


1998 ◽  
Vol 77 (1) ◽  
pp. 51-55 ◽  
Author(s):  
Jose A. Bonilla ◽  
Michael P. Pizzuto ◽  
Linda S. Brodsky

Aplasia of the epiglottis is a rare laryngeal anomaly. We present a case of absence of the epiglottis in a child whose clinical course has been followed for nine years. She required a tracheostomy at two years of age for obstructive sleep apnea which resulted in heart failure; she was eventually decannulated at age seven. This case report highlights the clinical challenges faced in the identification and treatment of the sequelae of this defect. Both endoscopic and computed tomography (CT) documentation are provided. Embryological development and a review of the literature are also discussed.


2012 ◽  
Vol 16 (6) ◽  
pp. 438-441 ◽  
Author(s):  
Efstathios Rallis ◽  
Chrysovalantis Korfitis

Background: Granulomatous rosacea is considered a clinical variant of rosacea and is characterized by hard yellow, brown, red or flesh-colored cutaneous papules or nodules that may be severe and may lead to scarring. The lesions typically appear on the cheeks and periorificial lesions; they are monomorphic in each patient and sit on relatively normal-appearing skin. The diagnosis should be established by excluding other granulomatous disorders and rosacea-like eruptions such as sarcoidosis, tuberculosis, and lupus miliaris disseminatus faciei. The clinical course is chronic and unpredictable, and management can be very difficult. Case Report: We report the case of a 28-year-old female with granulomatous rosacea who responded successfully to oral isotretinoin. No recurrence was noticed 6 months after the completion of treatment. Renseignements de base: La rosacée granulomateuse est considérée comme une variante clinique de la rosacée et est caractérisée par la présence de papules cutanées jaunes, brunes, ou fleshed-colored rouges dures ou par de nodules qui peuvent être graves et qui peuvent causer des cicatrices. Les lésions sont généralement localisées aux joues et sur les lésions péri-orificielles; elles sont monomorphes chez chaque patient individuel et elles siègent sur une peau d'apparence relativement normale. Le diagnostic doit être établi en excluant les autres troubles granulomateux et les éruptions rosacéiformes comme la sarcoïdose, la tuberculose, et la tuberculide papulonodulaire. L'évolution clinique est chronique et imprévisible, et la gestion peut être très difficile. Rapport de cas: Nous rapportons le cas d'une femme âgée de 28 ans présentant une rosacée granulomateuse qui a été traité avec succès avec l'isotrétinoïne administrée par voie orale. Aucune récidive n'a été observée 6 mois après la fin du traitement.


2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
Muazez Cevik

The aim of this paper is to stay a very rare umbilico-inguinal fistula (UIF) resulting from a delayed suture reaction after the use of silk suture to repair an inguinal hernia. A 3-year-old boy presented with persistent umbilical discharge. The initial diagnosis was omphalitis and he was treated with broad-spectrum antibiotics but a UIF was subsequently diagnosed. Surgery was performed to ascertain the cause of the UIF. This case demonstrates that silk suture used in inguinal hernia repair can lead to a UIF, which should be considered in the differential diagnosis of a patient presenting with persistent umbilical discharge.


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