A Rare Case of Malignant Fibrous Histiocytoma (Undifferentiated High-Grade Pleomorphic Sarcoma) of Malar Region E´Der A

The Journal of Craniofacial Surgery Volume 28, Number 3, May 2017 Brief Clinical Studies

A Rare Case of Malignant Fibrous Histiocytoma (Undifferentiated High-Grade Pleomorphic Sarcoma) of Malar Region E´der A. Sigua-Rodriguez, DDS, MSc, FIGURE 1. (A) Coronal section of paranasal computed tomography showing bilateral inferior and middle concha bullosa and pneumatization of crista galli. Douglas Rangel Goulart, DDS, PhD, (B) The endoscopic view of turbinates during surgery. Afonso Celso de Moraes Manzano, MD, MSc,y and Luciana Asprino, DDS, PhD

Although the asymptomatic patients do not require treatment, Abstract: Malignant fibrous histiocytoma is a sarcoma with rare outfracture, resection of lateral lamella, and submucous resection occurrence in the oral and maxillofacial region; surgery is the 10,11 are the surgical treatment options for inferior CB. Our patient most reliable treatment. Inadequate resection of the sarcoma on was 13 years old and for bilateral bullosa of the middle and inferior the oral and maxillofacial region is associated with a high conchas we resected the lateral lamella of the middle conchas and incidence of local recurrence and a poor prognosis. Only few performed submucous resection and lateralization for the inferior conchas. patients of malignant fibrous histiocytoma of the malar region Concha bullosa of both middle and (very rarely) inferior turbi- have been previously reported in the literature. The authors nates may take up too much space due to their enlargement with air. report a new patient of malignant fibrous histiocytoma on the They may well contribute to obstruction and impaired nasal breath- right malar region that treated a complete tumor surgical ing. Clinicians do not consider CB for the etiology for nasal excision without lymph node dissection. Examination of the obstruction in children. For this reason, our patient was misdiag- resected specimen revealed that the tumor was completely nosed for 3 years and all clinicians gave the diagnosis as adenoid removed. hypertrophy or concha hypertrophy. Therefore, the pneumatization of nasal structures should be evaluated well on the scans for any resistant nasal obstruction etiology or before sinus surgery is Key Words: Head and neck neoplasms, malignant fibrous undertaken in children. histiocytoma, sarcoma

alignant fibrous histiocytoma (MFH) was first reported as CONCLUSION M malignant fibrous xanthoma by O’Brien and Stout1 in In the patients presenting with nasal obstruction who do not respond 1964. It is a high grade and aggressive sarcoma and is considered to local treatments, turbinate pneumatizations should be kept in the most common soft tissue sarcoma in adults,2 being most mind even in pediatric patients. Computed tomography imaging is a commonly located in the extremities and retroperitoneum and valuable tool in making the diagnosis. relatively uncommon in the head and neck region, when detected, it has been observed in the nasal sinus, salivary gland, oral cavity, 3 REFERENCES mandible, larynx, auricula, and eyelid and occurs most commonly in accessory nasal sinuses, followed by the salivary 1. Bolger WE. Anatomy of the paranasal sinuses. In: Kennedy DW, Bolger glands.4 WE, Zinreich SJ, eds. Disease of the Sinuses. London, UK: BC Decker; Surgery is the most reliable treatment for MFH, adjuvant 2001:1–12 2. Ozturan O, Yenigun A, Degirmenci N, et al. ‘Conchae bullosis’: a rare chemotherapy is considered in high-grade tumors because these case with bilateral triple turbinate pneumatisations. J Laryngol Otol tumors may present subclinical or microscopic metastases at the 5 2013;127:73–75 time of diagnosis. Survival rate for MFH in the head and neck is 3. Giourgos G, Matti E, Carena P, et al. A unique case of multiple sites of low compared with MFH in the extremities and trunk.3 It is rarely pneumatization of the sinonasal bony framework in a pediatric patient. diagnosed before excision and has an unfavorable prognostic in Ear Nose Throat J 2010;89:E10–E11 some patients.6 Here, we report a rare case of a woman with 4. Hebert RL, Bent JP. Meta-analysis of outcomes of pediatric functional cutaneous malignant fibrous histiocytoma on the right malar endoscopic sinus surgery. Laryngoscope 1998;108:796–799 region. 5. Unlu HH, Akyar S, Caylan R, et al. Concha bullosa. J Otolaryngol 1994;23:23–27 6. Hilberg O, Grymer LF, Pedersen OF, et al. Turbinate hypertrophy. Evaluation of the nasal cavity by acoustic rhinometry. Arch From the Department of Oral and Maxillofacial Surgery, Piracicaba Otolaryngol Head Neck Surg 1990;116:283–289 Dental School, State University of Campinas (UNICAMP), Piracicaba; 7. Ingram WA, Richardson BE. Concha bullosa of an inferior turbinate. and yDepartment of Head and Neck Surgery, Santa Casa of Limeira Ear Nose Throat J 2003;82:605–607 Hospital, Limeira, Brazil. 8. Kantarci M, Karasen RM, Alper F, et al. Remarkable anatomic Received November 7, 2016. variations in paranasal sinus region and their clinical importance. Accepted for publication December 12, 2016. Eur J Radiol. 2004;50:296–302 Address correspondence and reprint requests to Douglas Rangel Goulart, 9. Yang BT, Chong VFH, Wang ZC, et al. CT appearance of pneumatized DDS, PhD, State University of Campinas, Piracicaba, Brazil; inferior turbinate. Clin Radiol 2008;63:901–905 E-mail: [email protected] 10. Braun H, Stammberger H. Pneumatization of turbinates. Laryngoscope The authors report no conflicts of interest. 2003;113:668–672 Copyright # 2017 by Mutaz B. Habal, MD 11. Woolford TJ, Jones NS. A concha bullosa crusher for use in endoscopic ISSN: 1049-2275 sinus surgery. J Laryngol Otol 2000;114:205–206 DOI: 10.1097/SCS.0000000000003566

# 2017 Mutaz B. Habal, MD e267 Copyright © 2017 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. Brief Clinical Studies The Journal of Craniofacial Surgery Volume 28, Number 3, May 2017

‘‘poorly differentiated malignant neoplasm.’’ The physical examination showed a neoplastic aspect of the skin lesion in the right malar with central necrosis, and without fixation to deep tissues (Fig. 1A). It measured about 4 cm in diameter with no lymph node enlargement on the parotid or on the cervical region.

Surgical Technique The patient underwent a surgery resection to ensure good access to the tumor and wide exposure, an incision was made around the lesion with safety margins of 2 cm and residual defect was repaired with dermocutaneous flap from the cervical region that was rotated superiorly to obliterate the defect (Fig. 1B). Postoperatively, the flap developed with partial necrosis and was performed debridement of the necrotic area (Fig. 1C), leaving pro- gress with granulation and healing by second intention of the exposed area; the flap was healthy and the patient recovered well (Fig. 1D). Postoperative pathologic examination confirmed that the surgical margin was negative. Findings on histopathology of the excised specimen were consistent with a malignant fibrous histiocytoma of storiform-variety (Fig. 2).

DISCUSSION The cause of MFH has not been identified in many patients in the 7 FIGURE 1. Surgical procedure and follow-up. (A) Neoplastic aspect of the skin literature, but some patients developed MFH after radiotherapy. lesion with central necrosis. (B) Dermocutaneous flap design. (C) There was no history of radiotherapy in our patient. The treatment Postoperatively, flap with partial necrosis. (D) Granulation and healing by performed was complete tumor surgical excision without lymph second intention of the exposed area. node dissection, because regional lymph node metastases are present in fewer than 15% of patients; lymph node dissection is not indicated in the absence of palpable cervical metastases, with the exception of tumors in the oral cavity, which appear to have a higher incidence of regional nodal involvement.7 In the case described in this article, we opted to perform only the surgical treatment; the effectiveness of surgery in combination with radiotherapy and/or chemotherapy has not been well established and few studies estimate the survival benefits of chemotherapy.8 The examination of the resected specimen revealed that the tumor had been completely removed; it is important to cite that FIGURE 2. Histological section shows a typical malignant fibrous histiocytoma inadequate resection of the sarcoma in the head and neck was featuring spindle cells arranged in a storiform pattern (hematoxylin and associated with a high incidence of local recurrence (85%) and a eosin, 200). poor prognosis, whereas the recurrence rate with radical resection was only 27%.9 The generally poorer prognosis for MFH of the CLINICAL REPORT head and neck reflects the greater difficulty in extensive radical resection of a tumor in the head and neck, compared with other Clinical History regions, due to interactions with surrounding organs.3 A 78-year-old female patient presented with the complaint of an Malignant fibrous histiocytoma is subclassified on the basis of ulcerated lesion and swelling on the right side of face that appeared a histologic appearance and biologic behavior into storiform-pleo- year ago. The lesion was associated with pain and had a progressive morphic, myxoid, giant cell, inflammatory, and angiomatoid types increasing. The patient has a personal history of hypertension, (Table 1). The storiform-pleomorphic subtype had historically diabetes mellitus, and lower limb amputation. Reviewing of the comprised the majority of MFH patients, accounting for up to clinical notes that were done by another institution, where the patient 70% of all reported patients. It is typically composed of a mixture of had undergone initial treatment of biopsy, showed a diagnosis of spindle cells and polygonal or rounded cells, arranged in a storiform

TABLE 1. Nomenclature and Categorization of Malignant Fibrous Histiocytoma Subtypes (2002 World Health Organization Classification)

Old Nomenclature of MFH Subtype Current Nomenclature of MFH Subtype Tumor Category

Storiform-pleomorphic MFH Undifferentiated high-grade pleomorphic sarcoma Fibrohistiocytic Myxoid MFH Myxofibrosarcoma Myofibroblastic Giant cell MFH Undifferentiated pleomorphic sarcoma with giant cells Fibrohistiocytic Inflammatory MFH Undifferentiated pleomorphic sarcoma with prominent inflammation Fibrohistiocytic Angiomatoid MFH Angiomatoid fibrous histiocytoma Tumors of uncertain differentiation

MFH, malignant fibrous histiocytoma. e268 # 2017 Mutaz B. Habal, MD Copyright © 2017 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. The Journal of Craniofacial Surgery Volume 28, Number 3, May 2017 Brief Clinical Studies pattern (Fig. 2). The tumor is usually of high grade containing and the formation of multiple abscesses and sinus tracts that may additional bizarre, multinucleated giant cells, as well as a marked discharge sulfur granules. Actinomycosis that presents on the skin 8 cellularity, nuclear pleomorphism, and abundant atypical mitoses. without endogenous origin is called primary cutaneous actinomy- Prognosis differs according to morphologic subtypes of MFH, for cosis, and the occurrence and treatment of primary cutaneous example the myxoid type has a higher rate of local recurrence with actinomycosis is rarely reported. This report describes the treatment approximately 50% to 60% of patients and gives rise to metastases with an overall risk of 20% to 35%.10 In this case report, the patient of primary cervicofacial actinomycosis with a literature review, and was diagnosed as storiform-variety, the variants of MFH are emphasizes the importance of surgical option for complete remis- important because of prognostic implications. sion of the disease. Storiform-pleomorphic MFH is usually found on the lower limbs of elderly patients (sixth and seventh decades) and only a Key Words: Actinomycosis, cervicofacial actinomycosis, few patients of malignant fibrous histiocytoma of the malar region cutaneous actinomycosis have been previously reported in the literature. An alternate name of undifferentiated high-grade pleomorphic sarcoma is being advo- cated by the WHO in its classification of soft tissue tumors ctinomycosis is an infectious bacterial disease caused by (Table 1). The tumor is still classified under the category of A Actinomyces species such as Actinomyces israelii or A ger- fibrohistiocytic tumors.11,12 encseriae. This disease is very rare and is characterized by con- tiguous spreading, subacute to chronic granulomatous inflammation and the formation of multiple abscesses and sinus tracts that may REFERENCES discharge sulfur granules.1 1. O’Brien JE, Stout AP. Malignant fibrous xanthomas. Cancer Actinomycetes are normal flora in the oral cavity, gastrointes- 1964;17:1445–1455 tinal tract, respiratory tract, and female genital tract. This organism 2. Singh B, Shaha A, Har-El G. Malignant fibrous histiocytoma of the head itself is not virulent, but if there is a breakdown of the mucosa in and neck. J Craniomaxillofac Surg 1993;21:262–265 which it exists, infection occurs. 