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INFLAMMATORY
MYOFIBROBLASTIC
TUMOUR OF LUNG
– A CASE REPORT
DR.SAUMIK SAHA. RMO-CTVS MCH-
KOLKATA.
CASE REPORT
 41yrs / F / hypertensive - referred from chest
dept. with diagnosis of SOL in rt upper lobe of
lung.
 Recurrent LRTI, diffuse Rt sided chestpain and
occassional haemoptysis x 1 year.
 Pulmonary tuberculosis was ruled out by routine
investigation.
Bronchoscopy
 Partial occlusion of right main bronchus by the
extraluminal mass
CECT thorax
 Right upper lobe hetrogenously
enhancing SOL (70mm x60mm x60mm)
with adjoining pleural thickening.
FNAC - few inflammatory cells with
alveolar macrophages and spindle
cells, with no malignant cells.
 CT guided trucut biopsy -
Proliferation of regular spindle cells
aligned in fascicles, mixed with
lymphocytes, plasma cells ,eosinophils
Immunohistochemical analysis -
positive staining for
ALK1,andVimentin.
 IgG/ IgM/ IgA for Echinococcus antibody was
negative.
D/D
 Hydatid cyst of lung
 IMT
 Organized pneumonia
 Lymphoma
 Solitary fibrous tumor
 Desmoid fibromatosis
 Angio myofibroblastoma
 Fibrosarcoma
 Leiomyoma
 Malignant fibrous histiocytoma
 Because of similar morphology of theses lesions, only
immunohistochemical investigations allowed the correct final
diagnoses.
Mode of tumor growth, the low mitotic index, the polyclonality of
lymphoid markers and the negativity of CD34 usually remove most of
these diagnoses
 Based on these data, the
diagnosis of IMT was made, and after
consultion withThe Oncology dept. surgical
resection was planned.
OT
After routine preoperative investigations
and optimization patient underwent Right
upper and middle lobectomy through standard
Right posterolatral thoracotomy through
5th ICS.
HPE
 Made of myofibroblastic spindle cells:
A variable mixture of
collagen, inflammatory cells, and usually
cytologically bland spindle cells showing
myofibroblastic differentiation.
Post operative histopathological
report confirmed the preoperative
diagnosis.
DISCUSSION
 The IMT is a very rare benign tumor representing
0.7% of all lung tumors. Having intermediate
malignant potential.
 Histology: Myofibroblastic spindle cells.There
rare 3 histological subtypes.
 Recurrence rates : 15% to 37%.
 30% of cases of IMT are closely related to
recurrent respiratory infections
 HISTORY
 IMT was first described in the lung by Brunn in
1939
 Völker reported a laryngeal IMT and compared it
with spindle cell carcinoma.
 IMT in the urinary bladder was 1st reportd by
Lekas
 Al-Jabri reported another case in the liver
 PATHOGENESIS- Uncertain
-Translocation of ALK gene .
-Immunogenic response to an infective or non
infective agent.
 Immunohistochemistry :
Reactivity for vimentin (99% sensitive for IMT)
and smooth muscle actin (SMA). Immunohistochemical
positivity for ALK (Anaplastic Lymphoma Kinase gene)
(99% specificity & 50% sensitivity)is detectable in just over
half of the cases with cytoplasmic staining. H-caldesmon
and PS100(Phosphatidyle Serine) estimation is also
useful in the diagnosis.
 Age/sex predominance : Both sexes
Children and young adults mostly.
 Recurrence rate: 4% in cases of incomplete resection.
 Survival rate: post surgical resection 5-year survival is
91.3%.
CONCLUSION
 IMT is a rare benign tumor. Clinical and
radiological presentation is variable and
nonspecific and the diagnosis is rarely made
before surgical management.Only histological
and immunohistochemical study can confirm the
diagnosis.
 Despite being a benign lesion, its potential for
recurrence and local invasion requires complete
surgical resection.
