scholarly journals Primary Cutaneous Follicle Center Lymphoma of the Eyelid: A Case Report and Review of the Literature in Light of Recent Changes to the WHO Classification of Lymphoid Neoplasms

2018 ◽  
Vol 5 (2) ◽  
pp. 147-152 ◽  
Author(s):  
Astrid C. Werner ◽  
Nora V. Laver ◽  
Dan S. Landmann
Keyword(s):  
Case Report ◽  
Who Classification ◽  
Clinical Stage ◽  
Survival Rates ◽  
Review Of The Literature ◽  
Systemic Involvement ◽  
Lymphoid Neoplasms ◽  
Cutaneous Lymphomas ◽  
Histopathologic Features

Primary cutaneous follicle center lymphoma (PCFCL) is a unique entity that represents up to 11% of all cutaneous lymphomas. PCFCL is associated with an indolent course and excellent 5-year survival rates, but can progress to secondary systemic involvement if left untreated. Histopathologic features of PCFCL can vary depending on the size, duration, and clinical stage of the lesion, making diagnosis somewhat challenging. Here, we present a case of a 50-year-old woman with an eyelid lesion that was initially classified as an inflamed cyst based on biopsy, but 1 year later, was determined to be PCFCL after repeat biopsy revealed different histology. In light of the recent changes to the WHO classification of lymphoid neoplasms, we review the unique clinical and histopathologic features of PCFCL that distinguish it from other more aggressive forms of cutaneous lymphoma in terms of course, prognosis, and management.

scholarly journals Ameloblastic Fibrosarcoma of the Mandible

1969 ◽  
Vol 33 (1) ◽  
pp. 58-60
Author(s):  
Jennifer T. Go ◽  
Jose M. Carnate
Keyword(s):  
Case Report ◽  
Who Classification ◽  
International Agency ◽  
Review Of The Literature ◽  
Patient Age ◽  
Epithelial Component ◽  
Patient Âgé ◽  
Ameloblastic Fibroma ◽  
Ameloblastic Fibrosarcoma

A 32-year old Filipino woman presented with a 3-year history of slowly enlarging left hemimandibular mass with no associated symptoms. Previous biopsy showed ameloblastoma. Imaging revealed a translucent mulitloculated mass with ill-defined borders. (Figure 1) On examination, the mass was irregularly shaped, measures 40 x 39 cm, slightly hyperpigmented and erythematous, warm with visible vessels. The patient underwent left segmental mandibulectomy with reconstruction and the specimen was sent for histopathologic evaluation.   Received was a mandibulectomy specimen weighing 1850 grams and measuring 17 x 14.5 x 14 cm. The body, angle, ramus and condyles of the left hemimandible are no longer identifiable grossly. There are ten teeth attached. Cut sections show multiple cystic spaces which measure from 0.8 to 15.0 cm in widest diameter, filled with abundant red-brown necrotic debris and yellow-brown purulent material. The mass has a grey-tan soft to fibrous cut surface with focal gritty areas. (Figure 2)   Microscopic examination shows a biphasic neoplasm composed of benign epithelial and malignant mesenchymal components. (Figure 3) The benign epithelial component is arranged in islands and strands with peripheral palisading, composed of bland cells without atypia. (Figure 4) The abundant mesenchymal component is composed of spindle cells in fascicles. The cells are moderately pleomorphic with enlarged hyperchromatic nuclei, prominent nucleoli, coarse chromatin and scant cytoplasm. The cells are suspended within loose stroma with variable degree of cellularity. Some mitoses are noted. (Figure 5)   Ameloblastic fibrosarcoma (AFS) belongs to a group of odontogenic sarcomas in which the epithelial component is cytologically benign while the ectomesenchymal component shows malignant features. The AFS is the most common type among the odontogenic sarcomas. It is regarded as the malignant counterpart of ameloblastic fibroma (AF). Although most cases arise de novo, the documentation of a prior or pre-existing precursor lesion from ameloblastic fibroma suggests otherwise.1,2 A study by Kobayashi et al. suggested that up to one-third of AFS arise from AF while a review of literature by Lai et al. demonstrated that 51% of AFS had previously documented AF at the same site, providing supporting evidence of a malignant transformation.3   Ameloblastic fibrosarcoma has a reported age range of 3 - 89 years, with overall mean patient age of 27.3 years. In cases of prior diagnosis of AF, AFS can occur at a mean patient age of 33 years whereas a mean patient age of 23 years where no prior diagnosis of AF was documented.1,4 It mainly affects males in the third or fourth decade of life and the posterior mandible is the most commonly affected site, with ratio of mandibular to maxillary incidence of 4:1.1,3,4  The clinical presentation is usually that of a painful, enlarging mass and most lesions are radiographically translucent and multiloculated with ill-defined borders.2,4   The histologic features of AFS reveal a mixture of benign odontogenic epithelium ranging from budding and branching cords to large islands composed of columnar or cuboidal peripheral cells arranged in palisading pattern, and an abundant malignant mesenchymal component showing marked cellularity, nuclear pleomorphism, and moderate mitotic figures.1,2,3 Ameloblastic fibroma differs from AFS by having no malignant component and immunohistochemical stains have been suggested to provide distinction, particularly when identifying a low-grade fibrosarcoma. The malignant component of AFS will show positivity in p53 and PCNA and will have a higher Ki-67 expression than AF.3,4   Although AFS are low to intermediate-grade sarcomas, a high incidence of recurrence is reported - about one third of patients experience recurrence and overall mortality rate is 25%. However, only less than 5% of cases with metastases have been reported.1,3 Long term follow up is thus indicated.   References             Wright JM. Odontogenic Sarcomas. In: El-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg PJ, editors. WHO Classification of Head and Neck Tumours, WHO Classification of Tumours, 4th Edition, Volume 9. Lyon: International Agency for Research on Cancer IARC; 2017, p. 214.   Servato JPS, De Faria PR, Ribeiro CV, Cardoso SV, Dias FL, Eisenberg ALA, Loyola AM. Ameloblastic fibrosarcoma: a case report and literature review. Braz Dent J 2017 Mar-Apr; 28(2):262-272. DOI: 1590/0103-6440201701050 PMID: 28492759   Loya-Solis A. Gonzalez-Colunga KJ, Perez-Rodriguez CM, Ramirez-Ochoa NS, Cecenas-Falcon L, Barboza-Quintana O. Ameloblastic fibrosarcoma of the mandible: a case report and brief review of the literature. Case Rep Pathol. 2015;2015:245026. Epub 2015 Mar 10 DOI: 1155/2015/245026 PMID: 25861504 PMCID: PMC4377457   Al Shetawi AH, Alpert EH, Buchbinder D, Urken ML. Ameloblastic fibrosarcoma of the mandible: a case report and a review of the literature. J Oral Maxillofac Surg 2015 Aug;73(8):1661.e1-7. Epub 2015 Apr 10. DOI: 1016/j.joms.2015.03.066 PMID: 25921823                

scholarly journals Gliosarcomas: A Rare Case Report at Viet Duc University Hospital and Review of the Literature

2021 ◽  
Vol 37 (2) ◽  
Author(s):  
Nguyen Thi Quynh ◽  
Nguyen Duc Hoan ◽  
Dao Thi Luan ◽  
Nguyen Tung Ngoc ◽  
Nguyen Sy Lanh
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Case Report ◽  
Who Classification ◽  
Biological Behavior ◽  
International Agency ◽  
Review Of The Literature ◽  
The Central Nervous System ◽  
The Brain

