Diagnosis for Case of the Week January 21, 2005

• With SOB, DOE and non-productive cough for 4 days
• Also with fever to 101, Nausea & vomiting, Fatigue
• Denies CP, sinus congestion, h/a or recent sick contacts
• Hx of pneumonia ~ 1 year ago, tx’d with abx
• Previous visits to ED in 3/04 and 9/04 for SOB
• No medications
• Smokes 1 ppd x 45+ years
• Works as a tree climber
• Physical
  • T 97.2, RR 24, 96% RA, HR 100, 130/94
  • Gen: thin WM, in NAD
  • HEENT: pearla, anicteric sclera
  • Chest: diminished breath sounds on R chest
  • CV: regular, no m/g/r
• Labs
  • WBC 16.4
  • Hgb 13.4
  • Plt 294
  • Alb 4.1
  • Alp 138
  • Alt 41
  • Ast 41
  • T.Bili 1.3
  • Na⁺ 145
  • Cl^- 102
  • BUN 27
  • K⁺ 3.9
  • HCO₃ 24.6
  • Cr 0.7
  • Glc 113
  • Ca⁺⁺ 9.5

Additional History

• Referred for bronchoscopy

• Reports continued cough, SOB, DOE

• Continued fatigue and reports poor sleep due to increased coughing when lying flat

• No further fevers after course of biaxin

• No further N/V, appetite ok

• ~5 lb weight loss in 2 weeks

Differential

• Bronchogenic Carcinoma

• Broncholith, Broncholithiasis

  • Broncholith = Calcified material within the lumen of the tracheobronchial tree

  • Arise from erosion and extrusion of calcified necrotic material from LN

  • Usually due to granulomatous infections

  • Present with cough, hemoptysis, fever, purulent sputum, episodic wheeze and lithoptysis

  • May also develop bronchoaortic or bronchesophageal fistulas, mediastinitis

• Papilloma

• Granular Cell Tumor

  • Neoplasm of neural derivation

  • Granular appearance of cells due to accumulation of secondary lysosomes in cell cytoplasm

  • More common in African-Americans,

  • Slight female predominance

  • Varying age at presentation, peak in 30s-50s

  • Found at any site, visceral involvement less common

  • Malignant GCTs rare, tend to be larger (> 4 cm) and symptomatic

• Adenoid Cystic Carcinoma

• Mucoepidermoid Carcinoma

  • Low grade malignancy with benign course or high grade with aggressive course

  • Symptoms include cough, fever, wheeze, chest pain, hemoptysis

  • On bronchoscopy appear as polypoid mass

  • Can involve bronchi, less commonly trachea

  • High grade forms may extend into parenchyma

  • On histology appears as distinct areas of squamous cells with mucus secreting columnar cells

• Carcinoid

• Leiomyoma

• Lipoma

• Tracheobronchopathia Osteochondroplastica

  • Osteocartilaginous nodules within submucosa of trachea, bronchi

  • Arise from cartilage rings, posterior membranous portion spared

  • Incidence, etiology unknown

  • Most cases in adults > 50 yo, male:female 3:1

  • Symptoms include dyspnea, cough, hemoptysis

• Metastatic Tumor

• Hamartoma

  • Term coined in 1904 by Albrecht

  • Disorganized mature, normal tissues or cells indigenous to the particular site found in abnormal proportions

  • Thought to arise from embryonic rests present in fetal life but not visible until adulthood

  • May represent mesenchymal clonal neoplasms

  • Benign lesions , malignant transformation extremely rare

  • Pulmonary Hamartoma

    • Most common form of benign lung tumor

    • Incidence 0.025%-0.32%

    • More frequent in men, median age 50-60, most smokers

    • 90% peripheral, 10% or less endobronchial

    • Usually asymptomatic

    • Solitary, round nodules

    • “Popcorn” calcification in 10%

    • Histology shows lobules of cartilage, fat, fibromyxoid tissue, smooth muscle, bone separated by clefts lined by non-neoplastic respiratory epithelium

    • Epithelial clefts less apparent on endobronchial tumors

  • Endobronchial Hamartoma

    • One study reviewed all bronchial bx proven EH dx at their institution over a 23 year period (1974-1997)

    • 47 EH diagnosed, 43 studied (4 without available clinical hx)

    • Mean age 50s-60s, predominently male

    • 9 asymptomatic, Recurrent respiratory infection or obstructive pneumonia in 37%, Hemoptysis 32%

    • 7 patients had EH found during w/u of concurrent lung CA

    • No predominant location noted on radiograph, bronch

    • Appearance on bronch of exophytic, polypoidal mass with smooth well-limited surface and no submucosal infiltration

    • Gross consistency spongiform

    • On histology 37.2% chondroid hamartoma, 30.2% lipoid

    • 26 of 36 with bronchial obstruction and no concurrent malignancy, 24 treated to remove

    • 22 of these 24 reevaluated with bronchoscopy with 81.8% with >80% reduction in obstruction

Histopathology:

Diagnosis: Endobronchial Hamartoma

Follow Up:

• Discharged after 23 hour admission

• Returned for bronchoscopy ~ 1 month later

• Notes decreased SOB, DOE but with persistent cough

• Denies Fever/Chills, Nausea/Vomiting, fatigue

References:

• Hughes et al, “Fine Needle Aspiration of Pulmonary Hamartoma,” Arch Pathol Lab Med, vol 129, January 2005.

• Borja et al, “Endobronchial Hamartoma,” Chest, 2002; 122: 202-205.

• Sharkey et al, “Endobronchial Hamartoma Presenting as Massive Hemoptysis,” Eur Respir J, 1996, 9, 2179-2180.

• UpTodate, Differential Diagnosis and Evaluation of the Solitary Pulmonary Nodule.

• UCSF Path Case 21, “Hamartoma.”

• Studer et al, “Mediastinal Abscess Due to Passage of a Broncholith,” Chest, 2002; 121: 296-297.

• Boujaoude et al, “Actinomyces in a Broncholith; Chicken v. Egg,” Chest, 2003; 124 (4): 270.

• Galvin et al, “Mucoepidermoid carcinoma,” International Thoracic Teaching Resource, Virtual Hospital.

• Robbins et al, Pathologic Basis of Disease.

Disclaimer:  This information is intended solely for resident review of presented cases which may or may not be pathologically proven. Information is derived from a number of published sources of varying reliability and does not represent original research from the institution. It is not intended to be comprehensive and should therefore not substitute for careful review of the literature. 

Return to Current Pulmonary Case of the Week

For medical and imaging questions contact: