Oral leiomyosarcoma

Oral leiomyosarcoma Report of a case and review of the to smooth-muscle tumors of the oral Allan G. Farma,n” Africa DEPARTMENT

literature cavity

and Rholem Kay, ** Cape Province,

OF ORAL

PATHOLOGY,

UNIVERSITY

pertaining

Republic

of South

OF STELLENBOSCH

Primary malignant smooth-muscle tumors of the mouth have rarely been reported. A review of the literature has yielded only eleven cases; to these, a new case is added. The age, sex, and site distribution of oral leiomyosarcomas are compa?ed with those of oral leiomyomas.

M

alignant smooth-muscle tumors are not uncommon in the genitourinary and gastrointestinal tracts, but the occurrence of these neoplasms in the oral cavity is rare. A review of the world literature has produced only eleven possible primary oral lesions. These are listed in Table I.I-l” CASE REPORT The patient, an unmarried, d&year-old, European woman, attended the Head and Neck Clinic of the Johannesburg General Hospital, complaining of a swelling and an itching sensation in the right cheek. Palpation revealed a firm mass in the region of the right infraorbital foramen, with fixation of the surrounding tissues. The overlying mucous membrane and skin were of normal color and consistency. There waa no associated lymphadenopathy. Nothing else of note was found in a general medical examination. The tumor was resected, together with a margin of clinically normal tissue. This involved a full-thickness excision of cheek tissues. Macroscopically, the lesion was composed of ovoid white masses which were continuous with the infraorbital nerve bundles. Histologic examination of the resected tissues revealed interlacing bundles and whorls of plump spindle and strap-like cells with blunt-ended nuclei, prominent nucleoli, and moderately eosinophilic, slightly granular cytoplasm (Figs. 1 and 2). The tumor mass stained red with Masson’s trichrome technique and blue with Mallory’s phosphotungstic acid hematoxylin technique. Longitudinal myofibrils were seen with varying degrees of clarity in sections stained with phosphotungstic acid hematoxylin. The cells showed a moderate degree of pleomorphism, *Department *“General

402

of Oral Pathology, Hospital and Brenthurst

University Clinic,

of Stellenbosch. Johannesburg.

Volume 43 Number 3

Oral leiwmyosarcoma 403

Table I. Clinical presentation of oral leiomyosarcoma Author Zielerl Hayn* Diss and Lbvy’ Carmody et al.’ Geever et al.& Stout and Hill6 Yannopoulos and Stout’ Miles and Waterhouse8 O’Day et al.s 0:Day et al.: giyr;g; a’. and Valletta” Dor et al.”

Age

Year 1908 1910 1924 1944

Sex F

(yr.) 67

Site Cheek

Me&stases Unknown

Trearment Unknown

Survival Unknown

F M

18

Unknown No

Radiotherapy Surgery

Died (8 mo.) Alive (6 mo.)

1949 i 1958 1962

F

61

Cheek Mandible C;b&ral)

Yes

Surgery

Unknown

M

No

Surgery

Alive (4.6 yr.)

1962

M

11 Tongue (mo.) Mandible 34

Yes

Died (2.6 yr.)

1964 1964 1964 1966

M M F M

; 19 11

Floor of mouth Soft palate Floor of mouth Gingivae

Surgery and radiotherapy Sur ery Ra 81lotherapy Surgery Surgery

Died (22 mo.)* Dig (30 mo.)* ;;Tn;;nyr.)

1967

M

57

My;tile

Surgery

Alive (2 yr.)

Surgery

Alive (6 mo.)

Yes Yes Yes Unknown

and No

soft tissue Present case 1975 F 88 Cheek *Also reported by Botting and associates.12

No

but mitotie activity was not a pronounced feature. Silver impregnation showed that reticulin fibers ran parallel to the tumor cells. The tumor involved the infraorbital nerve bundle, and much of the normal nerve architecture was lost. In places, normal and infiltrated nerves were demonstrated side by side (Fig. 3). A diagnosis of leiomyosarcoma was made histologically; this diagnosis was unanimously accepted when sections were circulated among the histopathologists at the South African Institute for Medical Research in Joliannesburg. The exact source of the smooth muscle could not be determined. However, the decision was that this was probably a primary lesion, since an extensive general examination failed to reveal any evidence of a primary source elsewhere, and there was no evidence of metastases. The patient was alive 6 months after surgical intervention, but was still complaining of an “itching sensation. ” It is feared that there is still some tumor in the infraorbital nerve.

