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Head and neck and intra-oral soft tissue sarcomas
ORAL ONCOLOGY
PERGAMON
Oral Oncology 34 (1998) 292-296
Head and neck and intra-oral
soft tissue sarcomas
M. Gorsky, J.B. Epstein* British Columbia Cancer Agency, 600 West 10th Avenue, Vancouver, British Columbia, Canada V5Z 4E6
Received 19 November 1997; accepted 25 November 1997
Abstract Intra-oral soft tissue sarcoma is a rare disease. We reviewed a tumour registry over a 45 year period, between January 1951 and January 1997, and identified a total of 11250 head and neck cancers in British Columbia. Of the head and neck cancers, there were 139 cases (1.24%) of sarcoma; and of these, there were only 16 cases (0.14%) of intra-oral soft tissue sarcoma. The initial presentation of intra-oral soft tissue sarcoma is most often as an asymptomatic mass and discomfort is reported by less than one half of the patients. The commonest lesion identified was rhabdomyosarcoma (RMS). In this review, we identified 2 unique cases, 1 case of intra-oral hemangiopericytoma and 1 case of oral carcinosarcoma. The prognosis remains poor. Treatment includes wide surgical excision which may be supplemented with radiotherapy and chemotherapy. 0 1998 Elsevier Science Ltd. All rights reserved. Keywords:
Oral soft tissue sarcoma epidemiology; Diagnosis; Outcome
1. Introduction This paper reviews the literature and presents a case series of sarcomas involving the head and neck, excluding Kaposi’s sarcoma. Soft tissue sarcomas are malignant neoplasms arising in connective tissue, originating from fibrous, fatty, muscular, synovial, vascular, or neural tissue. The tumour, composed of closely packed cells in a fibrillar or homogenous matrix, tends to be vascular and is usually highly invasive. Less than 20% of total body sarcomas occur in the head and neck regions, the most common being rhabdomyosarcoma (RMS). RMS accounts for 18% of the head and neck soft tissue sarcomas [l], followed by malignant fibrous histiocytoma, fibrosarcoma, and neurofibrosarcoma (1 l-16%). All other sarcomas, including unclassified lesions, account for 13% of those found in the head and neck [l]. The histological appearance of the sarcoma lesions is diverse. Furthermore, its accurate diagnosis is challenging and requires the use of specific immunohistochemical procedures and electron microscopy [2]. The treatment of choice is early, radical surgery which includes neck dissection. Intensification of treatment includes autologous bone marrow transplant. Radiation and chemotherapy are mainly used in * Corresponding author. Tel.: 604 877 6000, ext. 2356; fax: 604 872 4596. 1368-8375/98/$19.00 0 1998 Elsevier Science Ltd. All rights reserved. PII: S1368-8375(98)00005-O
combined treatments and for palliation. Combined treatment shows good results in children; as a 3-year survival rate was achieved in 83% of 89 patients following surgery, chemotherapy, and in most cases, radiotherapy [3]. These patients were aged 21 years or less and were diagnosed with localised RMS of orofacial and laryngopharyngeal sites. As revealed in the world literature, there are 100 malignant smooth muscle tumours with only 26 head and neck cases. These 26 cases were mainly reported as unique case presentations [4]. RMS, which is a childhood striated muscle tumour, is one of the most common types of head and neck soft tissue sarcomas [5]. However, there are only a small number of cases presented in the literature. For example, the M.D. Anderson Hospital and Tumor Institute [6] treated just 16 cases of oral RMS over a 40 year period. As presented in the literature, 71.2% of 121 cases of oral RMS were of embryonal origin [5]. The most common site was the palate. Although we searched the English language literature of the last 20years, we failed to detect any study that reviewed the occurrence and the types of oral soft tissue sarcomas in a large group. The purpose of this study was to review the occurrence of head and neck soft tissue sarcomas in British Columbia over the past 45years. We specifically analysed those sarcomas that occurred in the oral cavity.
M. Gorsky, J.B. Epstein/Oral Oncology 34 (1998) 292-296
2. Materials and methods Soft tissue sarcomas of the head and neck were identified from the Tumor Registry and the computer files of the British Columbia Cancer Agency between January 1951 and January 1997. The criteria for the head and neck tumour search included all soft tissue sarcomas involving the following sites: lip, oral cavity, jaws, salivary glands, pharynx, nasal cavity, ear, larynx, and thyroid. However, the search excluded the skull and facial bones. Cases of osteogenic sarcoma, chondrosarcoma, and Kaposi’s sarcoma were excluded from the study. The study included a total of 139 cases of soft tissue sarcoma of the head and neck. Of these, 123 patients had extra-oral soft tissue sarcomas and 16 patients were diagnosed with intra-oral lesions. Of the 16 patients, records were available for 9 patients, but only Registry data were available for 7 patients.
