Fibromatosis Aggressiva of Parapharyngeal Space – a Case

artykuł oryginalny / original research article

Fibromatosis aggressiva of parapharyngeal space – a case report and review of literature Fibromatoza przestrzeni przygardłowej – opis przypadku i przegląd piśmiennictwa.

Angelika Piotrowska1, Anna Rzepakowska2, Piotr Chęciński2, Kazimierz Niemczyk2 1Studenckie Koło Naukowe przy Katedrze i Klinice Otolaryngologii Warszawskiego Uniwersytetu Medycznego 2 Katedra i Klinika Otolaryngologii Warszawskiego Uniwersytetu Medycznego

Article history: Received: 17.05.2017 Accepted: 26.05.2017 Published: 30.06.2017

ABSTRACT: Desmoid fibromatosis is defined as a rare fibroblastic proliferation. Although histologically benign, desmoids are- lo cally invasive and associated with a high local recurrence rate. When located in the head and neck region, fibromatosis tends to be more aggressive and associated with significant morbidity, which may be attributed to the proximity of the vital structures. We report a case of 33-year-old woman with aggressive fibromatosis of parapharyngeal space. Complete surgical exci- sion of the lesion was performed and the postoperative period was without complications. The patient remains under careful follow-up and there is no recurrence of the disease. KEYWORDS: desmoid-type fibromatosis, parapharyngeal space tumor, head and neck neoplasms

STRESZCZENIE: Fibromatoza jest rzadką chorobą nowotworową. Charakteryzuje się miejscową agresywnością i dużą tendencją do wznowy miejscowej. Lokalizacja fibromatozy w rejonie głowy i szyi wiąże się z cięższym przebiegiem i gorszym roko- waniem w związku z występowaniem w tej okolicy ważnych życiowo struktur anatomicznych. Przedstawiono opis procesu diagnostycznego i postępowania terapeutycznego u 33-letniej pacjentki z fibromatozą przestrzeni przygardłowej po stronnie lewej. Zastosowana resekcja chirurgiczna zmiany z dostępu szyjnego pozwoliła na radykalne usunięcie nowotworu bez istotnych powikłań pooperacyjnych. Chora pozostaje w obserwacji ambulatoryjnej bez wznowy choroby. SŁOWA KLUCZOWE: fibromatoza złośliwa, przestrzeń przygardłowa, guzy przestrzeni przygardłowej, nowotwory głowy i szyi

INTRODUCTION retromandibular fossa laterally [1]. This region contains cranial nerves: glossopharyngeal, vagus, accessory and hypoglos- The parapharyngeal space is the most deeply located region of sal nerves, sympathetic trunk, internal carotid artery, internal the head and neck. It has a shape of an inverted pyramid with jugular vein and deep cervical lymph nodes [2]. the base facing upwards extending from the base of the skull to the apex that connects with the middle space of the neck at Primary tumors of the parapharyngeal space are a rare con- the level of the angle of the mandible [1]. This space is adja- dition. They represent 0.5% of all head and neck tumors and cent to the spine and prevertebral muscles to the posterior, to are benign in 80% of cases [3,4]. Most lesions of this region are the lateral wall of the nasopharynx medially and the oral part multiform adenomas originating from the deep lobe of the pa- of the middle pharynx medially and to the infratemporal and rotid gland, followed by a schwannoma and paraganglioma [5].

POLSKI PRZEGLĄD OTORYNOLARYNGOLOGICZNY, TOM 6, NR 2 (2017), s. 45-49 DOI: 10.5604/01.3001.0010.0179 45 artykuł oryginalny / original research article

Less frequent tumors include angiofibroma, sarcoma, menin- ures was reported. On observation, the symptoms exacerbated gioma, chordoma, angioma and lipoma [6]. and progression was recognized on an MRI scan performed two months after the biopsy. The patient was referred to the Fibromatosis (aggressive fibromatosis, desmoid-type fibroma- Department of Otolaryngology of the Medical University of tosis) refers to lesions caused by abnormal proliferation of fi- Warsaw for further treatment. broblasts. It occurs in 2 to 4 cases per 100,000,000 population annually, and constitutes around 3% of soft tissue tumors [7]. On physical examination, lateral deviation to the tongue to the It is characterized by locally aggressive growth, tendency for left was observed as well as adhesions of the mucous mem- local recurrence, but distant metastases are not seen in the brane lining the palatine arches and the pharynx on the left course of the disease [8]. Classification of fibromatosis in- side, which impaired mobility of the soft palate. Moreover, the cludes: superficial type involving fascia or the deep type in- pharyngeal reflexes were weak on ipsilateral side. Full blood volving muscle aponeurosis [9]. The deep type is further sub- count revealed normocytic anemia: RBC 3,56x106/μl (refer- divided into extraabdominal, intraabdominal and contained ence range 3,8-5,2), HGB 11,35 g/dl (reference range 12-16) within the abdominal wall [10]. The etiology is multifactorial; and low hematocrit 33,20% (reference range 37-47). Chest genetic predisposition, hormonal and physical factors such as X-ray showed no abnormalities. trauma or surgical intervention are all possible factors [11]. Also, an increased morbidity of fibromatosis was observed in The patient was qualified for a resection of the parapharyngeal familial adenomatous polyposis and Gardner’s syndrome has mass from the cervical access. During the surgery, the common been shown [12]. Hormones seem to play an important role carotid artery and internal jugular vein were identified. The -ex since, according to some sources, those tumors are prevalent ternal carotid artery was visible surrounded by a pathological amongst females [13] with peak incidence at the age of 25 to tissue and therefore it was ligated at the sinus. The submandib- 35 [14]. Moreover, it has been shown in studies that estrogens ular gland was resected as well. The tumor infiltrated the par- can promote proliferation of fibroblasts [15]. pharyngeal space medially to the parotid gland and descended reaching the level of the thyroid cartilage. The facial nerve was identified within the parotid gland, then the gland was displaced CASE REPORT superiorly. The tumor was resected starting from the inferior part removing fragments of tissue and separating it from the muscles A 33-year-old female patient was admitted to the Department of the lateral pharyngeal wall and prevertebral muscles and their with a diagnosis of a left parapharyngeal tumor. On admission, insertion into the base of the skull. At the level of the palatine the patient reported symptoms such as throbbing pain in the tonsil, where it was earlier attempted to excise the mass, it was left ear, left-sided hearing loss, pain in the left maxillary sinus, necessary to remove the tonsil due to infiltration of the mucous paresthesia of the tongue and pharynx on the left side, that had membrane. The margins of the mucous membrane were sewn been recurring for over a year. The history was positive for gas- together. The defect was filled with the sternocleidomastoid troesophageal reflux and gastric and duodenal peptic ulcer. muscle. The hypoglossal nerve, which was resected segmental- ly due to infiltration, was sutured end-to-end. Five months earlier, the patient was hospitalized at a different facility due to a left-sided tumor of the oral part of the middle During surgery, a lymph node and ten tissue fragments in total pharynx. The CT scan of the viscerocranium and neck revealed size of 7 x 6 x 2.5 cm were excised. On pathology study, three an abnormal structure with well-defined borders extending biggest slices revealed a beige homogenous mass with fibrous from the base of the dens of the axis superiorly, separated by structure and blood hemorrhages, adjacent to the external a narrow slit of fatty tissue from the masseter anteriorly, and surface at some points, which occupied 80% of the samples. directly adjacent to the lateral wall of the pharynx reaching On microscopic view, a lesion with ill-defined borders was the root of the tongue. described infiltrating the adipose, muscle and salivary gland tissues in a branching manner. Comparing the morphological, The lesion indented the nasopharynx and the oral part of the histochemical and immunohistochemical images, the diagnosis middle pharynx constricting the airways and adhering to the of desmoid fibromatosis was made. The postoperative course left submandibular gland. No infiltration of large cervical ves- was uncomplicated and the patient was fed through a gastric sels or abnormal lymph nodes were noted. Samples of the par- tube because the operation required opening of the orophar- apharyngeal mass were obtained from the oral and cervical ac- ynx. After the tube was removed, the patient was examined cesses. On pathology study, a mesenchymal tumor consisting by a laryngologist a few times due to minor dysphagia. She of a fibroblast-type fusiform cells with no atypia or mitotic fig- was discharged home in a good general and local condition

