Solitary fibrous tumor of the larynx: report of two new cases

Tumor fibroso solitário de laringe: relato de dois novos casos

Fábio Muradás Girardi Marinez Bizarro Barra Adriana Muradás Girardi About the authors

Abstracts

Solitary fibrous tumor is a rare neoplasm. Few cases have been described in the head and neck area, and less than 11 were located in the larynx. We described two new cases of solitary fibrous tumor of the larynx. A man, 64-year-old, and a woman, 77-year-old, both with submucosal and nodular supraglottic lesions, were submitted to surgical treatment and both showed CD-34 and bcl-2 immunoreactivity and S-100 and smooth-muscle actin negativity. After 24 and 22 months of postoperative follow-up, respectively, they did not show signs of active disease.

fibrous tissue tumor; laryngeal cancer


O tumor fibroso solitário é uma neoplasia rara. Foram descritos poucos casos em cabeça e pescoço, sendo não mais de 11 na laringe. Descrevemos dois novos casos de tumor fibroso solitário da laringe, um em um homem de 64 anos e outro em uma mulher de 77 anos, ambos com lesões supraglóticas submucosas e nodulares. Os casos foram submetidos a tratamento cirúrgico e ambos apresentaram imunorreatividade a CD-34 e bcl-2, e negatividade para S-100 e actina de músculo liso. Após 24 e 22 meses de seguimento pós-operatório, respectivamente, não apresentam sinais de doença em atividade.

neoplasias de tecido fibroso; neoplasias laríngeas


INTRODUCTION

Mesenchymal tumors of the head and neck, primarily those originating in the larynx, are rare and include several histological types. One of these neoplasms is the solitary fibrous tumor (SFT). SFT is a neoplasm considered part of the solitary fibrous tumor - hemangiopericytoma spectrum. This tumor was first described in the pleura. Approximately 50% of SFT cases are described in the thorax, while only 6% of the cases develop in the head and neck(55. GOLD, J. S. et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer, USA, v. 94, 2002.). At that location, the oral cavity is the most frequent subsite affected, although other cases have been reported in orbits, nose, paranasal sinuses, nasopharynx, parapharynx, larynx, major salivary glands, and thyroid(44. GANLY, I. et al. Solitary fibrous Tumors of the head and neck: a clinicopathologic and radiologic review. Arch Otolaryngol Head Neck Surg, USA, v. 132, n. 5, 2006.). No more than 11 cases have been reported in the larynx until this moment. We present two new cases that were surgically treated and a literature review of the larynx SFT reports.

CASE REPORT

Case report 1

A man, 64-year-old, with history of foreign body sensation in the pharynx for 6 months and progression to aspiration and hoarseness in the last e 3 months, was attended at our clinic with upper airway obstruction, which required an emergency tracheotomy. He denied smoking and alcohol intake and he was not taking any medications. A fiberoptic nasolaryngoscopy examination revealed a well-defined submucosal nodular lesion, which was about 3 cm in its greatest diameter, covered by intact mucosa, placed in the left aryepiglottic fold. It caused an almost complete obstruction of the laryngeal lumen and the left pyriform sinus. Cervical computed tomography (CT) scans showed a well-defined lesion, with no signs of associated local infiltration or lymphadenopathy, measuring 3.1 × 2.0 cm, extending to the upper margin of the false left vocal fold (Figure 1).

FIGURE 1
Computed tomography without contrast, showing a single, well-defined nodular lesion, that is hypodense in relation to muscle and left false fold space-occupying

The microscopic evaluation of the larynx biopsy suggested solitary fibrous tumor. The lesion was completely excised by partial supraglottic laryngectomy (Figure 2). There were no postoperative complications. The pathological examination revealed a greyish and opaque, encapsulated lesion, measuring 2.5 cm in its greatest diameter. At microscopy the tumor was composed by spindle cells elements in a uniform and solid pattern, with some areas of abundant collagen deposits, without signs of malignancy. The immunohistochemical panel showed a strong and diffuse immunoreactivity for CD34 and bcl-2; isolated cells were positive for CD99 and Ki-67, this one meaning low proliferative index. There was negativity for CD31 and smooth muscle actin (Table 1). The tumor resection was complete and surgical margins were free of disease. After 22 months the patient had no signs of active disease.

FIGURE 2
Surgical specimen (case 1)

Supraglottic partial laryngectomy product. A single and submucosal lesion placed on the left side of supraglottis is individualized.

