Pleomorphic liposarcoma of the head and neck: Presentation of two cases and literature review

Pleomorphic liposarcoma of the head and neck: Presentation of two cases and literature review

Accepted Manuscript Pleomorphic liposarcoma of the head and neck: Presentation of two cases and literature review Jay Agarwal, Sameep Kadakia, Abbas ...

1MB Sizes 4 Downloads 160 Views

Accepted Manuscript Pleomorphic liposarcoma of the head and neck: Presentation of two cases and literature review

Jay Agarwal, Sameep Kadakia, Abbas Agaimy, Anna Ogadzanov, Azita Khorsandi, Raymond L. Chai PII: DOI: Reference:

S0196-0709(17)30246-6 doi: 10.1016/j.amjoto.2017.04.012 YAJOT 1853

To appear in: Received date:

26 March 2017

Please cite this article as: Jay Agarwal, Sameep Kadakia, Abbas Agaimy, Anna Ogadzanov, Azita Khorsandi, Raymond L. Chai , Pleomorphic liposarcoma of the head and neck: Presentation of two cases and literature review. The address for the corresponding author was captured as affiliation for all authors. Please check if appropriate. Yajot(2017), doi: 10.1016/j.amjoto.2017.04.012

This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

ACCEPTED MANUSCRIPT Pleomorphic Liposarcoma of the Head and Neck: Presentation of Two Cases and Literature Review

T

Jay Agarwal, MD Resident Physician Department of Otolaryngology- Head and Neck Surgery New York Eye and Ear Infirmary of Mount Sinai New York, NY [email protected]

US

CR

IP

Sameep Kadakia, MD (Corresponding Author) Resident Physician Department of Otolaryngology- Head and Neck Surgery New York Eye and Ear Infirmary of Mount Sinai 310 e 14th st New York, NY 10003 [email protected]

M

AN

Abbas Agaimy, MD Attending Physician Department of Pathology Erlangen University Hospital Erlangen, Germany [email protected]

PT

ED

Anna Ogadzanov, MD Attending Physician Department of Otolaryngology-Head and Neck Surgery University Hospital of Erlangen Erlangen, Germany [email protected]

AC

CE

Azita Khorsandi, MD Attending Physician Department of Radiology New York Eye and Ear Infirmary of Mount Sinai New York, NY [email protected] Raymond L. Chai, MD, FACS Assistant Professor Department of Otolaryngology Icahn School of Medicine at Mount Sinai New York, NY [email protected] No conflicts of Interest No financial disclosures

ACCEPTED MANUSCRIPT

ABSTRACT Background: Pleomorphic liposarcoma (PL) is an exceedingly rare tumor of the head and neck. This aggressive liposarcoma variant portends a poorer prognosis compared to more typical sarcomatous tumors.

CR

IP

T

Methods: Multi-institutional study including two cases of PL, the first case occurring in the post-auricular region and the second in the cheek with later recurrence within the parotid bed.

US

Results: Both patients were treated surgically with wide local excision. One patient required reoperation to obtain negative margins. The other patient underwent a total parotidectomy with neck dissection for recurrent intraparotid nodal disease. Both patients underwent adjuvant radiotherapy. No patient had evidence of recurrent disease with minimum one year follow-up.

AC

CE

PT

ED

M

AN

Conclusion: Although the literature is sparse on this disease, wide surgical extirpation with negative margins and adjuvant radiation is recommended. Increased reports of this pathology can be helpful in providing practitioners with experienced-based information that can aid in early detection and treatment. We present the first case series of PL of the head and neck in the literature.

ACCEPTED MANUSCRIPT

1.1 Introduction

CR

IP

T

Pleomorphic liposarcomas (PL) are an exceedingly rare type of tumor in the head and neck region and can present variably depending on the subsite within the head and neck region. Although rare, as head and neck surgeons, it is important to have an understanding of the presentation, evaluation and management of these tumors. In this care series, we present two cases of PL arising in different head and neck subsites along with a general overview of the current literature on the subject. To our knowledge, this is the first series of head and neck pleomorphic liposarcomas in the English literature. 2.1 Methods

US

Two cases of PL from different institutions will be reviewed. Each patient’s presentation, workup, and management will be presented along with relevant pathology and imaging.

AN

3.1 Results 3.2 Case Presentation #1

PT

ED

M

A 60-year-old male with a history of human immunodeficiency virus (HIV) presented to the office with a 1-year history of a left post-auricular mass, which he feels had grown in the past several months. The patient reported no associated symptoms aside from a transient episode of pain, which resolved without intervention. He denied infectious or neurosensory symptoms. The patient has no history of tobacco or alcohol abuse.

CE

The patient underwent computed tomography (CT) of the neck with intravenous contrast, which showed a 3.2 cm poorly defined, rim enhancing cystic mass without underlying mastoid involvement. Figure A displays a representative axial image from this patient.

AC

The patient was taken to the operating room for resection of the post-auricular mass, including periosteum of the temporal/occipital bone along with portions of the sternocleidomastoid muscle, trapezius, and sub-occipital fat. There was no evidence of bony erosion at the time of surgery. Preliminary analysis of the specimen showed a malignant spindle cell neoplasm with a significant liposarcomatous component. MDM2 staining was negative. Final pathologic diagnosis was pleomorphic liposarcoma. The patient then underwent a staging PET-CT scan which showed no evidence of locoregional or distant disease. As the final pathology from the first surgery demonstrated focal involvement of the periosteum overlying the temporal bone, the patient was taken back to the operating room for a revision procedure including wide resection of the postauricular region including the scar, subcutaneous margins, and a drill-out of the outer cortex of the temporal bone. The subsequent defect was reconstructed with a superiorly based trapezius myocutaneous flap.

ACCEPTED MANUSCRIPT

The patient’s post-operative course was uneventful. He later underwent adjuvant radiation. He continues to be free of disease at one year follow-up. 3.3 Case Presentation #2

IP

T

A 56-year-old male presented to the office with a progressively growing subcutaneous mass of the left cheek. An excisional biopsy was performed on this specimen and found to be a PL. Following diagnosis of malignancy, a repeat wide local excision was performed to obtain adequate oncologic margins. No adjuvant therapy was performed and the patient did well in the interim.

AN

US

CR

Six months following surgery, the patient returned to the office with an ipsilateral mass in the tail of the parotid gland. A core needle biopsy was performed, revealing metastatic PL. A total parotidectomy with levels II-V selective neck dissection was performed, revealing cervical metastasis in a 1.1 cm single intraparotid lymph node without extracapsular spread. Figure B shows a photograph of the malignant focus in the intraparotid lymph node biopsy. Figure C shows a magnified view of the pathology. There was no residual disease in the parenchyma of the parotid gland other than the involved intraparotid node and the remaining lymphadenectomy specimen was free of disease as well.

M

Adjuvant radiotherapy was performed for this patient and at one year post-treatment, he has been doing well with no signs of recurrence.

ED

4.1 Discussion

CE

PT

Sarcomas are rare tumors that can be found throughout the body. Sarcomas specific to the head and neck are even less common and make up about 1% of all sarcomas in adults[1]. Liposarcomas are the most common subtype of sarcomas in adults and can be found throughout the body, however, are only rarely found in the head and neck region (29%)[2]. Because these tumors are so rare in the head and neck, few studies have been performed, making evidence-based management challenging.

AC

As an otolaryngologist, it is important to understand the proper evaluation and management of these rare tumors in the head and neck region. Most liposarcomas are seen in adults, with a mean age of 46 and a predominance in males[3]. However, the aggressive PL subtype is described to be more common in younger patients and females, with a mean age reported in one study to be 30 years old[4]. Associated factors include genetics, previous trauma, previous irradiation, and familial tumor syndromes such as neurofibromatosis[2]. On physical exam, the masses are firm, fixed, slow-growing and usually are not associated with regional lymphadenopathy. Imaging should be obtained to evaluate size, any local extension of the tumor and relationship to other nearby structures. According to one study that evaluated over 300 head and neck liposarcomas, the most common site was in the subcutaneous tissue of the head and neck[5]. Another study showed that liposarcomas located in the oral cavity have better disease-specific survival rates than liposarcomas of the face or scalp[4]. Oral and salivary gland liposarcomas

ACCEPTED MANUSCRIPT typically have a better prognosis than liposarcomas in other soft tissue regions regardless of histological subtype; however, limited data exists on the PL variant[6].

IP

T

There are various grading systems used for liposarcomas described in different studies and most correspond to histologic characteristics. Well-differentiated and myxoid liposarcomas have been shown to be low grade and are the most common type of liposarcomas in the head and neck. Other more aggressive variants include pleomorphic, round cell and dedifferentiated variants, which are much less common in the head and neck. A large retrospective study using a grading system that divides these tumors into three subtypes based on mitotic activity, necrosis and differentiation showed that the majority of head and neck liposarcomas are well differentiated or myxoid and fall within grades 1 or 2[5].

US

CR

PLs, which would be grade 3 on the scale, are exceedingly rare in the head and neck region. Of the head and neck PLs that have been reported, the disease specific survival and overall survival rate compared to other histological subtypes is significantly lower[5]. Studies have shown a 50% mortality rate for both round cell and pleomorphic variants of head and neck liposarcomas within 2 years. These two variants are also the most likely to result in recurrence after treatment[7].

PT

ED

M

AN

Of note, the pathology results for the patient in case 1 showed the MDM2 staining to be negative on preliminary review. Well-differentiated and dedifferentiated liposarcomas have been shown to have similar cytogenic features including amplification of the MDM2 and CDK4 genes. These gene amplifications can be detected on immunostains and have been shown to be useful in distinguishing well-differentiated liposarcomas from differentiated adipose tumors and de-differentiated liposarcomas from poorly differentiated sarcomas[8]. The lack of MDM2 staining could have initially been the reason for a pathological diagnosis of malignant spindle cell tumor prior to final review.

