RESUMEN
Extramedullary plasmacytoma of the larynx is rare, especially when coexisted with squamous cell carcinoma in situ. We report a 56-year-old woman with hoarseness for 6 months and dysphonia for a week. Fiberoptic laryngoscopic examination showed a red, smooth-surface swelling in the submucous region of the left ventricle and ventricular band of the larynx. The patient underwent vertical laryngectomy and modified left neck dissection. Postoperative pathologic examination revealed coexisting plasmacytoma and carcinoma in situ. Bone marrow biopsy and systemic radiogram showed no positive findings. The hepatic and renal functions were normal. Monoclonal immunoglobulin light chain of type kappa was detected in urine. Hence, a laryngeal extramedullary plasmacytoma with carcinoma in situ was diagnosed. No recurrence or progression was observed during a 2-year follow-up. Here, we discussed the risk factors, diagnosis, and therapy for this rare disease.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , ADP-Ribosil Ciclasa 1 , Metabolismo , Carcinoma in Situ , Diagnóstico , Diagnóstico por Imagen , Metabolismo , Patología , Cirugía General , Carcinoma de Células Escamosas , Diagnóstico , Diagnóstico por Imagen , Metabolismo , Patología , Cirugía General , Estudios de Seguimiento , Inmunoglobulina A , Metabolismo , Cadenas kappa de Inmunoglobulina , Metabolismo , Neoplasias Laríngeas , Diagnóstico , Diagnóstico por Imagen , Metabolismo , Patología , Cirugía General , Laringectomía , Laringoscopía , Mucina-1 , Metabolismo , Disección del Cuello , Plasmacitoma , Diagnóstico , Diagnóstico por Imagen , Metabolismo , Patología , Cirugía General , Sindecano-1 , Metabolismo , Tomografía Computarizada por Rayos XRESUMEN
<p><b>OBJECTIVE</b>To investigate the expression of phospho-platelet derived growth factor receptor alpha (P-PDGFR-alpha) in gastrointestinal stromal tumors (GIST) and extra-gastrointestinal stromal tumors (EGIST) and its clinical significance.</p><p><b>METHODS</b>Expression of P-PDGFR-alpha in 28 samples of positive CD117 and 13 samples of negative CD117 was detected by Envision immunohistochemical staining. Direct PCR sequencing was used to investigate the mutation status of c-kit gene exons 9, 11, 13, 17 and PDGFR-alpha gene exons 12 and 18.</p><p><b>RESULTS</b>The positive rate of P-PDGFR-alpha expression in GISTs with negative CD117 was 69.2%, which was significantly higher than that in GISTs with positive CD117 (7.1%, P<0.05). The positive rates of P-PDGFR-alpha expression in epithelioid GISTs(27.3%) and mixed GIST(63.3%) were both significantly higher than that in fusiform GISTs (9%, P<0.05). The positive rate of CD117 expression in fusiform GISTs (53.6%)was significantly higher than that in epithelioid GISTs (7.1%) and mixed GISTs(39.3%, P<0.05). C-kit gene mutation was found in 19 GIST cases with positive CD117. C-kit gene mutation was found in 19 of 28 GIST patients with positive CD117, among them, mutation of exon 11 occurred in 15 cases and exon 13 in 4 cases. No C-kit gene mutation was seen in 13 GIST patients with negative CD117. PDGFR-alpha gene mutation was found in 4 of 11 GIST cases with positive P-PDGFR-alpha and all occurred in exon 18.</p><p><b>CONCLUSIONS</b>Examination of P-PDGFR-alpha expression may provide reliable evidence for the further improvement of pathological diagnosis,pathological typing and treatment for GISTs with negative CD117. Phosphorylated protein induced by PDGFR-alpha mutation may be associated with the important alternative molecular mechanism and the biological behavior of GIST development.</p>