TABLE 1
Immunohistochemical panel for both solitary fibrous tumors of the larynx cases

Case report 2

A woman, 77-year-old, with history of foreign body sensation in the pharynx for 8 months and progression to dysphagia, aspiration, and dysphonia in the last 4 months was attended at our clinic, after being initially treated with radiotherapy for suspected supraglottic cancer. She denied smoking, and there were no signs of alcoholism. The patient had a prior biopsy performed outside our institution that was suggestive of malignant tumor, with epithelioid pattern and clear cells. The immunohistochemical analysis showed a malignant neoplasm with myoepthelial phenotype in the supraglottic larynx, suggesting salivary gland origin. The specimen was reviewed in our institution. Our primary diagnostic hypothesis was a neoplasm of mesenchymal origin, possibly a STF, due to S-100 and smooth muscle actin negativity (Table 1). Fiberoptic nasolaryngoscopy examination revealed no signs of tumor regression.It was observed a submucosal nodule with well-defined borders, located in the aryepiglottic folds, covering the glottis, with partial obstruction of the pyriform sinus, especially on the right. CT scan showed a lesion with diffuse uptake of contrast, occupying the paraglottic and supraglottic space, crossing the midline, and greatly reducing the column of air in this region. Due to the lesion extension, previously radiotherapy treatment, patient's age and clinical condition, the patient underwent to a total laryngectomy as the treatment of choice (Figure 3). There were no postoperative complications. The pathological investigation revealed a brown encapsulated tumor, measuring 4.8 × 3.6 × 2.4 cm, composed of two well-defined portions, the major lesion located on the left side. Macroscopy showed two grayish slightly lobulated tumors, measuring 3.6 and 1.8 cm in its major diameter, respectively. Both lesions compressed but they did not infiltrate adjacent structures. It was performed a complete resection with free and wide surgical margins. In microscopy, supported by immunohistochemical results (Figure 4 e Table 1), the findings were consistent with the diagnosis of solitary fibrous tumor of the larynx.

FIGURE 3
Surgical specimen (case 2)

Total laryngectomy product. Two well-delimited tumors, both occupying the supraglottis and the paraglottis space bilaterally are individualized.

FIGURE 4
Microscopic appearance

On the left, hematoxylin-eosin stain, showing mesenchymal proliferation with spindle cells associated to collagen deposition. On the right and in the middle, immunohistochemistry for CD34 and bcl-2, respectively, showing strong and diffuse reactivity. Original magnification 100×.

DISCUSSION

SFT is a rare entity, most often found in the thorax. It has a mesenchymal origin and it is recognized by proliferation of thinwalled blood vessels and cells that produce collagen. It is classified within the solitary fibrous tumor-hemangiopericytoma spectrum. It was first described in the pleura by Klemperer and Rabin in 1931(66. KLEMPERER, P. et al. Primary neoplasms of the pleura: a report of five cases. Am J Ind Med, v. 22, n. 1, 1992.), and subsequently in many extrapleural locations. SFT must be included in the differential diagnosis of lesions with fibrous component, including fibromatosis, fibrous histocytoma, fibrosarcoma, synovial sarcoma, and metastatic scirrhous carcinoma. All these entities exhibit a dense fibrous collagen matrix that can reveal low T2 signal intensity in Magnetic Resonance Imaging. At CT, the tumors were highly vascularized, polypoid-to spherical-shaped, arising from the submucosa. Usually they show isoattenuation relative to the adjacent muscles. Low-attenuation areas inside the tumor may be related to cystic or myxoid degeneration or stroma areas with low contrast uptake(33. CHANG, S.K. et al. CT, MR, and angiography findings of a solitary fibrous tumor of the larynx: a case report. Korean, v. 9, n. 6, 2008.). Its clinical behavior is variable, although most of them are slow-growing, with benign course. It is difficult to differentiate them from other slow-growing mesenchymal diseases, since the diagnosis depends on architectural, cytomorphological, and immunohistochemical aspects(44. GANLY, I. et al. Solitary fibrous Tumors of the head and neck: a clinicopathologic and radiologic review. Arch Otolaryngol Head Neck Surg, USA, v. 132, n. 5, 2006.). The histopathological characteristics does not differ among the anatomical sites; they are characterized by the lack of an architectural pattern, varied cellularity, with areas of stromal hyalinization, and often associated to a hemangiopericytoma-like vascular pattern. These tumors show immunoreactivity for CD34, CD99, bcl-2, and vimentin; and are negative for cytokeratin, S-100, and smooth muscle actin(11. ALAWI, F. et al. Solitary fibrous tumor of the oral soft tissues: a clinicopathologic and immunohistochemical study of 16 cases. USA, v. 25, n. 7, 2001.). When tumors develop in larynx, it usually affect middle-aged adults, like other extrapleural disease cases. Perhaps due to early presentation, most of lesions are < 5 cm in diameter(22. ALOBID, I. et al. Laryngeal solitary fibrous tumor treated with CO2 laser excision: case report. Eur Arch Otorhinolaryngol, Spain, v. 262, n. 4, 2005.). It generally presents with progressive dysphonia, foreign body sensation, voice quality change, dyspnea or cough, with slow evolution of symptoms, depending on which part of the larynx is involved (Table 2).