Gliosarcoma is a rare biphasic subtype of glioblastoma with the poor prognosis, principally affects adults; males are more frequently affected, with a male-to-female ratio of 1.8/1. Gliosarcomas are usually located in the cerebral hemispheres, involving the temporal, frontal, parietal, and occipital lobes in decreasing order of frequency. Rarely, gliosarcomas occur in the posterior fossa, lateral ventricles, or spinal cord. A case study: A 32-year-old woman presented with persistent nausea and headache. The preoperative diagnosis was Ependymoma in the right lateral ventricle of the brain. The patient underwent surgical resection of the tumor followed by external radiotherapy, and chemotherapy treatment. Histologic description: The tumor was made up of spindle cells with hyperchromic large nuclei and pink cytoplasm intermingled with large cells with markedly pleomorphic nuclei and abundant cytoplasm along with prominent mitotic activity. Tumour cells revealed positive staining for Ki67 (25%), Oligo2 (focal), GFAP (focal), SMA (focal); negative immunoreactivity for EMA, CD34, Bcl-2, TTF1. Pathological diagnosis: Gliosarcoma, grade IV. Conclusions: Gliosarcoma is an extremely rare neoplasm with an aggressive biological behavior. In terms of histopathology, gliosarcomas are characterized by a biphasic tissue pattern with alternating areas displaying glial and mesenchymal differentiation.   Keywords Gliosarcoma, glioblastoma multiforme, brain neoplasm. References [1] World Healh Organization, WHO Classification of Tumors of the Central Nervous System, International Agency for Research on Cancer (IARC) 69372 Lyon Cedex 08, France, 2016. [2] F. A. Hashmi, A. Salim, M. Shamim, M. Bari, Biological Characteristics and Outcomes of Gliosarcoma, The Journal of the Pakistan Medical Association, Vol. 68, No. 8, 2018, pp. 1273-1275. [3] P. Giglio, M. R. Gilbert, Encyclopedia of the Neurological Sciences (Second Edition), MA: Academic Press/Elsevier, Waltham, 2014. [4] R. K. Kevin, M. Anand, S. M. John, Adult Gliosarcoma: Epidemiology, Natural History, and Factors Associated with Outcome, Neuro Oncol, Vol. 11, No. 2, 2009, pp. 183-191, https://doi.org/10.1215/15228517-2008-076. [5] L. Han, X. Zhang, S. Qiu et al., Magnetic Resonance Imaging of Primary Cerebral Gliosarcoma: A Report of 15 Cases, Acta Radiologica, Vol. 49, No.9, 2008, pp. 1058-1067, doi:10.1080/02841850802314796. [6] D. N. Louis, H. Ohgaki, O. D. Wiestler et al., The 2007 WHO Classification of Tumours of the Central Nervous System, Acta Neuropathologica, Vol. 114, No. 2, 2007, pp. 97-109, doi:10.1007/s00401-007-0278-6. [7] L. Seth, P. Arie, I. James et al., Greenfield’s Neuropathology (Ninth Edition), CRC Press, Boca Raton, Florida, 2015. [8] L. Cervoni, P. Celli, Cerebral Gliosarcoma: Prognostic Factors, Neurosurgical Review, Vol. 19, No. 2, 1996, pp. 93-96, https://doi.org/10.1007/bf00418077. [9] J. Pardo, M. Murcia, G. Felip et al., Gliosarcoma: A Rare Primary CNS Tumor. Presentation of Two Cases, Reports of Practical Oncology & Radiotherapy, Vol. 15, No. 4, 2010, pp. 98-102, https://doi.org/10.1016/j.rpor.2010.05.003. [10] J. Lutterbach, R. Guttenberger, A. Pagenstecher, Gliosarcoma: A Clinical Study, Radiotherapy andOncology, Vol. 61, No. 1, 2001, pp. 57-64,https://doi.org/10.1016/S0167-8140(01)00415-7. [11] B. K. Kleinschmidt, T. Tihan, F. Rodriguez, Diagnostic Pathology: Neuropathology (Second Edition), Elsevier, Philadelphia, 2016. [12] H. F. Irwin, W. G. Sidney, Sarcoma Arising in Glioblastoma of the Brain, Am J Pathol, Vol. 31, No. 4, 1955, pp. 633-653. [13] A. S. Awadalla, A. M. A. Essa, H. H. A. Ahmadi et al., Gliosarcoma Case Report and Review of the Literature, The Pan African Medical Journal, Vol. 35, No. 26, 2020, https://doi.org/10.3109/02841869709001353.        

Familial Eruptive Syringomas: Case Report and Review of the Literature

2013 ◽  
Vol 17 (2) ◽  
pp. 84-88 ◽  
Author(s):  
Jenny Lau ◽  
Richard M. Haber
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Rare Clinical Entity ◽  
Benign Neoplasms ◽  
Review Of The Literature ◽  
Causative Gene ◽  
Unusual Condition ◽  
Clinical Variant ◽  
Insight Into

Background: Syringomas are benign neoplasms of eccrine origin. A clinical variant is eruptive syringomas, which presents as firm, smooth, yellow to pigmented papules that appear as successive crops on the neck, axillae, chest, abdomen, and/or periumbilical region. To our knowledge, there are only 10 published reports of familial eruptive syringomas. Herein we describe the eleventh report of familial eruptive syringomas, review the literature on this unusual presentation, and suggest a novel classification of familial syringomas based on our literature review. Observations: We report two cases of eruptive syringoma within a family. Eruptive syringomas were widely distributed on the trunk of a healthy 16-year-old female and her 19-year-old brother. Both the 19-year-old man and his mother also had infraorbital syringomas. Conclusion: Familial eruptive syringomas are a rare clinical entity that is likely autosomal dominantly inherited. Future reports of this unusual condition may provide further insight into the etiology of familial syringomas, and genetic analysis of cases may enable the causative gene mutation to be determined.