3. Nagano H, Deguchi K, Kurono Y. Malignant fibrous histiocytoma of the Actinomycosis that presents on the skin without endogenous bucca: a case report. Auris Nasus Larynx 2008;35:165–169 4. Barnes L, Kanbour A. Malignant fibrous histiocytoma of the head and origin is called primary cutaneous actinomycosis. Although acti- neck. A report of 12 cases. Arch Otolaryngology Head Neck Surg nomycosis can occur in various sites including the cervicofacial 1988;114:1149–1156 area, thoracic area, abdomen, and pelvis, the occurrence and treat- 1–8 5. Pereira CM, Jorge J Jr, Di Hipolito O Jr et al. Primary intraosseous ment of primary cutaneous actinomycosis is rarely reported. fibrosarcoma of jaw. Int J Oral Maxillofac Surg 2005;34:579–581 Herein, we will discuss a case of pure primary cutaneous 6. Hafner J, Schutz K, Morgenthaler W, et al. Micrographic surgery (’slow actinomycosis colonized as a mass-like lesion, which was con- Mohs’) in cutaneous sarcomas. Dermatology 1999;198:37–43 firmed with immunohistochemical staining and treated with proper 7. AL-Salihi KA, Samsudin AR. Malignant fibrous histiocytoma (MFH) of surgical procedure. the mandible and submandibular salivary gland: report of a case. Oral Oncol Extra 2004;40:123–125 8. Matushansky I, Charytonowicz E, Mills J, et al. MFH classification: CLINICAL PRESENTATION differentiating undifferentiated pleomorphic sarcoma in the 21st A 72-year-old woman visited the otolaryngology department in our Century. Expert Rev Anticancer Ther 2009;9:1135–1144 hospital and then transferred to our outpatient clinic for a palpable 9. Sabesan T, Xuexi W, Yongfa Q, et al. Malignant fibrous mass on her right cheek that appeared 1 month previously. There histiocytoma: outcome of tumours in the head and neck compared was no previous history of trauma or surgery to the orofacial area. with those in the trunk and extremities. Br J Oral Maxillofac Surg 2006;44:209–212 The lesion was a soft, tender, and elevated mass, approximately 10. Weiss SW, Enzinger FM. Myxoid variant of malignant fibrous 2 1 cm in size, on the right nasojugal area (Fig. 1 upper left). A histiocytoma. Cancer 1977;39:1672–1685 preoperative computed tomographic scan showed a 2 2.5-cm- 11. Fletcher C, Unni K, Mertens F (Eds). World Health Organization sized skin mass connected with an enhancing soft tissue mass at the Classification of Tumors. Pathology and Genetics of Tumours of Soft right nasolabial fold of the deep subcutaneous fat layer (Fig. 1, Tissue and Bone. Lyon: IARC Press; 2002. upper mid). The preoperative radiologic differential diagnoses of 12. Al-Agha OM, Igbokwe AA. Malignant fibrous histiocytoma: between each were an infected epidermal inclusion cyst and a malignant the past and the present. Arch Pathol Lab Med 2008;132:1030–1035 tumor. Under local anesthesia, surgical excision and biopsy were done, and the 2 2.5 cm mass was completely excised (Fig. 1, upper right). Chronic inflammation with abscess and bacterial colonies were seen with hematoxylin and eosin staining (Fig. 1, lower left), and positivity for tissue gram stain (Brown–Brenn stain) was observed upon immunohistochemical staining (Fig. 1, Cervicofacial Primary Cutaneous lower right). Microscopic and immunohistochemical examinations Actinomycosis: Surgical From the Department of Plastic and Reconstructive Surgery, College of Treatment for Complete Medicine, The Catholic University of Korea, Seoul, Korea. Received August 19, 2016. Remission of the Disease Accepted for publication November 28, 2016. Address correspondence and reprint requests to Hyung-Sup Shim, MD, Yeon Jin Jeong, MD, Hyo Wan Suh, MD, PhD, Department of Plastic and Reconstructive Surgery, Uijeongbu St. and Hyung-Sup Shim, MD, PhD Mary’s Hospital, The Catholic University of Korea, 271 Seoul, Korea; E-mail: [email protected] Abstract: Actinomycosis, an infectious bacterial disease caused by The authors report no conflicts of interest. Copyright # 2017 by Mutaz B. Habal, MD Actinomyces species, is very rare and is characterized by contig- ISSN: 1049-2275 uous spreading, subacute to chronic granulomatous inflammation DOI: 10.1097/SCS.0000000000003518