TABLE 2
Summary of literature review of larynx solitary fibrous tumor cases*

Pedicled form is the most common presentation(33. CHANG, S.K. et al. CT, MR, and angiography findings of a solitary fibrous tumor of the larynx: a case report. Korean, v. 9, n. 6, 2008.). They do not usually destroy or invade adjacent tissues, although they may cause a mass effect that can lead to obstruction of the larynx lumen. The most affected laryngeal subsites are epiglottis, aryepiglottic folds, ventricular folds, and commissures, areas of glottis and subglottis which have the greatest amount of submucosal soft tissue. Although the knowledge about this entity is limited, the SFT of head and neck are mostly benign and can be successfully treated by conservative surgery(22. ALOBID, I. et al. Laryngeal solitary fibrous tumor treated with CO2 laser excision: case report. Eur Arch Otorhinolaryngol, Spain, v. 262, n. 4, 2005.). Although not being described for SFT patients, total laryngectomy remains as a viable treatment option, even in cases of benign tumors, especially for those with lung-related contraindications to partial laryngectomy, when we can predict difficulties with adherence by patients to follow-up routines, when dealing with recurrent tumors, or when in situations technically impeditive for conservative surgeries(99. WENIG, B. M. et al. Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. Cancer, USA, v. 76, n. 11. 1995.). Follow-up data of those cases are scarce. However, recurrence is a rare event, especially in the head and neck, being observed only in extreme situations, associated with incomplete excision, and in cases with atypical or malignant microscopic features. In those cases, adjuvant radiotherapy may play a role, although the experience with this treatment modality in the management of these patients is limited to small case series(11. ALAWI, F. et al. Solitary fibrous tumor of the oral soft tissues: a clinicopathologic and immunohistochemical study of 16 cases. USA, v. 25, n. 7, 2001.). Initially, the second case was thought to be a myoepithelial carcinoma of the larynx. Myoepithelial carcinoma is characterized by multilobulated architecture with polymorphic cellular structure, and may appear in spindle cells clusters, on solid and trabecular layers or reticular pattern. The immunohistochemical analysis shows expression of epithelial markers, such as keratin, epithelial membrane antigen (EMA) S100, smooth muscle markers as smooth muscle actin and calponin. Likewise, complete excision of lesion is the treatment of choice, although radiotherapy and chemotherapy may also join the therapeutic arsenal(88. REN, J. et al. Primary myoepithelial carcinoma of palate. World J Surg, China, v. 104, n. 9. 2011.). The diagnosis of SFT based on a small biopsy specimen is difficult due to wide intratumoral morphologic variation and similarities with other mesenchymal tumors(77. LO MUZIO, L. et al. Solitary fibrous tumor of the oral cavity: the need for an extensive sampling for a correct diagnosis. J Oral Pathol Med, Italy, v. 36, n. 9, 2007.). Diagnostic failures have already been described(1010. YAMADA, H. et al. Solitary fibrous tumor of the buccal space resected in combination with coronoidectomy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, Japan, v. 114, 2012.). In theses cases, morphological and immunohistochemical features are essential in differentiating it from other entities.

CONCLUSION

SFT of the larynx is a rare disease. The few cases described, suggest a predilection for middle-aged men. Signs and symptoms of the disease will depend on which site is involved. The diagnosis is based on histopathological and immunohistochemical results. Although the literature data is limited, this neoplasm usually presents with favorable prognosis when complete excision of the lesion is performed.

REFERENCES

  • 1
    ALAWI, F. et al. Solitary fibrous tumor of the oral soft tissues: a clinicopathologic and immunohistochemical study of 16 cases. USA, v. 25, n. 7, 2001.
  • 2
    ALOBID, I. et al. Laryngeal solitary fibrous tumor treated with CO2 laser excision: case report. Eur Arch Otorhinolaryngol, Spain, v. 262, n. 4, 2005.
  • 3
    CHANG, S.K. et al. CT, MR, and angiography findings of a solitary fibrous tumor of the larynx: a case report. Korean, v. 9, n. 6, 2008.
  • 4
    GANLY, I. et al. Solitary fibrous Tumors of the head and neck: a clinicopathologic and radiologic review. Arch Otolaryngol Head Neck Surg, USA, v. 132, n. 5, 2006.
  • 5
    GOLD, J. S. et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer, USA, v. 94, 2002.
  • 6
    KLEMPERER, P. et al. Primary neoplasms of the pleura: a report of five cases. Am J Ind Med, v. 22, n. 1, 1992.
  • 7
    LO MUZIO, L. et al. Solitary fibrous tumor of the oral cavity: the need for an extensive sampling for a correct diagnosis. J Oral Pathol Med, Italy, v. 36, n. 9, 2007.
  • 8
    REN, J. et al. Primary myoepithelial carcinoma of palate. World J Surg, China, v. 104, n. 9. 2011.
  • 9
    WENIG, B. M. et al. Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm. Cancer, USA, v. 76, n. 11. 1995.
  • 10
    YAMADA, H. et al. Solitary fibrous tumor of the buccal space resected in combination with coronoidectomy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, Japan, v. 114, 2012.

Publication Dates

  • Publication in this collection
    May-Jun 2014

History

  • Received
    21 Aug 2013
  • Reviewed
    22 Aug 2013
  • Accepted
    25 Mar 2014
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