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{ بسم الله الرحمن الرحيم }. Non-Malignant Lung Tumors. ( Surgical point and cases). Dr.Yasser ahmed elsaid ; M.D. Cardiothoracic surgery Air Force Hospital. Classification can be confusing :- Many tumors go by different names Difficult to define benign vs. malignant

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non malignant lung tumors surgical point and cases

Non-Malignant Lung Tumors.(Surgical point and cases)

Dr.Yasserahmedelsaid ;

M.D. Cardiothoracic surgery

Air Force Hospital

slide3
Classification can be confusing :-
    • Many tumors go by different names
    • Difficult to define benign vs. malignant
    • Many benign lesions not truly neoplasms
    • Classify based on:
      • Cell Type.
      • Anatomical location.
      • Malignant potential.
slide4

Predominantly located in :

*airways.

*Parenchyma.

*pleura.

predominantly located in airways
Carcinoid (bronchial adenoma)

Cylindroma (adenoid cystic carcinoma)

Mucous gland adenoma

Granular cell myoblastoma

Mucoepidermoid Carcinoma

Papilloma/papillomatosis

Bronchial mucous gland adenoma

Peripheral nerve sheath tumors

Granular cell tumor (Abrikossoff’s tumor)

Predominantly located in airways:
predominantly located in parenchyma
Papillary adenoma

Alveolar adenoma

Neurofibroma

Meningioma

Fibroma

Glomus tumor

Endometrioma

Pseudolymphoma

Chondromas (Carney’s triad)

Teratoma

Pulmonary tumorlets

Pulmonary mucinous cystadenoma

Pulmonary sugar tumor (epithelioid myomelanocytoma)

Primary pleuropulmonary thymoma

Heterotopic meningeal proliferations

Chemodectoma (paragonglionoma)

Minute pulmonary chemodectomas

Predominantly located in parenchyma:
found in airways or parenchyma
Hamartoma

Leiomyoma/leiomyomatous hamartoma/BML

Glomus tumor/glomangioma

Chondroma/myxoma/fibromyxoma

Pneumocytoma

Angiomyolipoma

Myelolipoma

Cystic fibrohistiocytic tumor

Hemangioma/hemangiomatosis

Lipoma/lipoblastoma

Found in airways or parenchyma:
predominantly located on the pleura
Predominantly located on the pleura:
  • Adenomatoid tumor of the pleura.
  • Calcifying fibrous pseudotumor of the pleura.
  • Solitary fibrous tumor of the pleura.
pseudotumors
Tracheopathiaosteochondroplastica.

Inflammatory pseudotumor.

Mycobacterialpseudotumor.

Post-traumatic pseudotumor.

Nodular sarcoidosis.

Nodular amyloidosis.

Hematoma.

Lipoid pneumonia.

Rheumatoid granuloma.

Wegener’s granulomatosis.

Loculated interlobular pleural effusion.

Pulmonary infarct.

Lymphoid hyperplasia.

Pseudotumors:
slide11

*Pseudoneoplastic pleural changes:

    • Reactive mesothelial proliferations.
    • Hyaline pleural plaques.
    • Diffuse pleural fibrosis.
benign borderline tumors
Predominantly located in airways:

Papilloma/papillomatosis

Bronchial mucous gland adenoma

Peripheral nerve sheath tumors

Granular cell tumor (Abrikossoff’s tumor)

Carcinoid (bronchial adenoma)

Cylindroma (adenoid cystic carcinoma)

Mucous gland adenoma

Granular cell myoblastoma

Mucoepidermoid Carcinoma

Benign/Borderline Tumors
slide13

Predominantly located in parenchyma:

    • Pulmonary mucinous cystadenoma
    • Pulmonary sugar tumor (epithelioid myomelanocytoma)
    • Primary pleuropulmonary thymoma
    • Heterotopic meningeal proliferations
    • Chemodectoma (paragonglionoma)
    • Minute pulmonary chemodectomas
    • Papillary adenoma
    • Alveolar adenoma
    • Neurofibroma
    • Meningioma
    • Fibroma
    • Glomus tumor
    • Endometrioma
    • Pseudolymphoma
    • Chondromas (Carney’s triad)
    • Teratoma
    • Pulmonary tumorlets
slide14

Predominantly located on the pleura:

    • Adenomatoid tumor of the pleura

Calcifying fibrous pseudotumor of the pleura

    • Solitary fibrous tumor of the pleura
  • Found in airways or parenchyma:
    • Hamartoma
    • Leiomyoma/leiomyomatoushamartoma/BML
    • Glomus tumor/glomangioma
    • Chondroma/myxoma/fibromyxoma
    • Pneumocytoma
    • Angiomyolipoma
    • Myelolipoma
    • Cystic fibrohistiocytic tumor
    • Hemangioma/hemangiomatosis
    • Lipoma/lipoblastoma
slide15

Pseudotumors:

    • Tracheopathia osteochondroplastica
    • Inflammatory pseudotumor
    • Mycobacyterial pseudotumor
    • Post-taumatic pseudotumor
    • Nodular sarcoidosis
    • Nodular amyloidosis
    • Hematoma
    • Lipoid pneumonia
    • Rheumatoid granuloma
    • Wegener’s granulomatosis
    • Loculated interlobular
slide16
Classification can be confusing :-
    • Many tumors go by different names
    • Difficult to define benign vs. malignant
    • Many benign lesions not truly neoplasms
    • Classify based on:

* Cell Type.

* Anatomical location.

* Malignant potential.

slide17

Some Cases with

your kind

suggestion for diagnosis..

case 1
Case #1
  • 63 y/old m with a 2 pk/D. for 10 Y. of smoking.
  • referred for incidental finding on CXR.
    • Denies cough, sputum, hemoptysis, with non specific, wt loss.
    • Vitals, exam, and labs are normal.
slide25
What is the name of the syndrome characterized by multiple hamartomatous neoplasm's of ectodermal, mesodermal, and endodermal origin?
  • Carney syndrome
  • Meigs syndrome
  • Cowden syndrome
  • Sill syndrome
slide26
What is the name of the syndrome characterized by multiple hamartomatous neoplasm's of ectodermal, mesodermal, and endodermal origin?
  • Carney syndrome
  • Meigs syndrome
  • Cowden syndrome
  • Sill syndrome
hamartoma
Hamartoma
  • Most common benign pulmonary tumor
    • 5-8% of all Solitary pul. Nodules.
    • Rarely can have multiple lesions (Cowden syndrome)
  • >60 yrs
  • M>F (2-3:1)
  • Small, well circumscribed, Most < 4 cm
    • “Popcorn” calcification (25%)
    • Fat, cartilage, muscle
  • Any lung zone - 5% endobronchial
  • Tx: observation – surgical resection.
    • Sarcomatous transformation has been reported .
case 2
Case #2
  • 37 y/old female non-smoker was referred for hemoptysis.
    • Intermittent blood-streaked sputum for 2 mo
    • 2 weeks ago developed foul and purulent sputum
    • CXR obtained.
    • Patient was started on antibiotics and referred to us.
slide29
Past Medical History of 2nd Pneumonia in 4 months, otherwise PMH are unremarkable.
  • Vital Sing Stable, A febrile.
  • By exam . There is only dullness to percussion on the right side .
  • Labs – leukocytosis 13.5; .
  • otherwise normal.
slide35
Which paraneoplastic syndrome is most commonly associated with this tumor?
  • Lambert-Eaton Myasthenic syndrome
  • Carcinoid syndrome
  • Myasthenia gravis
  • Cushing syndrome
  • Dooley-Powers syndrome
slide36
Which paraneoplastic syndrome is most commonly associated with this tumor?
  • Lambert-Eaton Myasthenic syndrome
  • Carcinoid syndrome
  • Myasthenia gravis
  • Cushing syndrome
  • Dooley-Powers syndrome
carcinoid
Carcinoid
  • <3% of all pulmonary tumors
  • Neuroendocrine tumor derived from Kulchitsky cells
  • Mean age 45 yrs; slightly more common in females
  • Pulmonary tumors 1-2% of all carcinoids (90% GI)
    • 80% central – lobar or subsegmental
    • 20% peripheral – SPNs
  • Often asymptomatic
    • Central tumors – hemoptysis, cough, wheezing, atelectasis
carcinoid1
Carcinoid
  • Typical carcinoid – low grade
    • 15% will metastasize.
  • Atypical carcinoid – intermediate grade
    • <10% of pulmonary carcinoids
    • Often locally invasive
    • Mets to LNs and distant sites in >50%
  • Small cell carcinoma – high grade
carcinoid2
Carcinoid
  • Can produce serotonin, ACTH, somatostatin, bradykinin
    • Cushing syndrome (ACTH) – 2%
      • Most common cause of ectopic ACTH secretion
    • Carcinoid syndrome (serotonin) – rare in pulm tumors
    • Acromegaly (GH )
    • Zollinger-Ellison syndrome in pts with MEN and carcinoids
carcinoid3
Carcinoid
  • Approx 25% have areas of calcification on CT
  • Vascularity not a contraindication to biopsy
  • Tx: Surgical removal for limited disease
    • Bronchoscopic removal insufficient
    • Chemo/XRT limited efficacy
  • 10 yr survival
    • Typical 82%
    • Atypical 56%
case 3
Case #3
  • 10 y/o m was referred for hoarseness slowly progressive over several months
  • 2 episodes of blood tinged hemoptysis over the last week
  • PMH is unremarkable
  • Vitals, exam, labs, and CXR are normal
slide47