3. Results Between 1972 and 1995, the total number of cases of malignant diseases in British Columbia was 207 005 and for these 23 years complete reliable data were available. Of these total cases of cancer, there were 10 785 (5.21%) recorded cases of head and neck cancer. Over a 45 year period, between January 1951 and January 1997, a total of 11250 cancers were recorded in the head and neck, although the accuracy of this is not assured. Of the head and neck cancers, there were 139 cases (1.24%) of head and neck soft tissue sarcoma; and of these there were only 16 cases (0.14%) of intra-oral soft tissue sarcoma. The patient characteristics of the 139 patients are shown in Table 1. Of these patients, there were 73 males and 66 females. The mean age at diagnosis was 41.7 years and ranged between 1 and 89 years. The data were then measured separately for the 123 cases of extra-oral sarcoma and the 16 cases of intra-oral sarcoma. At the time of diagnosis, the mean age was lower in intra-oral sarcomas; 39.1 years versus 42.1 years in patients with extra-oral head and neck sarcomas. There Table 1 Profile of 139 patients
with head and neck soft tissue sarcomas
Gender Extra-oral sites Male Female Subtotal (male and female) Intra-oral sites Male Female Subtotal (male and female) Total
No.
Mean age (years)
Range
63 60 123
41.1 43.2 42.1
(4-84) (1-89) (1-89)
10 6 16
40.1 37.5 39.2
(12-63)
139
41.7
(l-89)
(3-75)
293
were more men than women in each of the two groups. In the extra-oral sarcoma group there were 63 (51.2%) men and in the intra-oral sarcoma group there were 10 men (62.5%). As seen in Table 2, the nasopharynx was the most commonly involved site and 27 (19.1%) cases were found in this location. The nasal cavity was involved in 20 (14.2%) cases. There were 16 (11.5%) cases of oral soft tissue sarcomas and 13 cases of sarcomas of the parotid gland, which accounts for 10.6% of the nonoral sarcomas. Table 3 presents, in detail, 9 of the 16 cases with soft tissue intra-oral sarcomas for whom the medical records were available. The tongue was involved in 3 cases (33.3%), and the palate in 2 cases (22.2%). The gingiva, the retromolar pad, the buccal mucosa, and the floor of the mouth were diagnosed in 1 patient each (11.1% each). The mean age at diagnosis was 39.6years, ranging from 3 to 75 years. 8 of the patients died and only 2 patients (22.2%) survived. Of the surviving patients, both were initially diagnosed with embryonal RMS, and remained disease-free for a mean time of 15 years. Of the 6 deceased patients, 5 died of their sarcoma in a mean survival time of 1.8 years, 1 patient died from other causes, and 1 patient was lost to follow-up. The youngest patient with oral sarcoma was a 3-yearold boy who had fibrosarcoma of the gingiva and he did not survive his disease. There were two other young children, both with the diagnosis of embryonal RMS, who survived their disease. All cases featured a swelling of the site of malignancy as the first clinical sign and only 5 (45.5%) featured symptoms of pain or soreness. 3 patients had a family history of neoplasms, but no history of sarcomas in the family was reported.
4. Discussion Soft tissue sarcomas account for less than 1% of head and neck neoplasms and have a diversity of type and location. The most common type is RMS (18%). Only 19% of head and neck sarcomas involve the sinonasal cavity, while oral involvement is very rare [l]. The treatment of choice is early and radical surgery. Generally, the response to chemotherapy and irradiation is not sufficient for cure [7], since a high local failure due to indistinct surgical margins occurs, and adjuvant radiotherapy and chemotherapy is often indicated [8]. This study surveyed the head and neck site distribution of soft tissue sarcomas. When a search for previous articles was performed, we failed to find any articles describing site prevalence. We found that almost 50% of our cases were those of the nasal and paranasal region and the mean age at diagnosis was 42 years, 52% being found in males. In a pathological review of 103
75
4
22
14
45
3
73
63
57
M
h4
M
F
F
M
M
F
M
(Years)
Age
Site
16
’
Lateral tongue
Retromolar
Rare of tongue
Gmgiva
Floor of mouth
Retromolar
Palate
Leiomycsarcoma
Lciomyosarcoma
rhabdomyosarcoma Malignant haemangiopericytoma Fibrosarcoma
ElUblyOMl
Embryonal rhabdomyosarcoma Fmbryonal rhabdomyosarcoma
Rhabdomyosarcoma
Diagnosis
17
Ethmoid sinus
soft tissue sarcomas
20
Oral cavity
with soft tissue sarcomas
Nasal cavity
Tongue
with oropharyngeal
27
Nasopharyngeal
No. Gender
Table 3 9 patients
No.
Site
Table 2 139 head and neck sites involved
13
Parotid gland
ccl1
Hodgkin’s
(6)
tonsils (16) None
carcinoma
Scpramous
None
None
None
None
Lymphatic leukaemia (1) None
other neoplasm (years) before
16
Maxillary sinus
M&W
Mass
Mass
Swelling
Mass
Mass
Mass
Signs
6
Pain
Pain
Sore
None
None
None
Pain
None
Sore
_
_
?
