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Year : 2018  |  Volume : 14  |  Issue : 2  |  Page : 465-467

Aggressive laryngeal fibromatosis: A case report and brief review

Department of Pathology, Apollo Gleneagles Hospital, Kolkata, West Bengal, India

Date of Web Publication8-Mar-2018

Correspondence Address:
Dr. Arpita Sutradhar
58, Canal Circular Road, Kolkata - 700 054, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.183554

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 > Abstract 

We describe a rare case of aggressive fibromatosis of the larynx occurring in a 72-year-old man. The lesion manifested with complaints of dyspnea and stridor. A computerized tomographic scan of the neck revealed irregular polypoidal growth invading right vocal cord, crossing midline. A malignant tumor was suspected. Although several laryngoscopy-driven biopsies were negative for malignancy, total laryngectomy was done since the lesion was not deemed amenable to conservative therapy. Tissue sections featured a moderately cellular lesion composed of spindle cells with bland, elongated nuclei, enmeshed in a variably collagenized ground substance. The spindle cells were immunopositive for vimentin and negative for cytokeratins, PGP-9.5, smooth muscle antigen, CD68, desmin, and S-100 protein and Ki-67 of 4%. No further therapy was administered. Based on the available literature, our data confirm that aggressive laryngeal fibromatosis in adult patients is a locally infiltrating disease. Total laryngectomy with clear margins is needed as to avoid the high risk of local recurrence.

Keywords: Aggressive fibromatosis, extra-abdominal fibromatosis, larynx

How to cite this article:
Khan EM, Sutradhar A, Bandopadhay S. Aggressive laryngeal fibromatosis: A case report and brief review. J Can Res Ther 2018;14:465-7

How to cite this URL:
Khan EM, Sutradhar A, Bandopadhay S. Aggressive laryngeal fibromatosis: A case report and brief review. J Can Res Ther [serial online] 2018 [cited 2021 Apr 13];14:465-7. Available from: https://www.cancerjournal.net/text.asp?2018/14/2/465/183554

 > Introduction Top

Extra-abdominal fibromatosis arises principally from the connective tissue of the muscle and overlying fascia or aponeurosis; it chiefly affects the muscles of the shoulder and pelvic girdles and thigh of adolescent and young adults. It is estimated that 3–4 cases per million occur in the US annually. Twenty-three percent of all extra-abdominal fibromatosis occur in the head and neck region.[1] At this site, the soft tissue of the neck is most commonly involved, followed by face, oral cavity, scalp, paranasal sinus, and orbit.[2] Larynx is an extremely unusual site. To the best of our knowledge, this is the sixth case to be documented.

Here, a case of 72-year-old male patient with laryngeal aggressive fibromatosis is reported.

 > Case Report Top

A 72-year-old man presented to the Department of Otolaryngology/Head and Neck Surgery, with dyspnea and stridor. He complained of respiratory distress for 2 days and dry cough for 1 month. The patient had no history of fever, chest pain, or hemoptysis.

However, he had a history of several such episodes in the last 4 years for which he was investigated thoroughly and four times he underwent laryngoscope-guided excisional biopsy by laser of a mass in the glottis region of larynx in various other hospitals. Biopsy was done and it turned out each time to be negative for malignancy.

This time, the patient was hospitalized and emergency tracheostomy was done. Computed tomography scan of the neck displayed an irregular polypoidal growth involving right vocal cord, crossing midline going up to transglottic [Figure 1]. There was no evidence of adjacent thyroid cartilage destruction, extra-laryngeal extension, or cervical node involvement. Chest X-ray findings were essentially normal.
Figure 1: Computed tomography scan neck

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The patient was put up for elective tracheostomy followed by total laryngectomy.

Gross specimen was that of a total laryngectomy specimen with a noduloulcerative tumor involving right glottis and subglottis measuring 3 cm × 2 cm × 1.5 cm free from all resected margins. Cut section shows whitish homogenous and fleshy cut surface [Figure 2].
Figure 2: Grossly noduloulcerative growth. Cut section shows fleshy cut surface

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Microscopically, sections show poorly circumscribed tumor comprising proliferation of well-differentiated fibroblasts and few histiocytes with variable amount of collagen [Figure 3] and focally infiltrative margins [Figure 4]. Mitosis and atypia are absent. There is no invasive malignancy. Resection margins are free form tumor. Immunohistochemistry revealed cells strongly positive for vimentin and very low Ki-67, approximately 4%, a marker for cell proliferation [Figure 5]. Cytokeratin, S-100, desmin, PGP-9.5, smooth muscle antigen (SMA), and CD68 were negative. Based on these data, the neoplasm was pathologically diagnosed as aggressive fibromatosis or desmoids tumor of larynx.
Figure 3: H and E, ×40: Well differentiated fibroblasts with collagen

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Figure 4: H and E, ×40: Infiltrating muscle

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Figure 5: Ki-67: Approximately 4% positive

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There were no complications during or after surgery. The patient is followed up periodically to monitor the possible recurrence but so far has remained symptom-free.

 > Discussion Top

Fibromatosis comprises a broad group of benign fibrous tissue proliferations of similar microscopic appearance whose biologic behavior is intermediate between that of benign fibrous lesions and fibrosarcoma. Like fibrosarcoma, the fibromatosis is characterized by infiltrative growth and a tendency toward recurrence; however, unlike this tumor, they never metastasize.