46 WWW.OTORHINOLARYNGOLOGYPL.COM artykuł oryginalny / original research article

with recommendations for a follow-up visit and ingestion of ceration, trismus, bleeding, ear pain, dysphagia, dyspnea, teeth thickened food. loss [23]. There are no characteristic symptoms and hence the diagnosis is based mainly on pathology assessment. On mac- On follow-up visits, no recurrence of fibromatosis was observed. roscopic view, it looks like a gray and white mass as present- ed in this case. On microscopic view, fibromatosis presents as an infiltration of well-differentiated fibroblasts, fibrocytes and DISCUSSION myofibroblasts suspended in a collagen or mucous matrix. No atypical mitoses or anaplastic elements are seen [8]. Such mor- Due to a deep location of the parapharyngeal space, tumors phological presentation allowed to make this diagnosis in our occupying this space tend to grow asymptomatically for a long patient. The radiological image is different depending on the time. Moreover, this location is associated with poor evalua- histological content of the tumor [14]. On ultrasound, fibroma- tion on standard laryngological examination or commonly ap- tosis presents as a homogenous hypoechogenic mass with var- plied ultrasound of the neck. Also, the surgical access to the ying vasculature on Doppler ultrasound [14]. On a computed parapharyngeal space is challenging for the operator, and the tomography scan, a homogenous infiltrating mass of soft tissue presence of nervous and vascular structures poses a risk of with slight enhancement following contrast administration can postoperative complications. be seen [9,11]. Magnetic resonance imaging shows a heterogene- ous cluster of soft tissue extending along fascia and sometimes The most common parapharyngeal space tumor is the multi- infiltrating adjacent vascular and nervous structures [9,11]. A form adenoma arising from the deep lobe of the parotid gland helpful feature is a presence of non-enhancing collagen fibers [16]. The most common clinical presentation of the parapharyn- with low T1- and T2-weighted signals [9,11]. Despite the fact geal space tumors is medial displacement of the lateral phar- that diagnostic imaging studies allow to diagnose fibromatosis, yngeal wall [17]. Tumors can, however, reach large size in this computed tomography and magnetic resonance can be useful location while remaining asymptomatic [18]. for evaluation of infiltration of surrounding anatomical structures, which is important when planning surgical intervention. Asymmetric displacement of the lateral wall of the oropharynx can be misdiagnosed as an inflammation or cancer of the pal- It is thought that the only effective treatment of desmoid-type fi- atine tonsil, which often results in unnecessary tonsillectomy. bromatosis is to surgically remove the tumor mass with healthy Symptoms suggesting malignancy include cranial nerve (facial, tissue margins [24,25]. There is a high risk of relapse in extraab- glossopharyngeal, vagus, hypoglossal) dysfunction. Also, rapid dominal fibromatosis, especially when head and neck are involved growth and concurrent pain suggest malignancy [18]. Because (40-70%), and most recurrences occur within 18 months since re- it is difficult to assess parapharyngeal masses on physical -ex section [26]. When total resection is impossible or surgery is as- amination, computed tomography and magnetic resonance sociated with a risk of unacceptable cosmetic defects and compli- imaging play an important role In diagnosis. Fine-needle as- cations, radiotherapy and chemotherapy may be considered [25]. piration biopsy (FNAB) of parapharyngeal tumors is possible and both the sensitivity and specificity of this methods is Nyuttens et al. compared cases of fibromatosis treated with very high according to some authors, reaching 96% and 99% surgical intervention only with those, in which surgery was respectively [19]. The risk of complications such as bleeding combined with adjuvant radiotherapy, indicating greater effi- and spreading cancer cells is significantly lower in the case of cacy of the latter scheme in terms of the disease-free survival FNAB compared to tissue sampling [20]. Final diagnosis is [27]. However, studies by other authors did not support this based on pathology evaluation. hypothesis [28]. Radiotherapy is associated with numerous side effects, especially in the head and neck region, hence the Extraabdominal form of fibromatosis occurs in one out of three controversy surrounding the issue of its application in benign patients affected by this disease. It is most commonly located tumors. Chemotherapy and radiotherapy are considered in the in the arm or thigh [21]. It rarely involves the head and neck case of unresectable tumors [25]. Sze et al. reported decreas- (only 5-10% of cases of extraabdominal fibrosis) [22]. Neoplas- ing tumor size in a patient with severe obstruction of the res- tic growth in fibromatosis can, however, reach considerable piratory tract when administered low-dose methotrexate and size and infiltrate surrounding tissues, compressing them and vinblastine [29]. Other authors achieved inhibition of rapidly causing their destruction. Head and neck location is associat- growing fibromatosis after administration of tamoxifen [30]. ed with more severe course and poorer prognosis due to vital structures found in this location [22]. Oral fibromatosis presents Despite rare occurrence of fibromatosis in the head and neck as a painless mass or edema. Rarer manifestations include ul- region, it should be considered in differential diagnosis.

POLSKI PRZEGLĄD OTORYNOLARYNGOLOGICZNY, TOM 6, NR 2 (2017), s. ??-?? 47 artykuł oryginalny / original research article

References

1. Wysocki J., Wanyura H., Krasny K., Szymański I.: Anatomia kliniczna przestrzeni przygardłowej. Journal of Stomatology. 2003; 56 (3): 199–208. 2. Aleksandrowicz R., Ciszek B.: Anatomia Kliniczna Głowy i Szyi. Warszawa, PZWL 2007: 535–538. 3. Hugues III K.V., Olsen K.D., McCafferey T.V.: Parapharyngeal space neoplasms. Head Neck. 1995; 17 (2): 124–130. 4. Olsen K.D.: Tumors and surgery of the parapharyngeal space. Laryngoscope. 1994; 104 (Suppl. 63): 1–28. 5. Pang K.P., Goh C.H., Tan H.M.: Parapharyngeal space tumors: an 18 year review. J. Laryngol. Otol. 2002; 116: 170–175. 6. Ritesh S., Rashmi J., Nita K., Shelly C., Ashish M.K.: Para pharyngeal space tumors: A review of 10-year experience. Internet J. Pathol. 2009; 8: 2. 7. Micke O., Seegenschmiedt M.H.: German Cooperative Group on Radiotherapy for Benign Diseases. Radiation therapy for aggressive fibromatosis (desmoid tumors): Results of a national Patterns of Care Study. Int. J. Radiat. Oncol. Biol. Phys. 2005; 61: 882–891. 8. Angiero F., Benedicenti S., Stefani M.: Fibromatosis of the Head and Neck: Morphological, Immunohistochemical and Clinical Features. Anticancer Rese- arch. 2008; 28: 1725–1732. 9. McDonald E., Yi E., Wenger D.: Best cases from the AFIP: extraabdominal desmoid-type fibromatosis. Radiographics. 2008; 28: 901–906. 10. Fibromatoses. W: Weiss S.W., Goldblum J.R., eds. Enzinger and Weiss’s soft tissue tumors. 4th ed. St Louis, Mo: Mosby. 2001; 320–329. 11. Wong S.: Diagnosis and management of desmoid tumors and fibrosarcoma. J. Surg. Oncol. 2008; 97: 554–558. 12. Slowik V., Attard T., Dai H., Shah R.,Septer S. et al.: Desmoid tumors complicating Familial Adenomatous Polyposis: a meta-analysis mutation spectrum of affected individuals. BMC Gastroenterology. 2015; 15: 84. 13. Kumar V., Khanna S., Khanna A.K., Khanna R.: Desmoid tumors: Experience of 32 cases and review of the literature. Indian. J. Cancer. 2009; 46: 34–39. 14. Al-Saraf A. et al.: Desmoid-Type Fibromatosis of the Tongue. Head and Neck Pathol. 2016; 10: 527–529. 15. Dhingra K.: Antiestrogens – tamoxifen, SERMs and beyond. Invest. New Drugs. 1999; 17: 285–311. 16. Gangopadhyay M., Bandopadhyay A., Sinha S., Chakroborty S.: Clinicopathologic study of parapharyngeal tumors. J. Cytol. 2012 Jan–Mar; 29 (1): 26–29. 17. Singh M1, Gupta SC, Singla A.:Our experiences with parapharyngeal space tumors and systematic review of the literature. Indian. J. Otolaryngol. Head Neck Surg. 2009; 61: 112–119. 18. Metgudmath R.: Surgical Management of Parapharyngeal Space Tumors: Our Experience. Indian. J. Otolaryngol. Head Neck Surg. 2013 Jul.; 65: 64–68. 