The Journal of Craniofacial Surgery

Brief Clinical Studies

diagnosis with FNA before the operation, and to see that it did not cause any additional symptoms. For limited neck patients, the common treatment is gross total resection by protecting the vagal nerve with a/the lateral cervical approach. For an introthoracic extension of neurogenic tumors that originated in the brachial and cervical plexus, posterior thoracotomy is performed, also with the same lateral cervical approach.8 In this patient, we were able to extract the tumor extending to the anterior and middle mediastinum by left anterior thoracotomy after left lung extinction. No sternotomy was needed. After resection, definitive diagnosis of schwannoma can be made histopathologically and classified by 1 of 2 microscopic patterns. In the Antoni A pattern, Verocay corpuscles are present and hypercellularity and nuclear palisading are also present. Antoni B exhibits a hypocellular pattern consisting mostly of loose tissue. To support the diagnosis of either, S-100 staining can be used. In our case, the tumor was hypercellular and stained thoroughly with S-100. Vagal schwannoma should be considered in the differential diagnosis of a lateral neck mass. Hoarseness is its most known frequent symptom. Considering possible intrathoracic extension beside the neck mass, the neck and thorax should be reviewed together by MR. For intrathoracic extension, a multidisciplinary approach is necessary together with thoracic surgery. Even a tumor with more than a 5 cm intrathoracic extension can be extracted by anterior thoracotomy without sternotomy.

ACKNOWLEDGMENT The authors appreciate the contributions and editorial assistance made by S. Delacroix, a native English speaker.

REFERENCES 1. Colreavy MP, Lacy PD, Hughes J, et al. Head and neck schwannomas: a 10 years review. J Laryngol Otol 2000;114:119–124 2. Whittam DE, Morris TM. Neurilemmoma of the larynx. J Laryngol Otol 1970;84:747–750 3. Langner E, Del Negro A, Akashi HK, et al. Schwannomas in the head and neck: retrospective analysis of 21 patients and review of the literature. Sao Paulo Med J 2007;125:220–222 4. Kanzaki M, Ohtsuka T, Obara T, et al. Surgically treated dumbbell schwannoma arising in the brachial plexus with intrathoracic extension. Jpn J Thorac Cardiovasc Surg 2003;51:62–64 5. Gyhra A, Israel J, Santander C, et al. Schwannoma of the brachial plexus with intrathoracic extension. Thorax 1980;35:703–704 6. Kline DG, Hudson AR. Tumors involving nerve. In: Kline DG, Hudson AR, eds. Nerve Injuries: Operative Results for Major Nerve Injuries, Entrapments, and Tumors. Philadelphia: WB Saunders; 1995:525–574 7. Gilmer-Hill HS, Kline DG. Neurogenic tumors of the cervical vagus nerve: report of four cases and review of the literature. Neurosurgery 2000;46:1498–1503 8. Rubio Correa I, Monje F, Gonza´lez-Garcı´a R, et al. Lateral cervical approach combined with posterior thoracotomy for the treatment of giant neurilemmoma of thechest involving the neck. J Oral Maxillofac Surg 2013;71:433–436

Recurrent Atypical Fibroxanthoma Versus Malignant Fibrous Histiocytoma Leslie G. Branch, MD, John G. Albertini, MD,y and Barry Leshin, MDy

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Abstract: Atypical fibroxanthoma (AFX) has been characterized variously as a benign noninvasive neoplasm with rare recurrence. We report 2 cases of recurrent AFX. There is ongoing controversy over the diagnosis of AFX versus malignant fibrous histiocytoma (MFH) in the clinical context of recurrent AFX. Histopathologic diagnosis of fibrohistiocytic neoplasms can be quite challenging and small foci of cancer can be easily overlooked. This is particularly problematic when scarring associated with recurrent tumors is present. The utility of en face sections, as utilized in Mohs micrographical surgery, and the employment of immunohistochemical stains may be helpful in diagnosis. Recurrence of AFX can occur but is difficult to distinguish from MFH. Key Words: Atypical fibroxanthoma, malignant fibrous histiocytoma, recurrent atypical fibroxanthoma