Respiratory

Papillomatosis

slide48
With regards to respiratory papillomatosis, which of the following is true?
  • The pulmonary nodules associated with this condition frequently demonstrate cavitations
  • It has been linked to infection with herpes simplex virus.
  • Most patients with laryngeal papillomatosis will go on to develop lower respiratory lesions
  • Local recurrence after removal of a lesion is rare
slide49
With regards to respiratory papillomatosis, which of the following is correct?
  • The pulmonary nodules associated with this condition frequently demonstrate cavitations
  • It has been linked to infection with HSV1
  • Most patients with laryngeal papillomatosis will go on to develop lower respiratory lesions
  • Local recurrence after removal of a lesion is rare
papillomatosis
Papillomatosis
  • Multiple, recurrent, coarsely lobulated tumors
  • On epithelial surfaces
  • Predilection for ciliated/squamous junction
  • Can present with hoarseness, hemoptysis, airway compromise/atelectasis
papillomatosis1
Papillomatosis
  • Juvenile-onset : typically diagnosed at 18-36 mo
    • 60% have mothers with genital papillomas
    • Frequent regression in puberty
    • Malignant transformation to squamous cell CA are rare.
  • Adult papillomatosis : <12% of all cases
    • Childhood disease not necessary
    • White, warty growths described as “grape-bunch”
    • Less aggressive than the juvenile form
papillomatosis2
Papillomatosis
  • Laryngeal lesions common
    • <2% go on to develop distal airway lesions
    • Parenchymal lesions 1.5 – 5 cm with potential to cavitate
  • Treatment:
    • Laser/cryo/phototherapy for endotracheal lesions
    • Antiviral therapy topical/systemic – off label
    • Interferon – off label
    • Radiation
  • Commonly recur at site of resected lesions.
  • Increased risk of squamous cell carcinoma.
papillomatosis3
Papillomatosis
  • Solitary papillomatosis : less common
    • Age typically > 40; M>F
    • Lobar or segmental bronchi
    • Spherical tumors 0.5 – 1.5 cm
    • High risk of squamous cell carcinoma (33%)
      • Mean latency of 24 years
      • Increase risk with radiation therapy
    • Tx with resection and long-term survival(controvese).
case 4
Case #4
  • 54 y/o m nonsmoker was referred for wheezing slowly worsening over 18 mo. Ago.
  • No response to steroids or bronchodilators
  • No history of asthma or lung disease
  • Exam reveals wheezing L>R
  • Labs and CXR unremarkable
  • PFTs blunting of the expiratory curve.
slide61
Which of the following statements regarding this tumor is true?
  • Smoking is an independent risk factor
  • It predominantly occurs in males
  • It more commonly occurs in patients > 65 yrs
  • These tumors should be surgically resected whenever possible .
slide62
Which of the following statements regarding this tumor is true?
  • Smoking is an independent risk factor
  • It predominantly occurs in males
  • It more commonly occurs in patients > 65 yrs
  • These tumors should be surgically resected whenever possible.
cylindroma
Cylindroma
  • Adenoid cystic carcinoma.
  • 1/1,000 respiratory tract tumors.
  • Arises from tracheobronchial mucous glands.
  • Mid 40s; M=F
  • Symptoms: cough, hemoptysis, wheezing .
  • Polypoid/rounded with sub-mucosal extension
    • Can ulcerate
cylindroma1
Cylindroma
  • Slow growing, but locally invasive
    • “malignancy in slow motion”
  • Tx: surgical resection with end-to-end anastomosis
    • Laser therapy for unresectable tumors
    • Poor response to chem/XRT
    • Surveillance for recurrence
    • Good long term survival
case 5
Case #5
  • 69 y/old femal referred for evaluation of pulmonary nodules
  • Recent hospitalization for Community acq. Pneumonia:
    • CT chest revealed RLL Pneumonia and too numers to count pulm nod 1-4mm .
    • Follow up scan showed resolution of RLL process only
  • Currently she denies any pulmonary or infectious symptoms.
  • No history of TB exposure, or cancer.
  • Vitals, exam, labs, PFTs are all normal.
slide71
Histologically, these tumor most closely resemble which of the following?
  • Squamous cell carcinoma
  • Carcinoid
  • Hemangioma
  • Sarcoma
  • Fungus balls
slide72
Histologically, these tumor most closely resemble which of the following?
  • Squamous cell carcinoma
  • Carcinoid
  • Hemangioma
  • Sarcoma
  • Fungus balls
pulmonary tumorlets
Pulmonary Tumorlets
  • Small (<5mm), numerous pulm nodules
  • Elderly women with bronchiectasis or fibrosis
  • Proliferation of neuroendocrine cells adjacent to small bronchioles
  • Histologically resemble tiny carcinoids
  • Usually benign and discovered incidentally
    • 1 report of Cushing syndrome
    • Several reports of hilar LN involvement
case 6
Case #6
  • 41 y/o f presented to the exam. room complaining of abdominal pain :
    • It is associated with constipation which resolved with laxatives.
    • During the work-up an abdominal CT was done.
    • Multiple round nodules were seen in the lung bases.
    • Referred to the pulmonary clinic for evaluation.
  • History of present illness – chronic, dry cough previously attributed to GERD and allergies.
  • Past medical history – significant for Hysterectomy at age 36 for tx of uterine leiomyomatosis
  • Past history – no tobacco use or occupational exposures.
  • Vitals, exam, labs – unremarkable.
case 61
CT guided biopsy showed whorled smooth muscle fibers

