The grade of neuroendocrine carcinoma (NEC) that is associated with hemoptysis, pneumonia, and tumor cells arranged in cords and clusters is:
Grade I neuroendocrine carcinoma (NEC) (classic or typical carcinoid) is a low-grade NEC; 80% arise in the epithelium of the central airways. It occurs primarily in younger patients. Because of the central location, it classically presents with hemoptysis, with or without airway obstruction and pneumonia. Histologically, tumor cells are arranged in cords and clusters with a rich vascular stroma. This vascularity can lead to life-threatening hemorrhage with even simple bronchoscopic biopsy maneuvers. Regional lymph node metastases are seen in 15% of patients, but rarely spread systemically or cause death.
Which of the following is NOT a known predictive or prognostic tumor marker for adenocarcinoma?
Establishing a clear histologic diagnosis early in the evaluation and management of lung cancer is critical to effective treatment. Molecular signatures are also key determinants of treatment algorithms for adenocarcinoma and will likely become important for squamous cell carcinoma as well. Currently, differentiation between adenocarcinoma and squamous cell carcinoma in cytologic specimens or small biopsy specimens is imperative in patients with advanced stage disease, as treatment with pemetrexed or bevacizumab-based chemotherapy is associated with improved progression-free survival in patients with adenocarcinoma but not squamous cell cancer. Furthermore, life-threatening hemorrhage has occurred in patients with squamous cell carcinoma who were treated with bevacizumab. Finally, EGFR mutation predicts response to EGFR tumor kinase inhibitors and is now recommended as first-line therapy in advanced adenocarcinoma. Because adequate tissue is required for histologic assessment and molecular testing, each institution should have a clear, multidisciplinary approach to patient evaluation, tissue acquisition, tissue handling/processing, and tissue analysis. In many cases, tumor morphology differentiates adenocarcinoma from the other histologic subtypes. If no clear morphology can be identified, then additional testing for one immunohistochemistry marker for adenocarcinoma and one for squamous cell carcinoma will usually enable differentiation. Immunohistochemistry for neuroendocrine markers is reserved for lesions exhibiting neuroendocrine morphology. Additional molecular testing should be performed on all adenocarcinoma specimens for known predictive and prognostic tumor markers ( eg, EGFR, KRAS, and EML4-ALK fusion gene). Ideally, use of tissue sections and cell block material is limited to the minimum necessary at each decision point. This emphasizes the importance of a multidisciplinary approach; surgeons and radiologists must work in direct cooperation with the cytopathologist to ensure that tissue samples are adequate for morphologic diagnosis as well as providing sufficient cellular material to enable molecular testing.
Because the lesions have low cellularity and poor yield with fine needle aspiration (FNA), an open incisional biopsy for lesions over 3 to 4 em is often necessary. Surgery consists of wide local excision with a 2- to 4-cm margin and intraoperative frozen section assessment of resection margins. Typically, chest wall resection, including the involved rib(s) and one rib above and below the tumor with a 4- to S-cm margin of rib, is required. A margin of less than 1 em results in much higher local recurrence rates. If a major neurovascular structure would have to be sacrificed, leading to high morbidity, then a margin of less than 1 em would have to suffice. Survival after wide local excision with negative margins is 90% at 10 years.
A 57-year-old non-small-cell lung cancer patient with a potentially resectable tumor found on computed tomography (CT) scan who can walk on a flat surface indefinitely without oxygen or stopping to rest, secondary to dyspnea will most likely tolerate:
Patients with potentially resectable tumors require careful assessment of their functional status and ability to tolerate either lobectomy or pneumonectomy. The surgeon should first estimate the likelihood of pneumonectomy, lobectomy, or possibly sleeve resection, based on the CT images. A sequential process of evaluation then unfolds.
A patient's history is the most important tool for gauging risk. Specific questions regarding performance status should be routinely asked. If the patient can walk on a flat surface indefinitely, without oxygen and without having to stop and rest secondary to dyspnea, he will be very likely to tolerate lobectomy. If the patient can walk up two flights of stairs (up two standard levels), without having to stop and rest secondary to dyspnea, he will likely tolerate pneumonectomy. Finally, nearly all patients, except those with carbon dioxide (CO2) retention on arterial blood gas analysis, will be able to tolerate periods of single-lung ventilation and wedge resection.
An "onion-peel" appearance of a rib on CT is suggestive of:
Primitive neuroectodermal tumors (PNETs) (neuroblastomas, ganglioneuroblastomas, and ganglioneuromas) derive from primordial neural crest cells that migrate from the mantle layer of the developing spinal cord. Histologically, PNETs and Ewing sarcomas are small, round cell tumors; both possess a translocation between the long arms of chromosomes 11 and 22 within their genetic makeup. They also share a consistent pattern of proto-oncogene expression and have been found to express the product of the MIC2 gene. Ewing sarcoma occurs in adolescents and young adults who present with progressive chest wall pain, but without the presence of a mass. Systemic symptoms of malaise and fever are often present. Laboratory studies reveal an elevated erythrocyte sedimentation rate and mild white blood cell elevation. Radiographically, the characteristic onion peel appearance is produced by multiple layers of periosteum in the bone formation. Evidence of bony destruction is also common. The diagnosis can be made by a percutaneous needle biopsy or an incisional biopsy.
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