+
?
+
Mother
Mother and father
None
Unknown
_ +
None
None
None
None
Family history of cancer
_
_
_
_
Alcohol
6
Sphenoid and accessory sinuses
_
_
+
Tobacco
6
Pharynx and hypopharynx
Symptoms
External lower lip
Local excision
Treatment
3
Glotis and supraglotis
Surgery, irradiation and palliative chemotherapy, disease progressed Partial glossectomy, recurred in 2 years, irradiation with no response
Radiation and chemotherapy
Surgery, irradiation and chemotherapy Irradiation and chemotherapy = no response, surgery = recurrence Chemotherapy and irradiation Surgery. No further information Unknown
4
Thyroid gland
4
2
8.5
0.5
Unknown
12
2
18.5
0.5
(years)
Follow-up
2
Middle ear
Alive, free of disease
Died of disease Alive, free of disease Died of disease
status
1
Tonsils
Died of disease
Died of disease
Died of disease Died, neoplasm of other tissues
2
Larynx
M. Gorsky. J.B. Epstein/Oral Oncology 34 (1998) 292-296
cases, Eeles et al. [9], also found that the age of onset was relatively young (median 32 years), 53% being men. In comparison to carcinoma, the soft tissue sarcoma is a neoplasm involving younger ages, including children and adolescents, in which almost 15% of all sarcomas were seen [lo]. Risk factors have not been identified for these connective tissue malignancies. We did not identify any potential risk factors, and found that only 4 of 10 patients (40%) used tobacco. Most oral soft tissue sarcomas, despite the head and neck predilection of RMS, have been published as isolated case reports. The majority of RMS is diagnosed in young ages and 43% of them are of the embryonal type and occur most commonly in males [5]. We found that the majority of our cases with RMS (75%) were those of embryonal RMS and 3 of 4 were males. Data from a previous study [l l] indicate that the oral manifestation of RMS occurs in approximately 20% of head and neck soft tissue sarcomas. The predominant oral site is the palate [12]. However, others have indicated that the tongue was more commonly involved [13]. Of 142 RMS cases of various sites, only 1.4% were noted in the tongue [14]. Only 1 case of tongue RMS was identified in our current study. RMS is a result of malignant changes of primitive pluripotential mesenchymal cells rather than that of differentiated muscle [15,16]. Although the behaviour of RMS within the oral region is not well defined, an excellent prognosis was considered to be related to soft tissue oral lesions [5,6]. It was reported by Bras et al. [6] that 87.5% of their patients were diseasefree after 4years of follow-up. Since only 27% of our patients with oral sarcoma were alive and 66% of them were diagnosed with RMS, an improved prognosis is implied compared with other sarcomas. However, 50% of the patients with RMS in the present study died of the disease. Leiomyosarcoma (LMS) accounts for 7% of all soft tissue sarcomas [ 171. It is most common in the gastrointestinal tract and in the female genital tract [18]. LMS is a very rare diagnosis and only 38 cases were reported up to 1993 [17]. In the oral cavity, LMS is considered a very aggressive condition with a high rate of recurrence after surgery (35%). The most common presentation for intra-oral LMS is a symptomatic or asymptomatic mass and its most common oral site is that associated with the jaws (58%) [17]. In this paper, we present 2 cases (18.2%) of LMS that involve the tongue and the retromolar area, and manifest clinically as a painful soft tissue mass. Distant metastasis of the intra-oral LMS occurs in approximately 40% of cases, with the lungs being the most common metastatic site. Only 15% of metastases were noted in the cervical lymph nodes [17]. As in the other locations, an early extended surgical excision of the oral LMS facilitates the best prognosis [19]. Radiotherapy is used as an adjuvant to postoperative therapy and not as
295
the first mode of treatment [20]. Chemotherapy is used mainly for palliation [19,20]. Hemangiopericytoma is a tumour originating from the capillary pericytes. Only approximately 3% of these tumours occur in the head and neck [21]. This vascular neoplasm is a very rare tumour and only a few cases of involvement of the head and neck region were found in the literature [22,23], none of which presented in the oral cavity. Our current series includes the case of a 45year-old woman with hemangiopericytoma of the floor of the mouth that presented as a painless growing mass. This case of malignant hemangiopericytoma is very unique, and adds a case of this rare type of neoplasm to the literature in general. The most common clinical approach is excisional surgery. However, because of a recurrence rate of 42% and a metastatic rate of 13% [21,24], life-long follow-up is required. Carcinosarcoma is a neoplasm showing features of This epithelial and mesenchymal differentiation. uncommon disease is prevalent in the bladder, prostate and lungs, but head and neck involvement is very rare. In the salivary glands, these tumours are known as ‘true’ malignant mixed tumours [25], and there has been speculation regarding the possibility of a common precursor cell of myoepithelial origin [26]. Radiotherapy is not an effective therapeutic modality for carcinosarcoma [27], and surgery is necessary. However, as for other sarcomas, adjuvant irradiation may be of benefit in cases where the surgical margins are positive. A male patient presenting a painful mass on the tongue was diagnosed as having carcinosarcoma. 1 case in our series was identified. The patient survived the disease following surgery, irradiation and chemotherapy for over 8years, and then unfortunately died of leukaemia. Takata and others [28] presented a case of a 79-year-old man with a diagnosis of carcinosarcoma of the tongue which they believed to be the first report of this malignancy in this location. To the best of our knowledge, no similar case has been reported since and, therefore, our patient represents the second case in the literature. We identified a case of fibrosarcoma of the gingiva in a 3-year-old boy who presented with a growing gingival mass. He died of the disease 6months after diagnosis. This tumour is rare and it accounts for only 2% of the malignant mesenchymal tumours in the oral cavity [29], the extraosseous type having a tendency to be more aggressive than the intraosseous type [24]. The main clinical approach to this tumour is excisional surgery, which facilitates a better prognosis compared with that of other sarcomas, despite that prognosis has been reported to relate to the degree of cellular differentiation [30]. Clinicians must be aware that while intra-oral soft tissue sarcoma is rare, early diagnosis is of great importance. The first clinical sign of oral soft tissue sarcoma is a growing mass that may not be symptomatic. In our present series, over 54% of the cases were
296
M. Gorsky, J.B. Epstein/Oral Oncology 34 (1998) 292-296
asymptomatic at presentation. Therefore, biopsy of every undiagnosed growing mass should be conducted to exclude a possible malignant process.
Acknowledgements The authors acknowledge the assistance of Lisa Dykman and Norm Phillips for the computer searches and the epidemiologic support provided, and Karen McKay for editorial review.
References 111Weber RS, Benjamin RS, Peters LJ, Ro JY, Achon 0, Goepfert H. Soft tissue sarcomas of the head and neck in adolescents and adults. American Journal of Surgery 1986;152:386392. [21 Angervall L, Kindblom LG. The diagnosis of soft tissue tumours. The Goteborg experience. In: Ryan J, Bahn LO, editors. Recent Concepts in Sarcoma Treatment. Boston: Kluwer Academic Publishers, 1988. p. 22-6. [31 Wharam MD, Beltangady MS, Heyn RM, et al. Paediatric orofacial and laryngopharyngeal rhabdomyosarcoma: an Intergroup Rhabdomyosarcoma Study report. Archives of Otolaryngology Head and Neck Surgery 1987;113:122551227. of the base of the tongue I41 Aydin H, Dreyer T. Leiomyosarcoma treated with radiotherapy: a case report. Oral Oncology, European Journal of Cancer 1994;30B(5):351-355. of [51 Peters E, Cohen M, Altini M, Murray J. Rhabdomyosarcoma the oral and palatal region. Cancer 1989;63:963-966. of the oral b51 Bras J, Batsakis JG, Luna MA. Rhabdomyosarcoma soft tissues. Oral Surgery Oral Medicine Oral Pathology 1987;64:5855596. [71 Coustal B, Barthelemy I, Michelet V, et al. Treatment of primary soft tissue sarcoma of the head and neck. Revue de Stomatologie et de Chirurgie Maxillo-Faciale 1994;95:423426. PI Le Vay J, O’Sullivan B, Catton C, et al. An assessment of prognostic factors in soft tissue sarcoma of the head and neck. Archives of Otolaryngology Head and Neck Surgery 1994; 120:98 l-986. [91 Eeles RA, Fisher C, A’Hern RP, et al. Head and neck sarcomas: prognostic factors and implications for treatment. British Journal of Cancer 1993;68:201-217. [lOI Young JL Jr., Miller RW. Incidence of malignant tumors in U.S. children. Journal of Pediatrics 1975;86:254258. of the head and [ill Dito WR, Batsakis JG. Rhabdomyosarcoma neck. Archives of Surgery 1962;84:5822588. [121 Barnes L. Surgical Pathology of the Head and Neck. New York: Marcel Dekker, 1985. p. 787797. H, Kozawa Y, Takagi M, Otake S. Rhabdomyov31 Yamamoto sarcoma of the left mandible. Journal of Oral and Maxillofacial Surgery 1984;42:613618.