REVIEW OF THE LITERATURE AND KNOWN CASES Of the eleven diagnosed casesof primary oral leiomyosarcomas reported in the literature and the one new case, four occurred in the cheeks and three in the mandible. The floor of the mouth was involved twice and the tongue, gingivae, and the soft palate once each. A list of possible casesof primary oral leiomyosarcomas is given in Table 1.*-12 Milian and associateP* l4 reported a leiomyosarcoma arising in the right cheek of a 42-year-old female patient. However, she had been treated previously for a smooth-muscle tumor of the uterus, so that the oral lesion was probably metastatic rather than primary. Laurent and associates15reported the occurrence of a “leiorhabdopoietic” sarcoma of the parotid gland in an a-year-old boy. This case has been excluded because of the histologic ambiguity. In addition to the casesaccepted as possibly being primary oral leiomyosarcomas, Cheek and Nickey16 have reported a leiomyosarcoma of the anterior and internal jugular veins in a 62year-old white woman. Of twenty-five casesreported as venous leiomyosarcomas between 1871 and 1965, this was the only one arising in the head and neck area. A review of the literature has produced fifty-one cases of oral leiomyoma

Oral Surg. March, 1977 Table

II. Clinical presentation of oral leiomyoma Author

Year

Blant? Glas? Fein19 SehrtZO Wei?’ Pete? stout23 stout23 Burford et al.*’ Abulafia and GrinspanzS Duhig and Aye? Duhig and Aye? Yannolopoulos and Stout’ Yannolopoulos and Stout’ Kohn et al?’ Watabe and Morishima*’ Hagy et al.”

1884 1905 1905 1907 1914 1937 1938 1938 1944 I956 1959 1959 1962 1962 1963 1963 1964 1964 1964 1964 1965 1965 1965 1965 I966 1967 1967 1967 1968 1969 1969 1969 1969 1969 1969 1969 1970 1971 1973 1973 1973 1973 1973 1973 1973 1974 1975 1975 1975 1975 1975

h‘XS30

Kist and Bhaska?I Bianch? BertelI? Utz3’ FrenkeP5 PettiniaB Lewit? Merrill and Downs? Merrill and DownsSB Manhold and Doyle6’ Collins and EIzay3B Garrett’O McGowan and Jones” McGowan and Jones” MacDonald’Z MacDonald’z Colangelo and Benagiano’” Weisinger et al.“’ Costas and Curuchaga’s Papadriano? Cherrick et al.” Cherrick et al.” Cherrick et al.” Cherrick et al.” Cherrick et al.” Cherrick et al.” Cherrick et al.” Galili and Shteyer’B Farman’* Farman” Farman’# Farman’B Farman’

Sex

1

Agefyr.1

33 44 20 Student :z 5: 32 48 4% II Infant 76 I2 ii 43 26 I3 73 11 El 70 2 48 2: II 59 2 2: 56 47 2 2; 5; Adult 6

[

Sire

Tongue Tongue Soft palate Cheek Uvula Sublingual salivary gland Tongue Tongue Tongue and floor of mouth Cheek Lower lip Lower ho Palate Tongue Lio Gingivae Palate Cheek Tongue Gingivae Cheek Cheek Cheek Tongue Cheek Cheek ;p;; lip Lower lip Upper lip Upper lip Tongue Parotid salivary gland Gingivae Cheek Tongue Palate Buccal sulcus Palate Palate Palate Gingivae Lower lip Soft palate Tongue Tongue Lower lip . Lower .11p Submandibular salivary gland

(Table II) .1i-48 In contrast to leiomyosarcoma, none of the benign lesions of smooth muscle occurred centrally within the mandible. Oral leiomyomas arose in in the tongue ( 13)) cheeks ( 11)) palate (lo), lips (lo), gingivae (4)) and salivary glands (3). The age range of the patients in whom oral leiomyosarcomas were discovered was 11 months to 88 years. The corresponding age range of patients with oral leiomyomas was infancy to 76 years. No marked sex predilection was detected for oral smooth-muscle tumors. For

Volume Number

43 3

Fig. 1. The neoplasm consisted with very little stromal connective Original magnification, x100.)

Oral leiomyosarcoma

essentially of sheets and whorls tissue. (Mallory’s phosphotungstic

of “spindle-shaped” acid hematoxylin

405

cells stain.

leiomyomas, twenty-five occurred in males and twenty-one in females ; for leiomyosarcomas, seven arose in males and five in females. DISCUSSION

Smooth-muscle tumors of the oral cavity are rare. Farman4g found only five oral cases among 7,748 benign smooth-muscle tumors of the whole body that he analyzed. A review of the literature has produced only eleven cases of primary leiomyosarcoma and fifty-one cases of oral leiomyoma. The scarcity of smooth-muscle tumors of the oral cavity is probably due to the paucity of smooth muscle in this region. Smooth muscle in the mouth is mainly localized to the tunica media of blood vessels and the walls of larger iymphatics. Abulafia and GrinspanZs reported that it may be found in aberrant hair follicles in the cheeks. Garrett40 and Lucas30 stated that smooth muscle may also be present in the circumvallate papillae of the tongue. Cheek and NickeyX6 believe that some smooth-muscle tumors may develop from primitive mesenchyme. The formation of smooth-muscle walls around developing arterioles in granulation tissue lends support to this hypothesis.50 Oles51 and Farman* showed that the apparent rarity of smooth-muscle tumors in the oral cavity is not due to histologic misdiagnosis. They stained previously diagnosed fibrous tissue lesions with special stains for muscle, and all cases investigated proved to be negative for muscle. Diagnosis

Specially stained sections are required to distinguish muscle from other sues. Mallory’s phosphotungstic acid hematoxylin (C.I. No. 75290) stains cytoplasm of muscle blue and the fibrocytes red. It stains all nuclei blue. Gieson’s method (C.I. Nos. 10305 and 42685) stains collagen red and muscle

tisthe Van yel-

Oral March,

Surg. 1977

Fig. 2. Individual tumor cells showed moderate pleomorphism, prominent nucleoli and (with varying degrees of clarity) longitudinal myofihrils. (Mallory’s phosphotungstic hematoxylin stain. Original magnification, x400. J

(N), acid

low. Masson’s trichrome (C.I. Nos. 16150,42585,42686, 51050, and 75290) has been criticized by 01~“’ for giving false-positive reactions for muscle. However, with adequate controls this stain is useful for differentiating between fibrous tissue (green) and muscle (red). The well-differentiated smooth muscle found in benign tumors contains nonstriated longitudinal myofibrils within its cytoplasm. These are most prominent when stained by phosphotungstic acid hematoxylin. Longitudinal myofibrils can also be helpful in the diagnosing of leiomyosarcoma. A positive finding is diagnostic of smooth muscle. IJnfortunately, a negative finding does not rule out leiomyosarcoma, since myofibrils are not generally found in the more dedifferentiated lesions. Stout and Latte9 described the microscopic features of leiomyosarcoma as consisting of interlacing cords of elongated cells with blunt nuclei. As a rule, but not invariably, the mitotic rate is elevated. The cells generally show anaplastic features. Enzinger and associates”3 point out that the lesion may be richly vascular, and that the cytoplasm of the cells is eosinophilic. They consider mitotic activity to be the major distinguishing factor between leiomyomas and their malignant counterparts. In contrast to this view, Botting and associates12 found no great correlation between the mitotic rate and the aggressiveness of the tumor. Spiro and IMcPeak54 reported two histologically benign smooth-muscle tumors which metastasized. Thus, the potential of smooth-muscle tumors to metastasize cannot be ascertained histologically, and the histologic degree of differentiation may be misleading insofar as behavior is concerned. For one to make a diagnosis of primary leiomyosarcoma in the oral cavity, the patient should be thoroughly examined in order to exclude other possible

Volume Number

43 3

Oral leiomyosarcoma

Fig. 3. Small neurovascular bundles (a) and nerves that had been infiltrated Original magnification, x63.)

at the periphery by the neoplastic

of the tumor contained cells (B). (Masson’s

normal trichrome

407

nerves stain.

primary sites. Even after the most extensive examination, the possibility of a very small primary lesion being present elsewhere cannot be entirely ruled out. The possibility of a leiomyosarcoma being a metastatic deposit could explain the apparent difference of site distribution between oral cases of leiomyoma and leiomyosarcoma. Three of the malignant tumors were found in the mandible, whereas all leiomyomas were peripheral. Solomon and associates,55 in reviewing metastatic lesions of the oral tissues, found that the vast majority of these occurred in the mandible, and that metastatic lesions in the oral soft tissue were unusual. The incidence of malignancy in oral smooth-muscle tumors appears to be proportionately high when compared with that in the same lesions in the female genital tract. Stout and Hill6 stated that the ratio of leiomyosarcomas to leiomyomas is less than one in 200 for the female genital tract. Twenty per cent of smooth-muscle tumors in the mouth, reported in the literature, were malignant. Behaviour

and

Prognosis

No general statement concerning the behavior of oral leiomyosarcoma can be made with such a small number of examples being available. Patients with distant metastaseshad poorer prognoses than did those with no metastasesor only local involvement of lymph nodes. This article Witwatersrand.

was prepared in the Department of Oral Pathology of the University We wish to acknowledge the technical assistance of Mr. A. Scott.

of the

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Burg. 1977

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Oral

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leiomyosarcoma

40. Garrett, J. R.: Angiomyoma of the Palate-Report of a Case, ORAL SURG. 1969. 41. McGowan, D. A., and Jones, J. H.: Angioma (Vascular Leiomyoma) of the ORAL Suno. 27: 649-652, 1969. 42. MacDonald, D. G.: Smooth Muscle Tumours of the Mouth, Br. J. Oral Surg. 1969. 43. Colaneelo. G.. and Benaoiano. E.: Su xlcuni oarticolari asnetti istonatolooici angio&atosi de1 cave oral”, Ann. Stomatol. (R&a) 18: 507-52f, 1969.* 44. Weisinger, E., Doyle, J., and Ladov, M. J.: Case Report: Leiomyoma of the J. New Jersev Dent. Sot. 40: 355-356. 1969. 45. Costas, J. B.; and Curuchaga, E.: L&omioma de la cavidad bucal, An. Esp. 29: --.

xvG.192. -_- ---, 197n. - _._.

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Odontoestom.

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Reprint requests to: Dr. Allan G. Farman Department of Oral Pathology University of Stellenbosch Private Bag XI 7505 Tygerberg Cape Province, Republic of South

Africa