scholarly journals Congenital eruption cyst: a case report

2010 ◽  
Vol 21 (3) ◽  
pp. 259-262 ◽  
Author(s):  
Ramón Manuel Alemán Navas ◽  
María Guadalupe Martínez Mendoza ◽  
Mário Roberto Leonardo ◽  
Raquel Assed Bezerra da Silva ◽  
Henry W. Herrera ◽  
...  
Keyword(s):  
Case Report ◽  
Tooth Extraction ◽  
Who Classification ◽  
World Health ◽  
Close Monitoring ◽  
Cystic Lesions ◽  
Separate Entity ◽  
Epithelial Cysts ◽  
Health Organization

Congenital pathologies are those existing at or dating from birth. Occurrence of congenital cystic lesions in the oral cavity is uncommon in neonates. Eruption cyst (EC) is listed among these unusual lesions. It occurs within the mucosa overlying teeth that are about to erupt and, according to the current World Health Organization (WHO) classification of epithelial cysts of the jaws, EC is a separate entity. This paper presents a case of congenital EC successfully managed by close monitoring of the lesion, without any surgical procedure or tooth extraction. Eruption of the teeth involved, primary central incisors, occurred at the fourth month of age. During this time neither the child nor mother had any complication such as pain on sucking, refusal to feed, airway obstruction, or aspiration of fluids or teeth.

The Revised European-American Classification of Lymphoid Neoplasms (REAL): a new perspective for the classification of cutaneous lymphomas

1997 ◽  
Vol 24 (6) ◽  
pp. 329-341 ◽  
Author(s):  
Christian A. Sander ◽  
Peter Kind ◽  
Peter Kaudewitz ◽  
Mark Raffeld ◽  
Elaine S. Jaffe
Keyword(s):  
European American ◽  
Lymphoid Neoplasms ◽  
Cutaneous Lymphomas ◽  
New Perspective

scholarly journals InterLymph hierarchical classification of lymphoid neoplasms for epidemiologic research based on the WHO classification (2008): update and future directions

Blood ◽  
2010 ◽  
Vol 116 (20) ◽  
pp. e90-e98 ◽  
Author(s):  
Jennifer J. Turner ◽  
Lindsay M. Morton ◽  
Martha S. Linet ◽  
Christina A. Clarke ◽  
Marshall E. Kadin ◽  
...  
Keyword(s):  
Who Classification ◽  
Hierarchical Classification ◽  
International Classification Of Diseases ◽  
World Health ◽  
Molecular Characteristics ◽  
Lymphoid Tissues ◽  
Epidemiologic Research ◽  
Lymphoid Neoplasms ◽  
Health Organization

Abstract After publication of the updated World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues in 2008, the Pathology Working Group of the International Lymphoma Epidemiology Consortium (InterLymph) now presents an update of the hierarchical classification of lymphoid neoplasms for epidemiologic research based on the 2001 WHO classification, which we published in 2007. The updated hierarchical classification incorporates all of the major and provisional entities in the 2008 WHO classification, including newly defined entities based on age, site, certain infections, and molecular characteristics, as well as borderline categories, early and “in situ” lesions, disorders with limited capacity for clinical progression, lesions without current International Classification of Diseases for Oncology, 3rd Edition codes, and immunodeficiency-associated lymphoproliferative disorders. WHO subtypes are defined in hierarchical groupings, with newly defined groups for small B-cell lymphomas with plasmacytic differentiation and for primary cutaneous T-cell lymphomas. We suggest approaches for applying the hierarchical classification in various epidemiologic settings, including strategies for dealing with multiple coexisting lymphoma subtypes in one patient, and cases with incomplete pathologic information. The pathology materials useful for state-of-the-art epidemiology studies are also discussed. We encourage epidemiologists to adopt the updated InterLymph hierarchical classification, which incorporates the most recent WHO entities while demonstrating their relationship to older classifications.

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