P41 Doval DC, Kannan
V, Acharya RS, Mukherjee G, Shenoy AM, Bapsy PP. Rhabdomyosarcoma of the tongue. British Journal of Oral and Maxillofacial Surgery 1994;32: 1833186. 1151Proops DW, Mann JR. The presentation of rhabdomyosarcomas of the head and neck in children. Journal of Laryngology & Otology 1984;98:381l390. P61 Almanaseer IY, Trujillo YP, Taxy JB, Okuno T. Systemic rhabdomyosarcoma with diffuse bone marrow involvement. Case report of an unusual presentation. American Journal of Clinical Pathology 1984;82:3499353. of the P71 Schenberg ME, Slootweg PJ, Koole R. Leiomyosarcomas oral cavity. Report of four cases and review of the literature. Journal of Cranio-Maxillo-Facial Surgery 1993;21:3422347. [I81 Kyriakos ML. Tumors and tumorlike conditions of soft tissues. In: Kissane JM, Anderson RE, editors. Anderson’s Pathology, 8th Edition. St. Louis: C.V. Mosby, 1985. p. 1642-1704. of soft 1191Wile AG, Evans HL, Romsdahl MM. Leiomyosarcoma tissue: a clinicopathologic study. Cancer 1981;48:1022-1032. WI Chang AE, Rosenberg SA, Glatstein EJ, Antman KH. Sarcomas of soft tissues. In: De Vita VT, Hellman S, Rosenberg SA, editors. Cancer. Principles and Practice of Oncology, 3rd Edition. Philadelphia: JB Lippincott, 1989. p. 1371-73. [211 Walike JW, Bailey BJ. Head and neck hemangiopericytoma. Archives of Otolaryngology 1971;93:345-353. of the head and WI Philippou S, Gellrich NC. Hemangiopericytoma neck region. A clinical and morphological study of three cases. International Journal of Oral and Maxillofacial Surgery 1992;21:99-103. 1231 Delsupech KG, Jorissen M, Sciot R, De Vos R, Van Damme B, Ostyn F. Hemangiopericytoma of the head and neck: A report of four cases and a literature review. Acta Oto-Rhino-Laryngologoca Belgica 1992;46:421427. 1241 Batsakis JG. Tumors of the Head and Neck: Clinical and Pathological Considerations, 2nd Edition. Baltimore: Williams and Wilkins, 1979. 1251Garner SL, Robinson RA, Maves MD, Barnes CH. Salivary gland carcinosarcoma: true malignant mixed tumor. Annals of Otology, Rhinology & Laryngology 1989;98:611614. WI Stephen J, Batsakis JG, Luna MA, von der Heyden U, Byers RM. True malignant mixed tumors (carcinosarcoma) of salivary glands. Oral Surgery Oral Medicine Oral Pathology 1986;61:597602. v71 Batsakis JG, Rice DH, Howard DR. The pathology of head and neck tumors: spindle cell lesions (sarcomatoid carcinomas, nodular fasciitis and fibrosarcoma) of the aerodigestive tracts. Part 14. Head and Neck Surgery 1982;4:499-513. and PI Takata T, Nikai H, Ogawa I, Ijuhin N. Ultrastructural immunohistochemical observations of a true malignant mixed tumor (carcinosarcoma) of the tongue. Journal of Oral Pathology and Medicine 1990;19:261-265. ~291O’Day RA, Soule EH, Gores RJ. Soft tissue sarcomas of the oral cavity. Mayo Clinical Proceedings 1964;39:169-181. BG. Oral fibrosarcoma. Oral Surgery [301 Reade PC, Radden 1966;22:217-225.
PERGAMON
Oral Oncology 34 (1998) 292-296
Head and neck and intra-oral
soft tissue sarcomas
M. Gorsky, J.B. Epstein* British Columbia Cancer Agency, 600 West 10th Avenue, Vancouver, British Columbia, Canada V5Z 4E6
Received 19 November 1997; accepted 25 November 1997
Abstract Intra-oral soft tissue sarcoma is a rare disease. We reviewed a tumour registry over a 45 year period, between January 1951 and January 1997, and identified a total of 11250 head and neck cancers in British Columbia. Of the head and neck cancers, there were 139 cases (1.24%) of sarcoma; and of these, there were only 16 cases (0.14%) of intra-oral soft tissue sarcoma. The initial presentation of intra-oral soft tissue sarcoma is most often as an asymptomatic mass and discomfort is reported by less than one half of the patients. The commonest lesion identified was rhabdomyosarcoma (RMS). In this review, we identified 2 unique cases, 1 case of intra-oral hemangiopericytoma and 1 case of oral carcinosarcoma. The prognosis remains poor. Treatment includes wide surgical excision which may be supplemented with radiotherapy and chemotherapy. 0 1998 Elsevier Science Ltd. All rights reserved. Keywords:
Oral soft tissue sarcoma epidemiology; Diagnosis; Outcome
1. Introduction This paper reviews the literature and presents a case series of sarcomas involving the head and neck, excluding Kaposi’s sarcoma. Soft tissue sarcomas are malignant neoplasms arising in connective tissue, originating from fibrous, fatty, muscular, synovial, vascular, or neural tissue. The tumour, composed of closely packed cells in a fibrillar or homogenous matrix, tends to be vascular and is usually highly invasive. Less than 20% of total body sarcomas occur in the head and neck regions, the most common being rhabdomyosarcoma (RMS). RMS accounts for 18% of the head and neck soft tissue sarcomas [l], followed by malignant fibrous histiocytoma, fibrosarcoma, and neurofibrosarcoma (1 l-16%). All other sarcomas, including unclassified lesions, account for 13% of those found in the head and neck [l]. The histological appearance of the sarcoma lesions is diverse. Furthermore, its accurate diagnosis is challenging and requires the use of specific immunohistochemical procedures and electron microscopy [2]. The treatment of choice is early, radical surgery which includes neck dissection. Intensification of treatment includes autologous bone marrow transplant. Radiation and chemotherapy are mainly used in * Corresponding author. Tel.: 604 877 6000, ext. 2356; fax: 604 872 4596. 1368-8375/98/$19.00 0 1998 Elsevier Science Ltd. All rights reserved. PII: S1368-8375(98)00005-O
combined treatments and for palliation. Combined treatment shows good results in children; as a 3-year survival rate was achieved in 83% of 89 patients following surgery, chemotherapy, and in most cases, radiotherapy [3]. These patients were aged 21 years or less and were diagnosed with localised RMS of orofacial and laryngopharyngeal sites. As revealed in the world literature, there are 100 malignant smooth muscle tumours with only 26 head and neck cases. These 26 cases were mainly reported as unique case presentations [4]. RMS, which is a childhood striated muscle tumour, is one of the most common types of head and neck soft tissue sarcomas [5]. However, there are only a small number of cases presented in the literature. For example, the M.D. Anderson Hospital and Tumor Institute [6] treated just 16 cases of oral RMS over a 40 year period. As presented in the literature, 71.2% of 121 cases of oral RMS were of embryonal origin [5]. The most common site was the palate. Although we searched the English language literature of the last 20years, we failed to detect any study that reviewed the occurrence and the types of oral soft tissue sarcomas in a large group. The purpose of this study was to review the occurrence of head and neck soft tissue sarcomas in British Columbia over the past 45years. We specifically analysed those sarcomas that occurred in the oral cavity.
M. Gorsky, J.B. Epstein/Oral Oncology 34 (1998) 292-296
2. Materials and methods Soft tissue sarcomas of the head and neck were identified from the Tumor Registry and the computer files of the British Columbia Cancer Agency between January 1951 and January 1997. The criteria for the head and neck tumour search included all soft tissue sarcomas involving the following sites: lip, oral cavity, jaws, salivary glands, pharynx, nasal cavity, ear, larynx, and thyroid. However, the search excluded the skull and facial bones. Cases of osteogenic sarcoma, chondrosarcoma, and Kaposi’s sarcoma were excluded from the study. The study included a total of 139 cases of soft tissue sarcoma of the head and neck. Of these, 123 patients had extra-oral soft tissue sarcomas and 16 patients were diagnosed with intra-oral lesions. Of the 16 patients, records were available for 9 patients, but only Registry data were available for 7 patients.
3. Results Between 1972 and 1995, the total number of cases of malignant diseases in British Columbia was 207 005 and for these 23 years complete reliable data were available. Of these total cases of cancer, there were 10 785 (5.21%) recorded cases of head and neck cancer. Over a 45 year period, between January 1951 and January 1997, a total of 11250 cancers were recorded in the head and neck, although the accuracy of this is not assured. Of the head and neck cancers, there were 139 cases (1.24%) of head and neck soft tissue sarcoma; and of these there were only 16 cases (0.14%) of intra-oral soft tissue sarcoma. The patient characteristics of the 139 patients are shown in Table 1. Of these patients, there were 73 males and 66 females. The mean age at diagnosis was 41.7 years and ranged between 1 and 89 years. The data were then measured separately for the 123 cases of extra-oral sarcoma and the 16 cases of intra-oral sarcoma. At the time of diagnosis, the mean age was lower in intra-oral sarcomas; 39.1 years versus 42.1 years in patients with extra-oral head and neck sarcomas. There Table 1 Profile of 139 patients
with head and neck soft tissue sarcomas
Gender Extra-oral sites Male Female Subtotal (male and female) Intra-oral sites Male Female Subtotal (male and female) Total
No.