AC

CE

Treatment for head and neck liposarcomas includes wide surgical excision to ensure negative margins. Even though these liposarcomas are usually encapsulated, without wide local excision, microscopic residual disease may be left. Studies have shown that the mortality due to head and neck liposarcomas are more commonly from local recurrence than other sites and as a result, adjuvant radiotherapy should be considered depending on tumor size and histology. One study showed that patients treated with both surgery and radiotherapy, controlled for histological subtype and staging, showed decreased local recurrence at five years than surgery alone[9]. Radiation should especially be considered for those with high-grade tumors, positive margins, large tumors, and complex anatomic subsites. However, due to these tumors being exceedingly rare in the head and neck and the limited data available, the current literature is not consistent and do not stratify which patients truly benefit from multimodality treatment methods. Future prospective studies need to be performed. 5.1 Conclusion Pleomorphic liposarcomas of the head and neck are exceedingly rare tumors with a poor prognosis. Due to the rarity of this tumor, there is limited literature on the disease and as a

ACCEPTED MANUSCRIPT

AC

CE

PT

ED

M

AN

US

CR

IP

T

result, limited data on the ideal management and treatment strategies for these tumors. In this case series, we present two cases of PL in the head and neck region from two separate institutions to help further the understanding of this disease. Future prospective studies are needed to determine the most effective treatment modalities, including the role of chemotherapy and radiation therapy.

ACCEPTED MANUSCRIPT

AC

CE

PT

ED

M

AN

US

CR

IP

T

6.1 References [1] Gritli S, Khamassi K, Lachkhem A, Touati S, Chorfa A, Ben Makhlouf T, et al. Head and neck liposarcomas: A 32 years experience. Auris Nasus Larynx 2010;37:347–51. doi:10.1016/j.anl.2009.08.003. [2] Golledge J, Fisher C, Rhys-Evans PH. Head and neck liposarcoma. Cancer 1995;76:1051–8. [3] Reitan JB, Kaalhus O, Brennhovd IO, Sager EM, Stenwig AE, Talle K. Prognostic factors in liposarcoma. Cancer 1985;55:2482–90. doi:10.1002/10970142(19850515)55:10<2482::AID-CNCR2820551029>3.0.CO;2-R. [4] Davis EC, Ballo MT, Luna MA, Patel SR, Roberts DB, Nong X, et al. Liposarcoma of the head and neck: The University of Texas M. D. Anderson Cancer Center experience. Head Neck 2009;31:28–36. doi:10.1002/HED.20923. [5] Gerry D, Fox NF, Spruill LS, Lentsch EJ. Liposarcoma of the head and neck: analysis of 318 cases with comparison to non-head and neck sites. Head Neck 2014;36:393–400. doi:10.1002/hed.23311. [6] Fanburg-Smith JC, Furlong MA, Childers ELB. Liposarcoma of the Oral and Salivary Gland Region: A Clinicopathologic Study of 18 Cases with Emphasis on Specific Sites, Morphologic Subtypes, and Clinical Outcome. Mod Pathol 2002;15:1020–31. doi:10.1097/01.MP.0000027625.79334.F5. [7] McCulloch TM, Makielski KH, McNutt MA, JG B, Stoller FM DD, Enzinger FM WD, et al. Head and Neck Liposarcoma: A Histopathologic Reevaluation of Reported Cases. Arch Otolaryngol - Head Neck Surg 1992;118:1045–9. doi:10.1001/archotol.1992.01880100035010. [8] Binh MBN, Sastre-Garau X, Guillou L, de Pinieux G, Terrier P, Lagacé R, et al. MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes: a comparative analysis of 559 soft tissue neoplasms with genetic data. Am J Surg Pathol 2005;29:1340–7. [9] Eeles R, Fisher C, A’Hern R, Robinson M, Rhys-Evans P, Henk J, et al. Head and neck sarcomas: prognostic factors and implications for treatment. Br J Cancer 1993;68:201–7. doi:10.1038/bjc.1993.314.

ACCEPTED MANUSCRIPT Figures Captions:

CR

IP

T

Figure A: Rim enhancing centrally hypodense left post auricular lesion directly apposing the adjacent mastoid air cells which are completely intact. This lesion does not extend to the overlying skin and does not invade the external auditory canal. This lesion measures 3.2 x 1.8 x 3.0 cm.

US

Figure B: 10x H&E stain of wholemount of intraparotid lymph node metastasis.

AC

CE

PT

ED

M

AN

Figure C: 40x H&E stain of intraparotid lymph node metastasis showing highly pleomorphic tumor cells with prominent cytoplasmic vacuoles and atypical mitotic figures.

M

AN

US

CR

IP

T

ACCEPTED MANUSCRIPT

AC

CE

PT

ED

Figure A

ED

M

AN

US

CR

IP

T

ACCEPTED MANUSCRIPT

AC

CE

PT

Figure B

ED

M

AN

US

CR

IP

T

ACCEPTED MANUSCRIPT

AC

CE

PT

Figure C