Pathology quiz case 2. Diagnosis: malakoplakia of the left tongue base.
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Biomedical subjects
Publications and source records attributed to Vicente A Resto.
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OBJECTIVES: Distinction of intestinal-type sinonasal adenocarcinoma (ITAC) from adenocarcinoma of intestinal origin metastatic to the sinonasal cavity may be extremely difficult on histologic grounds alone. We studied the role of cytokeratin (CK) and mucin (MUC) expression in differentiating ITAC, metastatic adenocarcinoma of intestinal origin, and non-intestinal-type sinonasal adenocarcinoma (non-ITAC). METHODS: We stained specimens from 5 cases of ITAC and 4 cases of non-ITAC, along with 4 colonic and 3 duodenal adenocarcinoma controls, with CK7 and CK20, MUC2 and MUC5, neuron-specific enolase (NSE), chromogranin (CHR), and carcinoembryonic antigen (CEA) in order to examine the possible combinations of markers that best aid in the diagnosis of these lesions. We also performed a retrospective review of our clinical experience with these rare lesions. RESULTS: CK7 staining was positive in all ITAC and non-ITAC cases, whereas all cases displaying gastrointestinal-type differentiation (ITAC and metastatic intestinal cases) stained positive for both CK20 and MUC2. Staining for MUC5, NSE, CHR, and CEA was variable. CONCLUSIONS: Tumors with the CK7+, CK20+, MUC2+ immunophenotype are likely primary sinonasal lesions, whereas tumors with the CK7-, CK20+, MUC2+ profile warrant further clinical evaluation to exclude metastatic disease from the gastrointestinal tract. Complete surgical resection of ITAC remains the mainstay of therapy.
Sinonasal malignancies are highly heterogeneous lesions that are challenging to diagnose, treat, and follow clinically. Outcomes remain relatively poor and are associated with late diagnosis, difficult surgical anatomy, and a lack of effective adjuvant modalities of treatment. New multimodal treatment protocols addressed to specific tumor entities are being assessed with promising results. Because of the relative rarity of these lesions, however, progress is slow. The best therapeutic outcomes are achieved in the setting of early diagnosis combined with a meticulous, comprehensive, multidisciplinary approach to the management of these complex tumors.
OBJECTIVE: To review the biology of nasal glioma and encephalocele and to present an algorithm for preoperative evaluation and surgical management. DESIGN: Retrospective review and analysis. SETTING: Tertiary care medical center: 1970 to 2002. PATIENT: Sixteen patients with glioma (n = 10) and encephalocele (n = 6). OUTCOME: Age at the time of presentation, sex, signs and symptoms, imaging findings, surgical approach, pathology, complications, rate of recurrence, and follow-up were recorded. RESULTS: Ten patients presented with nasal glioma with a mean age of 9 months. All patients underwent surgical excision. No complication was encountered with a mean follow-up of 3.5 years. Six patients presented with encephaloceles with a mean age of 15.5 months. All patients underwent surgical excision. Complications included cerebrospinal fluid leak (n = 1) and epiphora (n = 1). Follow-up was 1 to 14 years (mean, 4 years). CONCLUSION: Nasal glioma and encephalocele are rare, benign, congenital lesions with a potential for intracranial extension. Evaluation should include a complete rhinologic and neurologic examination. Preoperative imaging with a thin-cut axial and coronal computed tomography scan and/or multiplanar magnetic resonance imaging is essential. Surgical intervention should be performed soon after diagnosis to alleviate the increased risk of meningitis. A frontal craniotomy approach is recommended if intracranial extension is identified based on preoperative evaluation, followed by an extracranial resection. If there is no evidence of intracranial extension, a conservative extracranial approach is recommended.