First described by McFarlane in 1832,[3] it was 6 years later that Muller[4] first coined the term desmoid, from the Greek word “desmos” meaning tendon-like. Also known as aggressive fibromatosis, desmoid lesions are benign locally aggressive slow-growing lesions that arise from deep musculoaponeurotic tissue. Reports suggest that desmoid tumors account for 0.03% of tumors and approximately 3% of all soft tissue lesions.[5] In this instance, where surgery may be necessary, it is important to note the high recurrence rate of 15–77%[6] with a recent report from Ozger et al., demonstrating a 52.6% recurrence rate at 8 years.[7]

The clinical manifestations of aggressive fibromatosis in the present case were compatible with those reported generally for this type of neoplasm. Aggressive fibromatosis of larynx is extremely rare and only five cases have been reported in the literature. Here, we report the sixth case.

The larynx is a favored site for the occurrence of papilloma and the closely related hemangioma. The larynx is also a vulnerable area for deep granulomatous infections (tuberculosis, leprosy, and botryomycosis of histoplasmosis) which can present as nodular lesions. Another granulomatous lesion, sarcoidosis, can present a similar picture. The larynx may be a preferred site for neurilemoma, neurofibroma, fibroma, or myxoma, all of which can present as painless, circumscribed nodules of varying size.[8],[9] A diagnosis of aggressive fibromatosis should be confirmed by histological examination of the entire resected specimen and by the exclusion of other types of pathologies.

Extra-abdominal desmoid type fibromatosis often begins as a painless, deep soft tissue mass in the lower extremity (e.g., thigh, foot, or ankle), upper extremity (e.g., shoulder or hand), or the head and neck. In our case, patient presented as painless soft tissue mass with some pressure symptoms.

Grossly, extra-abdominal desmoid type fibromatosis is usually composed of nonencapsulated gray-white tissue confined to the musculature and overlying fascia or aponeurosis. The tumors are firm and glistening white on cut section and resemble scar tissue. Our case presented as noduloulcerative mass and on cut section, it was whitish homogenous and fleshy.

The tumors are composed of alternating bundles of locally infiltrating, monomorphic elongated, spindle-shaped fibroblast and myofibroblast bundles within a collagenous stroma. Mitotic activity and cellularity are low (four mitoses per 50 high-power field). Necrosis and hemorrhage are absent features in this tumor, and we had similar findings in our case.

The cells are usually actin-positive and CD34- and S-100-negative.[10] Masson's trichrome stain and reticulin stain can be used to reveal collagen interlaced between tumor cells. In our case, Masson's trichrome stain showed collagen between tumor cells. The tumor in our case was strongly vimentin-positive but negative for SMA, desmin, S-100, CD68, and cytokeratin.

Surgery and radiation therapy, either alone or in combination, have been used to treat extra-abdominal desmoids. For potentially resectable lesions, surgery provides excellent local control, even in those with recurrent disease. In our case, complete surgical resection was done and the patient is asymptomatic till date.


We would like to thank Dr. Shantanu Panja, MS ENT (PGIMER, Chandigarh, India), Fellow Head and Neck and Skull Base Surgery, USA, Consultant ENT and Head and Neck Oncosurgeon, Department of Otolaryngology, Head and Neck Surgery, Apollo Gleneagles Hospital Limited, Kolkata, West Bengal, India.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 > References Top

Dahn I, Jonsson N, Lundh G. Desmoid tumours. A series of 33 cases. Acta Chir Scand 1963;126:305-14.  Back to cited text no. 1
Conley J, Healey WV, Stout AP. Fibromatosis of the head and neck. Am J Surg 1966;112:609-14.  Back to cited text no. 2
Hosalkar HS, Torbert JT, Fox EJ, Delaney TF, Aboulafia AJ, Lackman RD. Musculoskeletal desmoid tumors. J Am Acad Orthop Surg 2008;16:188-98.  Back to cited text no. 3
Pakos EE, Tsekeris PG, Goussia AC. Desmoid tumours of the extremities and trunk: A review of the literature. Int Orthop 2005;29:210-3.  Back to cited text no. 4
Papagelopoulos PJ, Mavrogenis AF, Mitsiokapa EA, Papaparaskeva KT, Galanis EC, Soucacos PN. Current trends in the management of extra-abdominal desmoid tumours. World J Surg Oncol 2006;4:21.  Back to cited text no. 5
Dalén BP, Bergh PM, Gunterberg BU. Desmoid tumors: A clinical review of 30 patients with more than 20 years' follow-up. Acta Orthop Scand 2003;74:455-9.  Back to cited text no. 6
Ozger H, Eralp L, Toker B, Agaoglu F, Dizdar Y. Evaluation of prognostic factors affecting recurrences and disease-free survival in extra-abdominal desmoid tumors. Acta Orthop Traumatol Turc 2007;41:291-4.  Back to cited text no. 7
Iglesias Moreno MC, Bhathal Guede B, González Gimeno MJ, Martín Villares C, Ortega Medina L, Arrazola J, et al. Neurilemmoma of the larynx: A case report. Acta Otorrinolaringol Esp 2004;55:446-50.  Back to cited text no. 8
Kim KM, Kim SC, Jeong HJ, Kie JH. Myxoma: Life-threatening benign nonepithelial tumor of the larynx. Yonsei Med J 1997;38:187-9.  Back to cited text no. 9
Ferenc T, Sygut J, Kopczynski J, Mayer M, Latos-Bielenska A, Dziki A, et al. Aggressive fibromatosis (desmoid tumors): Definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background. Pol J Pathol 2006;57:5-15.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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