ABSTRACT
Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.
Subject(s)
Antineoplastic Agents/therapeutic use , Biopsy , Child, Preschool , Follow-Up Studies , Humans , Magnetic Resonance Imaging , Male , Neuroectodermal Tumors, Primitive, Peripheral/diagnosis , Neuroectodermal Tumors, Primitive, Peripheral/drug therapy , Neuroectodermal Tumors, Primitive, Peripheral/radiotherapy , Orbital Neoplasms/diagnosis , Orbital Neoplasms/drug therapy , Orbital Neoplasms/radiotherapy , Radiotherapy, Adjuvant , Tomography, X-Ray ComputedSubject(s)
Humans , Female , Adult , Facial Paralysis/etiology , Neuroectodermal Tumors, Primitive, Peripheral/complications , Skull Neoplasms/complications , Temporal Bone , Biopsy, Fine-Needle , Fatal Outcome , Immunohistochemistry , Magnetic Resonance Imaging , Neuroectodermal Tumors, Primitive, Peripheral/diagnosis , Neuroectodermal Tumors, Primitive, Peripheral/drug therapy , Skull Neoplasms/diagnosis , Skull Neoplasms/drug therapy , Tomography, X-Ray ComputedABSTRACT
Seminal vesicle tumor is a rare disease with unclear origin. Generally, it is presented as a pelvic mass that can be detected by sonography and digital rectal exam. The authors report a 25-year-old patient with a pelvic mass which the magnetic resonance and surgical specimen reveal a seminal vesicle tumor. Immunohistochemical findings favored a primitive neuroectodermal tumor of the seminal vesicle. Herein, the treatment, histological and histochemical findings of this entity are discussed.
Subject(s)
Humans , Male , Adult , Genital Neoplasms, Male/diagnosis , Neuroectodermal Tumors, Primitive, Peripheral/diagnosis , Seminal Vesicles/pathology , Antineoplastic Combined Chemotherapy Protocols , Chemotherapy, Adjuvant , Follow-Up Studies , Genital Neoplasms, Male/drug therapy , Genital Neoplasms, Male/surgery , Immunohistochemistry , Magnetic Resonance Imaging , Neuroectodermal Tumors, Primitive, Peripheral/drug therapy , Neuroectodermal Tumors, Primitive, Peripheral/surgery , Seminal Vesicles/surgery , Treatment OutcomeABSTRACT
Presentamos un caso de colonización de catéter venoso central de tipo Porth-a-cath y fungemia por el hongo filamentoso oportunista Fusarium oxysporum, en un paciente de cuatro años portador de un tumor neuroectodérmico periférico abdominal. El cuadro consistió en un episodio de neutropenia febril de corta duración, sin foco clínico, con hallazgo de Fusarium oxysporum en hemocultivos centrales cuantitativos y cultivo mediante técnica de Maki del extremo distal del dispositivo. La rápida recuperación de la neurotropenia y el retiro del dispositivo permitieron a nuestro juicio la regresión del cuadro, a pesar de no usar antifúngicos por vía general. El paciente no ha presentado nuevos episodios atribuibles a micosis hasta 20 meses después del episodio
Subject(s)
Humans , Male , Child, Preschool , Catheterization, Central Venous/adverse effects , Fusarium/pathogenicity , Neutropenia/microbiology , Abdominal Neoplasms/drug therapy , Catheters, Indwelling/adverse effects , Fungemia , Fusarium/isolation & purification , Neuroectodermal Tumors, Primitive, Peripheral/drug therapyABSTRACT
Os autores fizeram um levantamento bibliográfico sobre o conceito, classificaçäo, diagnóstico e prognóstico dos tumores neuroectodérmicos primitivos periféricos (PNETs), fazendo um comentário final quanto ao tratamento destas patologias