A

typical fibroxanthoma (AFX) is usually a firm erythematous ulcerated cutaneous nodule measuring 1.5 to 2 cm in diameter. AFX occurs most frequently on sun-exposed areas of the head and neck in elderly Caucasian males.1–4 AFX is thought to be a superficial variant of malignant fibrous histiocytoma (MFH) and is characterized by an indolent growth pattern confined to the dermis. Lesions with similar histologic appearance and immunohistochemical profile but extending into the subcutaneous tissue with necrosis, intravascular invasion, or neural invasion are considered MFH.1,3–7 Others regard AFX and MFH as distinct entities due to their differing risk factors or immunohistochemical markers and have reclassified cases of recurrent AFX as MFH.5,6 We report 2 cases of recurrent AFX and discuss the diagnosis of AFX versus MFH in the clinical context of recurrent AFX.

PATIENT 1 A 73-year-old male patient presented with a 1.1  0.8 cm superficially eroded nodule on the left temple (Fig. 1A) excised by Mohs micrographic surgery (MMS). Two years later, the patient presented with a recurrent 3.5  2.5 cm mass 2 cm below the scar on the left temple. Due to its depth of invasion, the lesion was reclassified as MFH and excised with MMS. The patient has been free of recurrence for 8 months.

PATIENT 2 An 87-year-old male patient presented with a slowly enlarging ulcerated plaque measuring 1.5  1.4 cm on the left concha and antihelix that was previously treated with curettage and electrodessication (Fig. 1B). The lesion was excised with MMS and closed with island pedicle flap from the postauricular sulcus. The patient had 3 recurrences, each about 1  1 cm in size, over the next 10.5 months and every time MMS was used for excision. The last excision included the entire upper half of the ear and AFX with foci of perineural involvement was found. A split-thickness skin From the Department of Plastic and Reconstructive Surgery, Wake Forest Baptist Health, Winston-Salem; and yThe Skin Surgery Center, Winston-Salem, NC. Received June 25, 2014. Accepted for publication January 5, 2015. Address correspondence and reprint requests to Leslie Branch, MD, 1 Medical Center Blvd, Winston-Salem, NC 27157; E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001550 #

2015 Mutaz B. Habal, MD

Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

The Journal of Craniofacial Surgery



Volume 26, Number 4, June 2015

Brief Clinical Studies

that remove all foci of scar tissue and inflammation from previous surgery is particularly important for recurrent tumors like case 2, in which the initial destructive treatment likely created noncontiguous tumor foci accounting for the subsequent multiple recurrences.

REFERENCES

FIGURE 1. Case 1 initial AFX lesion (A). Case 2, initial lesion (B).

graft was then placed over the wound. The patient has been free of recurrence for 9.5 years.

DISCUSSION AFX has been characterized as a benign noninvasive neoplasm and as a low-grade malignant neoplasm with an indolent growth pattern. There is controversy over whether AFX is a distinct entity or a superficial variant of MFH. AFX has been found to have a local recurrence rate ranging from 0% to 20% with rare metastases.1,3,5,6 Multiple local recurrences of AFX as described in Case 2 above have rarely been reported.4 Davidson et al found that tumors extending beyond the dermis had 29.4% chance of local recurrence and 11.8% chance of metastasis.1 Neoplasm extending to resection margins, perineural, intravascular, or deep fascia invasion, necrosis, large size, or an immunocompromised status are all considered to be risk factors for recurrence or metastasis.1,2 Some believe that recurrent AFX is a consequence only of incomplete excision.7 Before each surgery in his report, the slides from the previous recurrence were reviewed by dermatopathology consultants who could not identify misdiagnosed tumor margins. We believe recurrences in our patients were the consequence of false-negative margins arising from noncontiguous growth pattern. Case 1 was reclassified from AFX to MFH at the time of recurrence. This further confirms the difficulty of distinguishing between the 2 entities. Locally invasive or metastasizing lesions may have initially been superficial MFH or other high-grade sarcomas rather than AFX.4–6 MFH has a much higher recurrence rate ranging from 19% to 75% and metastasis from 10% to 55% with 40%-50% recurring within 2 years.5,8,9 Microscopically and immunohistochemically, the 2 lesions are almost indistinguishable.1,7 Both AFX and MFH have pleomorphic spindle cells, atypical mitotic figures, and multinucleated giant cells.3,6 The few minor differences that have been described include fascicular/diploid pattern with ‘‘xanthomatous’’ cytoplasmic vacuolation in AFX and storiform/aneuploid pattern with necrosis in MFH.3,6 AFX is a diagnosis of exclusion, and all the positive markers are very nonspecific. AFX and MFH are negative for S-100, HMB-45, desmin, cytokeratin, and CD34.6 They are both positive for CD10,2,3 vimentin,3,6 and CD68.6 LN-2 stains negatively in 90% of AFX and positive in 90% of MFH, but it is not diagnostic.4 Our cases both stained positively for CD10. LN-2 staining was not performed. Histopathologic diagnosis of fibrohistiocytic neoplasms can be quite challenging. Furthermore, review of resection margins on frozen sections can be difficult where small foci of cancer can be easily overlooked. This is particularly problematic when scarring associated with recurrent tumors is present. There remains some confusion and inconsistency regarding the treatment of AFX. Most patients receive wide local excision, but MMS has been shown to have a lower recurrence rate.1,4,7,8 AFX recurrences may be more common than initially recognized. Larger margins, such as 1-cm margins, may be more beneficial as recommended by some reports.3,5 Wider margins #

2015 Mutaz B. Habal, MD

1. Davidson JS, Demsey D. Atypical fibroxanthoma: clinicopathologic determinants for recurrence and implications for surgical management. J Surg Oncol 2012;105:559–562 2. Mirza B, Weedon D. Atypical fibroxanthoma: a clinicopathological study of 89 cases. Australas J Dermatol 2005;46:235–238 3. Singh M, Mann R, Ilankovan V, et al. Atypical fibroxanthoma—a retrospective immunohistochemical study of 42 cases. J Oral Maxillofac Surg 2012;70:2713–2718 4. Iorizzo LJ 3rd, Brown MD. Atypical fibroxanthoma: a review of the literature. Dermatol Surg 2011;37:146–157 5. Withers AH, Brougham ND, Barber RM, et al. Atypical fibroxanthoma and malignant fibrous histiocytoma. J Plast Reconstr Aesthet Surg 2011;64:e273–e278 6. Ly H, Selva D, James CL, et al. Superficial malignant fibrous histiocytoma presenting as recurrent atypical fibroxanthoma. Australas J Dermatol 2004;45:106–109 7. Gonza´lez-Garcı´a R, Nam-Cha SH, Mun˜oz-Guerra MF, et al. Atypical fibroxanthoma of the head and neck: report of 5 cases. J Oral Maxillofac Surg 2007;65:526–531 8. Hollmig ST, Sachdev R, Cockerell CJ, et al. Spindle cell neoplasms encountered in dermatologic surgery: a review. Dermatol Surg 2012;38:825–850 9. Henderson MT, Hollmig ST. Malignant fibrous histiocytoma: changing perceptions and management challenges. J Am Acad Dermatol 2012;67:1335–1341

A Giant Lymphangioma on the Neck Sedat Aydin, MD, Mehmet Go¨ khan Demir, MD, and Ays¸egu¨l Selek, MDy Abstract: Lymphangioma is a congenital malformation of the lymphatic system, which is located mostly on the head and neck region. Most of the cases are seen at age younger 2 years. It is rare in adult population and when detected, it can be treated with surgical excision. We present a 50-year-old patient with giant lymphangioma on the posterior cervical region of the neck treated by surgical treatment successfully. Key Words: Lymphangioma, cystic hygroma, lymphatic tissue, neck mass, adult From the Ear Nose and Throat Department; and yPathology Department, Kartal Dr. Lutfi Kirdar Education and Research Hospital, Istanbul Turkey. Received July 22, 2014. Accepted for publication January 14, 2015. Address correspondence and reprint requests to Sedat Aydin, MD, istasyon caddesi merdivenli sok o¨zkan apt 5/6 Kartal, Istanbul, Turkey 34860; E-mail: [email protected] The authors report no conflict of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001567

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Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

Recurrent Atypical Fibroxanthoma Versus Malignant Fibrous Histiocytoma.

Atypical fibroxanthoma (AFX) has been characterized variously as a benign noninvasive neoplasm with rare recurrence. We report 2 cases of recurrent AF...
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