Mean age (years)
Range
63 60 123
41.1 43.2 42.1
(4-84) (1-89) (1-89)
10 6 16
40.1 37.5 39.2
(12-63)
139
41.7
(l-89)
(3-75)
293
were more men than women in each of the two groups. In the extra-oral sarcoma group there were 63 (51.2%) men and in the intra-oral sarcoma group there were 10 men (62.5%). As seen in Table 2, the nasopharynx was the most commonly involved site and 27 (19.1%) cases were found in this location. The nasal cavity was involved in 20 (14.2%) cases. There were 16 (11.5%) cases of oral soft tissue sarcomas and 13 cases of sarcomas of the parotid gland, which accounts for 10.6% of the nonoral sarcomas. Table 3 presents, in detail, 9 of the 16 cases with soft tissue intra-oral sarcomas for whom the medical records were available. The tongue was involved in 3 cases (33.3%), and the palate in 2 cases (22.2%). The gingiva, the retromolar pad, the buccal mucosa, and the floor of the mouth were diagnosed in 1 patient each (11.1% each). The mean age at diagnosis was 39.6years, ranging from 3 to 75 years. 8 of the patients died and only 2 patients (22.2%) survived. Of the surviving patients, both were initially diagnosed with embryonal RMS, and remained disease-free for a mean time of 15 years. Of the 6 deceased patients, 5 died of their sarcoma in a mean survival time of 1.8 years, 1 patient died from other causes, and 1 patient was lost to follow-up. The youngest patient with oral sarcoma was a 3-yearold boy who had fibrosarcoma of the gingiva and he did not survive his disease. There were two other young children, both with the diagnosis of embryonal RMS, who survived their disease. All cases featured a swelling of the site of malignancy as the first clinical sign and only 5 (45.5%) featured symptoms of pain or soreness. 3 patients had a family history of neoplasms, but no history of sarcomas in the family was reported.
4. Discussion Soft tissue sarcomas account for less than 1% of head and neck neoplasms and have a diversity of type and location. The most common type is RMS (18%). Only 19% of head and neck sarcomas involve the sinonasal cavity, while oral involvement is very rare [l]. The treatment of choice is early and radical surgery. Generally, the response to chemotherapy and irradiation is not sufficient for cure [7], since a high local failure due to indistinct surgical margins occurs, and adjuvant radiotherapy and chemotherapy is often indicated [8]. This study surveyed the head and neck site distribution of soft tissue sarcomas. When a search for previous articles was performed, we failed to find any articles describing site prevalence. We found that almost 50% of our cases were those of the nasal and paranasal region and the mean age at diagnosis was 42 years, 52% being found in males. In a pathological review of 103
75
4
22
14
45
3
73
63
57
M
h4
M
F
F
M
M
F
M
(Years)
Age
Site
16
’
Lateral tongue
Retromolar
Rare of tongue
Gmgiva
Floor of mouth
Retromolar
Palate
Leiomycsarcoma
Lciomyosarcoma
rhabdomyosarcoma Malignant haemangiopericytoma Fibrosarcoma
ElUblyOMl
Embryonal rhabdomyosarcoma Fmbryonal rhabdomyosarcoma
Rhabdomyosarcoma
Diagnosis
17
Ethmoid sinus
soft tissue sarcomas
20
Oral cavity
with soft tissue sarcomas
Nasal cavity
Tongue
with oropharyngeal
27
Nasopharyngeal
No. Gender
Table 3 9 patients
No.
Site
Table 2 139 head and neck sites involved
13
Parotid gland
ccl1
Hodgkin’s
(6)
tonsils (16) None
carcinoma
Scpramous
None
None
None
None
Lymphatic leukaemia (1) None
other neoplasm (years) before
16
Maxillary sinus
M&W
Mass
Mass
Swelling
Mass
Mass
Mass
Signs
6
Pain
Pain
Sore
None
None
None
Pain
None
Sore
_
_
?
+
?
+
Mother
Mother and father
None
Unknown
_ +
None
None
None
None
Family history of cancer
_
_
_
_
Alcohol
6
Sphenoid and accessory sinuses
_
_
+
Tobacco
6
Pharynx and hypopharynx
Symptoms
External lower lip
Local excision
Treatment
3
Glotis and supraglotis
Surgery, irradiation and palliative chemotherapy, disease progressed Partial glossectomy, recurred in 2 years, irradiation with no response
Radiation and chemotherapy
Surgery, irradiation and chemotherapy Irradiation and chemotherapy = no response, surgery = recurrence Chemotherapy and irradiation Surgery. No further information Unknown
4
Thyroid gland
4
2
8.5
0.5
Unknown
12
2
18.5
0.5
(years)
Follow-up
2
Middle ear
Alive, free of disease
Died of disease Alive, free of disease Died of disease
status
1
Tonsils
Died of disease
Died of disease
Died of disease Died, neoplasm of other tissues
2
Larynx
M. Gorsky. J.B. Epstein/Oral Oncology 34 (1998) 292-296
cases, Eeles et al. [9], also found that the age of onset was relatively young (median 32 years), 53% being men. In comparison to carcinoma, the soft tissue sarcoma is a neoplasm involving younger ages, including children and adolescents, in which almost 15% of all sarcomas were seen [lo]. Risk factors have not been identified for these connective tissue malignancies. We did not identify any potential risk factors, and found that only 4 of 10 patients (40%) used tobacco. Most oral soft tissue sarcomas, despite the head and neck predilection of RMS, have been published as isolated case reports. The majority of RMS is diagnosed in young ages and 43% of them are of the embryonal type and occur most commonly in males [5]. We found that the majority of our cases with RMS (75%) were those of embryonal RMS and 3 of 4 were males. Data from a previous study [l l] indicate that the oral manifestation of RMS occurs in approximately 20% of head and neck soft