INFLAMMATORY MYOFIBROBLASTIC TUMOUR OF LUNG

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INFLAMMATORY MYOFIBROBLASTIC TUMOUR OF LUNG

  • 1. INFLAMMATORY MYOFIBROBLASTIC TUMOUR OF LUNG – A CASE REPORT DR.SAUMIK SAHA. RMO-CTVS MCH- KOLKATA.
  • 2. CASE REPORT  41yrs / F / hypertensive - referred from chest dept. with diagnosis of SOL in rt upper lobe of lung.  Recurrent LRTI, diffuse Rt sided chestpain and occassional haemoptysis x 1 year.  Pulmonary tuberculosis was ruled out by routine investigation.
  • 3.
  • 4. Bronchoscopy  Partial occlusion of right main bronchus by the extraluminal mass
  • 5. CECT thorax  Right upper lobe hetrogenously enhancing SOL (70mm x60mm x60mm) with adjoining pleural thickening.
  • 6.
  • 7. FNAC - few inflammatory cells with alveolar macrophages and spindle cells, with no malignant cells.  CT guided trucut biopsy - Proliferation of regular spindle cells aligned in fascicles, mixed with lymphocytes, plasma cells ,eosinophils
  • 8. Immunohistochemical analysis - positive staining for ALK1,andVimentin.
  • 9.  IgG/ IgM/ IgA for Echinococcus antibody was negative.
  • 10. D/D  Hydatid cyst of lung  IMT  Organized pneumonia  Lymphoma  Solitary fibrous tumor  Desmoid fibromatosis  Angio myofibroblastoma  Fibrosarcoma  Leiomyoma  Malignant fibrous histiocytoma  Because of similar morphology of theses lesions, only immunohistochemical investigations allowed the correct final diagnoses. Mode of tumor growth, the low mitotic index, the polyclonality of lymphoid markers and the negativity of CD34 usually remove most of these diagnoses
  • 11.  Based on these data, the diagnosis of IMT was made, and after consultion withThe Oncology dept. surgical resection was planned. OT After routine preoperative investigations and optimization patient underwent Right upper and middle lobectomy through standard Right posterolatral thoracotomy through 5th ICS.
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  • 15. HPE  Made of myofibroblastic spindle cells: A variable mixture of collagen, inflammatory cells, and usually cytologically bland spindle cells showing myofibroblastic differentiation. Post operative histopathological report confirmed the preoperative diagnosis.
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  • 19. DISCUSSION  The IMT is a very rare benign tumor representing 0.7% of all lung tumors. Having intermediate malignant potential.  Histology: Myofibroblastic spindle cells.There rare 3 histological subtypes.  Recurrence rates : 15% to 37%.  30% of cases of IMT are closely related to recurrent respiratory infections
  • 20.  HISTORY  IMT was first described in the lung by Brunn in 1939  Völker reported a laryngeal IMT and compared it with spindle cell carcinoma.  IMT in the urinary bladder was 1st reportd by Lekas  Al-Jabri reported another case in the liver  PATHOGENESIS- Uncertain -Translocation of ALK gene . -Immunogenic response to an infective or non infective agent.
  • 21.  Immunohistochemistry : Reactivity for vimentin (99% sensitive for IMT) and smooth muscle actin (SMA). Immunohistochemical positivity for ALK (Anaplastic Lymphoma Kinase gene) (99% specificity & 50% sensitivity)is detectable in just over half of the cases with cytoplasmic staining. H-caldesmon and PS100(Phosphatidyle Serine) estimation is also useful in the diagnosis.  Age/sex predominance : Both sexes Children and young adults mostly.  Recurrence rate: 4% in cases of incomplete resection.  Survival rate: post surgical resection 5-year survival is 91.3%.
  • 22. CONCLUSION  IMT is a rare benign tumor. Clinical and radiological presentation is variable and nonspecific and the diagnosis is rarely made before surgical management.Only histological and immunohistochemical study can confirm the diagnosis.  Despite being a benign lesion, its potential for recurrence and local invasion requires complete surgical resection.