Citation, DOI, disclosures and case data
At the time the case was submitted for publication Sophie Gregory had no financial relationships to ineligible companies to disclose.View Sophie Gregory's current disclosures
Presenting complaint of fresh hemoptysis on background of several months history of cough continuing despite smoking cessation. Intermittent chest discomfort but no dyspnea. No other significant medical history.
Chest X-ray at presentation
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There is a large mass in the right lower zone which obscures the right hemidiaphragm suspicious for malignancy. The left lung airspace is clear. No cardiomegaly. No bony lesions or fractures. External ECG leads.
CT Chest +C at presentation
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There is a large bulky right infrahilar mass of slightly heterogeneous enhancing soft tissue attenuation, causing obstructive atelectasis of the anterior and lateral basal segments of the right lower lobe. The mass measures 88mm AP x 85mm transverse x 97mm vertical diameter. No cavitation or calcification. No air bronchograms are visible within the mass. It appears there is a central hilar and infrahilar mass, of mixed slightly higher and lower attenuation with a possible necrotic component inferolaterally. At the superior aspect of the mass there is narrowing and abrupt obstruction of the right lower lobe bronchus and its segmental branches. There is extrinsic displacement of the right pulmonary veins, with a possible filling defect in the right inferior pulmonary vein. There is also extrinsic narrowing of the right interlobar pulmonary artery, by the adjacent infrahilar mass. At the posterior aspect of the mass, there is displacement of the atelectatic medial and posterior basal segments of the right lower lobe, which enhance slightly more than the central infrahilar mass. Ipsilateral right hilar lymph nodes are enlarged. No mediastinal or contralateral lymph node enlargement. No supraclavicular or upper abdominal lymphadenopathy. No pleural or pericardial effusion. Normal contralateral left lung and pleura. No destructive bony thoracic lesion. No abnormality of the imaged upper abdomen.
A large central neoplastic mass causing distal obstructive atelectasis of the lateral and anterior basal segments of the right lower lobe.
The diagnosis of a right lung biphasic pulmonary blastoma with large right lung mass and hilar/mediastinal lymphadenopathy was made on the basis of imaging and bronchoscopy guided biopsy. The patient received neoadjuvant chemo/radiotherapy with 4 cycles of carboplatin and etoposide before proceeding to have a right middle and lower lobe lobectomy (histology below). Following surgery they then received adjuvant chemotherapy with 4 cycles of carboplatin and etoposide.
Histological tumor type: Pulmonary blastoma.
- Tumor bed: 105mm (macroscopic measurement).
- Residual viable tumor: Scattered microscopic foci identified over an area measuring approximately 60mm (reconstructed measurement); largest microscopic focus in a single section measures 12mm. Treatment effect: 5% residual viable tumor; 10% fibroinflammatory stromal tissue; and 85% necrosis.
Visceral pleural invasion: Absent.
Lymphatic invasion: Not identified.
Vascular invasion: Not identified.
Perineural invasion: Not identified.
Spread through airspaces (STAS): Not identified.
Peribronchial/Hilar lymph nodes:
- Specimen 1. Right middle and lower lobe: Involved. Residual viable tumor is present with treatment retreatment related changes present (see microscopic comments above).
- Specimen 4. Right no. 11 lymph node: No evidence of malignancy. No evidence of treatment related changes.
- Specimen 2. Right no. 4 lymph node: No evidence of malignancy. No evidence of treatment related changes.
- Specimen 3. Right no. 7 lymph node: Involved. Residual viable tumor is present with treatment retreatment related changes present (see microscopic comments above). The lymph nodes otherwise show reactive changes, with sinus histiocytosis and carbon pigment deposition.
PATHOLOGICAL STAGE (AJCC 8th ed):
ypT3: Tumor greater than 5cm but less than 7cm.
ypN2: Metastasis in ipsilateral mediastinal lymph node.
pR0: Complete resection, margins histologically negative, no residual tumor.
- ALK: Negative
- ROS1: Negative.
- EGFR: No variant detected. No variant detected in BRAF or KRAS.
- PD-L1 TPS: 0%.
The tumor block contains ~60% viable tumor cell nuclei with dissection.
1-4. Right middle and lower lobe; No. 4, No. 7 & No. 11 lymph node right lung:
- Scattered microscopic foci of residual viable pulmonary blastoma, present over an area measuring 60mm size (tumor bed 105mm).
- Neoadjuvant treatment effect: 5% residual viable tumor; 10% fibroinflammatory stromal tissue; and 85% necrosis.
- Viable metastatic tumor within peribronchial and separately submitted No. 7 lymph nodes.
- No evidence of malignancy in separately submitted No. 4 & No. 11 lymph nodes.
- Stage (AJCC 8th ed.): ypT3 N2
- Residual tumor status: pR0 (margins clear).