No atypia or increased mitotic activity

Estrogen and Progesterone receptor positive

Case #6
slide79
Which of the following is a risk factor for the development of this tumor?
  • History of breast or uterine cancer
  • Smoking
  • Family history of Benign Met. Liomat.
  • Hysterectomy
  • Male gender
slide80
Which of the following is a risk factor for the development of this tumor?
  • History of breast or uterine cancer
  • Smoking
  • Family history of BML
  • Hysterectomy
  • Male gender
slide81
BML
  • Extrauterine spread of benign uterine leiomyomas
  • Occurs several years after hysterectomy or uterine trauma
  • Usually asymptomatic
  • Multiple well rounded lesions on radiographs
  • Histology can help differentiate between low grade sarcoma
slide82
BML
  • Tumors often responsive to hormonal therapy
  • Indolent course
    • Can spontaneously remit
  • Reports of successful treatments with:
    • Surgical castration
    • Progesterone
    • Gonadotropin releasing hormone analogues.
    • Aromitase inhibitors
    • Lutenizing hormone agonists
    • Selective estrogen receptor modulators.
case 71
Case #7
  • 36 y/o m c/o wheezing and dysnea on exertion slowly progressive over several months.
  • No history of smoking or asthma.
  • No response to bronchodilators or ICS.
slide88
Which of the following statements regarding mucous gland adenoma is true?
  • It is one of the most common endobronchial tumors.
  • The term “mucous gland adenoma” is actually a misnomer, as the tumor is not a true adenoma.
  • These tumors normally arise in the lobar or segmental bronchi.
  • I don’t really care; stop asking these stupid questions.
slide89
Which of the following statements regarding mucous gland adenoma is true?
  • It is one of the most common endobronchial tumors
  • The term “mucous gland adenoma” is actually a misnomer, as the tumor is not a true adenoma
  • These tumors normally arise in the lobar or segmental bronchi
  • I don’t really care; stop asking these stupid questions
mucous gland adenoma
Mucous Gland Adenoma
  • Only true “bronchial adenoma”
  • Extremely rare
  • Men and Women age 20s to 60s
  • Found in main, lobar, or segmental bronchi
  • Papillary tumors 0.5 to 7 cm; slimy, mucoid surface
  • If present, symptoms due to airway obstruction
  • Hystologically resembles tracheal mucous glands
  • Can be difficult to distinguish from mucoepidermoid CA
  • Tx: surgical or endobronchial resection
conclusion
Conclusion
  • A large number of non-malignant neoplasms of the lung have been described.
  • Many are very rare, limiting clinical experience
  • Can be categorized by:
    • Most common anatomic location.
    • Cell type.
    • Malignant potential.
slide92

Clinicians should be familiar with : the presentation, clinical course, and appropriate therapy of the more common types of non-malignant neoplasms.

  • Treatment (controversy) Surgical interference
  • vs,
  • follow up .