19. Mondal P., Basu N., Gupta S., Bhattacharya N., Mallick M.G.:et al.: Fine needle aspiration cytology of parapharyngeal tumors. J. Cytol. 2009 Jul.–Sep.; 26 (3): 102–104. 20. Ferro M.F. et al.: Surgical treatment of benign parapharyngeal space tumours. Presentation of two clinical cases and revision of the literature. Med. Oral Pa- tol. Oral Cir. Bucal. 2008 Jan 1; 13 (1): 61–64. 21. Niv A. et al.: Desmoid tumor (aggresive fibromatosis) of the Neck. Indian Journal of Otolaryngology and Head and Neck Surgery. 2000; Vol 52, No 2. 22. Wang C.P. et al.: Desmoid tumor of the head and neck. Head Neck. 2006; 28: 1008–1013. 23. Fowler C.B., Hartman K.S., Brannon R.B.: Fibromatosis of the oral and paraoral region. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 1994; 77: 373–388. 24. Ramamurthy R. et al.: Recurrence Patterns and Management Options in Aggressive Fibromatosis. Indian J. Surg. Oncol. (September 2012); 3 (3): 222–227. 25. Kasper B., Ströbel P., Hohenberger P.: Desmoid Tumors: Clinical Features and Treatment Options for Advanced Disease. The Oncologist. 2011; 16: 682–693. 26. Kruse A.L., Luebbers H.T., Grätz K.W. et al.: Aggressive fibromatosis of the head and neck: a new classification based on a literature review over 40 years (1968–2008). Obwegeser. Oral Maxillofac. Surg. 2010; 14: 227–232. 27. Nuyttens J.J., Rust P.F., Thomas C.R. Jr., Turrisi A.T. 3rd.: Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A com- parative review of 22 articles. Cancer. 2000; 88: 1517–1523. 28. Constantinidou A., Jones R.L., Scurr M., Al-Muderis O., Judson I.: Advanced aggressive fibromatosis: Effective palliation with chemotherapy. Acta Oncol. 2011; 50: 455–461. 29. Sze H., Yeung M.W.: Fibromatosis of the neck causing airway obstruction managed effectively with weekly low-dose methotrexate and vinblastine. Hong Kong Med. J. 2009; 15: 221–223. 30. Kinzbrunner B., Ritter S., Domingo J. et al.: Remission of rapidly growing desmoids tumors after tamoxifen therapy. Cancer. 1983; 52: 2201–2204.

48 WWW.OTORHINOLARYNGOLOGYPL.COM artykuł oryginalny / original research article

Word count: 2000 Tables: – Figures: – References: 30

Access the article online: DOI: 10.5604/01.3001.0010.0179

Table of content: http://otorhinolaryngologypl.com/resources/html/articlesList?issueId=10000

Corresponding author: Anna Rzepakowska, Ul. Banacha 1a, 02-097 Warszawa, +48 22 599 27 16, [email protected]

Competing interests: The authors declare that they have no competing interests.

Cite this article as: Piotrowska A., Rzepakowska A., Chęciński P., Niemczyk K.,: Fibromatosis aggressiva of parapharyngeal space – a case report and review of literature; Pol Otorhino Rev 2017; 6(2): 45-49

POLSKI PRZEGLĄD OTORYNOLARYNGOLOGICZNY, TOM 6, NR 2 (2017), s. ??-?? 49