tissue sarcomas. The predominant oral site is the palate [12]. However, others have indicated that the tongue was more commonly involved [13]. Of 142 RMS cases of various sites, only 1.4% were noted in the tongue [14]. Only 1 case of tongue RMS was identified in our current study. RMS is a result of malignant changes of primitive pluripotential mesenchymal cells rather than that of differentiated muscle [15,16]. Although the behaviour of RMS within the oral region is not well defined, an excellent prognosis was considered to be related to soft tissue oral lesions [5,6]. It was reported by Bras et al. [6] that 87.5% of their patients were diseasefree after 4years of follow-up. Since only 27% of our patients with oral sarcoma were alive and 66% of them were diagnosed with RMS, an improved prognosis is implied compared with other sarcomas. However, 50% of the patients with RMS in the present study died of the disease. Leiomyosarcoma (LMS) accounts for 7% of all soft tissue sarcomas [ 171. It is most common in the gastrointestinal tract and in the female genital tract [18]. LMS is a very rare diagnosis and only 38 cases were reported up to 1993 [17]. In the oral cavity, LMS is considered a very aggressive condition with a high rate of recurrence after surgery (35%). The most common presentation for intra-oral LMS is a symptomatic or asymptomatic mass and its most common oral site is that associated with the jaws (58%) [17]. In this paper, we present 2 cases (18.2%) of LMS that involve the tongue and the retromolar area, and manifest clinically as a painful soft tissue mass. Distant metastasis of the intra-oral LMS occurs in approximately 40% of cases, with the lungs being the most common metastatic site. Only 15% of metastases were noted in the cervical lymph nodes [17]. As in the other locations, an early extended surgical excision of the oral LMS facilitates the best prognosis [19]. Radiotherapy is used as an adjuvant to postoperative therapy and not as
295
the first mode of treatment [20]. Chemotherapy is used mainly for palliation [19,20]. Hemangiopericytoma is a tumour originating from the capillary pericytes. Only approximately 3% of these tumours occur in the head and neck [21]. This vascular neoplasm is a very rare tumour and only a few cases of involvement of the head and neck region were found in the literature [22,23], none of which presented in the oral cavity. Our current series includes the case of a 45year-old woman with hemangiopericytoma of the floor of the mouth that presented as a painless growing mass. This case of malignant hemangiopericytoma is very unique, and adds a case of this rare type of neoplasm to the literature in general. The most common clinical approach is excisional surgery. However, because of a recurrence rate of 42% and a metastatic rate of 13% [21,24], life-long follow-up is required. Carcinosarcoma is a neoplasm showing features of This epithelial and mesenchymal differentiation. uncommon disease is prevalent in the bladder, prostate and lungs, but head and neck involvement is very rare. In the salivary glands, these tumours are known as ‘true’ malignant mixed tumours [25], and there has been speculation regarding the possibility of a common precursor cell of myoepithelial origin [26]. Radiotherapy is not an effective therapeutic modality for carcinosarcoma [27], and surgery is necessary. However, as for other sarcomas, adjuvant irradiation may be of benefit in cases where the surgical margins are positive. A male patient presenting a painful mass on the tongue was diagnosed as having carcinosarcoma. 1 case in our series was identified. The patient survived the disease following surgery, irradiation and chemotherapy for over 8years, and then unfortunately died of leukaemia. Takata and others [28] presented a case of a 79-year-old man with a diagnosis of carcinosarcoma of the tongue which they believed to be the first report of this malignancy in this location. To the best of our knowledge, no similar case has been reported since and, therefore, our patient represents the second case in the literature. We identified a case of fibrosarcoma of the gingiva in a 3-year-old boy who presented with a growing gingival mass. He died of the disease 6months after diagnosis. This tumour is rare and it accounts for only 2% of the malignant mesenchymal tumours in the oral cavity [29], the extraosseous type having a tendency to be more aggressive than the intraosseous type [24]. The main clinical approach to this tumour is excisional surgery, which facilitates a better prognosis compared with that of other sarcomas, despite that prognosis has been reported to relate to the degree of cellular differentiation [30]. Clinicians must be aware that while intra-oral soft tissue sarcoma is rare, early diagnosis is of great importance. The first clinical sign of oral soft tissue sarcoma is a growing mass that may not be symptomatic. In our present series, over 54% of the cases were
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M. Gorsky, J.B. Epstein/Oral Oncology 34 (1998) 292-296
asymptomatic at presentation. Therefore, biopsy of every undiagnosed growing mass should be conducted to exclude a possible malignant process.
Acknowledgements The authors acknowledge the assistance of Lisa Dykman and Norm Phillips for the computer searches and the epidemiologic support provided, and Karen McKay for editorial review.
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