Canale, