Thursday 15 August 2013

MALIGNANT FIBROUS HISTEOCYTOMA

             MALIGNANT FIBROUS HISTEOCYTOMA
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                         DR.K.K.DESARDA.  DR.S. PUNTAMBEKAR  KEM HOSPITAL PUNE.




INTRODUCTION

Malignant fibrous histiocytoma (MFH) is a tumor derived from mesenquimal tissue composed by 5 cell types  The term MFH was suggested to describe a tumor histiocytic-like with fibrous tissue . Its origin is believed to be from totipotent cells for presenting 2 different cell types. In histological terms, MFH is divided into 5 subtypes.

MFH is usually malignant when occurs on profound soft tissue. Around 3% of such tumors occur in the area of head and neck, and it is rare in larynx. New immunohistochemical and by electronic microscopy techniques have increased accuracy of histopathological diagnosis.
We described a rare case of MFH in the larynx and discussed its diagnosis and treatment.


CASE REPORT

54-year-old-male patient, smoker for 30 years was  seen in ENT OPD at KEM Hospital, Pune. in 2005 with the complaints of change of voice,odynophagia, and Foreign body   sensations in the throat for 18 months duration. There was no signs of respiratory distress or he neither had symptoms of dyspnoea, dysphagia nor important medical medical problems. Videolaryngoscopy, revealed smooth polypoidal mass on the anterior two third of left vocal cord with no paresis . There was no palpable cervical lymphadenopathy. In June, 2005, a microlaryngoscopy with polyp excision was performed and the biopsy taken from the mass was subjected for histopathological study, which was reported as  malignant fibrous histcytoma.He was then advised for surgical excision.and frontolateral left  laryngectomy was performed. The post operative recovery was excellent.He was reviewed at monthly intervals for almost two yrs, and there was recurrence seen.
 


  Videolaryngoscopy showing polypoidal mass over the left vocal cord


             Light microscopy showing large proliferation of fibroblasts mixed in several histiocytic, multinucleated cells.



DISCUSSION

The term MFH was suggested by Kauffman and Stout in 1961 in order to describe a histiocytic-like tumor with predominance of fibroblasts

MFH on head and neck area is more common in men, in a proportion of 3:1 and it affects this area only in 3% of the cases  on the skin and soft tissues. Nose, paranasal sinus and mandible are the most common areas  with malignant feature when in profound areas  There are 30 cases reported  and most of them are subglotti , preferably in men on glottic area and in women in subglottic  Patient with glottic MFH are usually older and smoker, opposing from subglottic MFH.

It is histologically divided into 5 types: pelomorphic, myxoid, inflammatory, giant cells and angiomatous  It is derived from mesenquimal tissue composed by 5 cell types: fibroplast, histiocytic, indistinguished, giant multinucleated and xantomatous cells  Its origin is believed to be from totipotent cells for presenting two different cell types. Structural analysis through electronic microscopy, immunohistochemical techniques and culture of tissue help in order to differ this type of tumor . The immunohistochemical anti-vimentin V9 was positive in this case.

This tumor often presents only clinical symptoms, such as dyspnoea, dysphagia, hemoptysis, stridor and tiredness when achieves larger sizes . Diagnosis for it is usually established after the removal of the tissue mass.

Differential diagnosis should be performed with pleomorphic rhabdomyosarcoma, fibrosarcoma, spinocellular carcinoma, angiosarcoma, hemangiopericytoma, pleomorphic liposarcoma and lymphoma

Surgery is the therapy with block resection of tumor. The hemilaryngectomy technique was chosen, though tumor was limited to left vocal fold. Cervical emptying was not performed because physical exam. did not presente lymphonode involvement. Radiotherapy is reserved to high risky patients, recurrence of non-operated patients and cases of metastasis to distance . Some authors suggest chemotherapy for other types of sarcomas .  Adjuvant radiotherapy and chemotherapy with surgery requires more studying.

MFH of the larynx is a rare type of tumor, but a high recurrence, fast growth, unexpected clinical behaviour and it tends to local and distance metastasis, especially to brain and lungs There is a lack of prognosis when there is sign of metastasis and glottic MFH, what differs from subglottic MFH


CONCLUSION

MFH is a rare type of tumor and there are few reported cases in the literature,thus makes its prognosis and therapy  difficult. Surgical therapy is the one with ample resection of tumor. It has high recurrence rate and hence periodical review is mandatory.


BIBLIOGRAPHY

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