ALK-positive inflammatory myofibroblastic lung tumor: surgery and 2-year recurrence-free surveillance

This lung cancer diagnosis at a glance

Subtype
Inflammatory Breast Cancer
Biomarkers
ALK-1-positive; TPM4-ALK rearrangement; TP53 missense variant VAF 2.3%; DNMT3A frameshift mutation VAF 2.3%
Sex
Female
Treatment
surgery and surveillance
Outcome
Cancer-Free / NED

Treatment course, step by step

  1. Robotic bronchoscopy with endobronchial ultrasound was nondiagnostic
  2. robotic-assisted right lower lobectomy with wedge resection of the middle lobe and mediastinal lymph node dissection
  3. pathology showed inflammatory myofibroblastic tumor with negative margins, no lymphovascular invasion, no visceral pleural involvement, ALK-1 positivity, TPM4-ALK rearrangement, and negative lymph nodes
  4. multidisciplinary tumor board recommended surveillance CT and systemic therapy only if recurrence occurs
  5. stable without recurrence for 2 years.

What happened, in summary

This 67-year-old woman had nasal congestion, cough, nausea, fever, and shortness of breath with exertion. She had a 42 pack-year smoking history, a remote history of basal cell cancer of the nose, and a family history of lung cancer in her mother. Chest X-ray showed a right lower-lobe lung nodule. CT measured the nodule at 1.6 × 1.5 × 1.3 cm and showed no metastasis. PET imaging showed intense FDG uptake in the right lower-lobe nodule, with SUV 11.6, plus mild uptake in the right superior hilum. Pulmonary function testing showed decreased diffusion capacity without obstructive or restrictive pattern, and echocardiography showed normal systolic and diastolic function. Robotic bronchoscopy with endobronchial ultrasound sampled the nodule but did not give a definitive diagnosis; the tissue showed reactive lung changes, spindle cells, lymphocytes, and plasma cells. Because biopsy could not resolve the diagnosis, she underwent robotic-assisted right lower lobectomy with wedge resection of the middle lobe and mediastinal lymph-node dissection. Final pathology showed an inflammatory myofibroblastic tumor with negative margins, no lymphovascular invasion, and no visceral pleural involvement. Lymph nodes were negative for malignancy. Tumor testing showed ALK-1 positivity and a TPM4-ALK rearrangement, with low-level TP53 and DNMT3A variants. A multidisciplinary tumor board recommended CT surveillance, with systemic therapy only if recurrence occurred. During follow-up, she had intermittent shortness of breath with exertion but remained stable without recurrence for 2 years. ALK inhibitors were discussed as options for unresectable or recurrent ALK-positive IMT, but they were not delivered in this patient’s treatment course. For this patient, the completed treatment was surgical removal followed by surveillance.

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This is a lay summary of an account first published by PMC / PubMed Central. Read the original in full

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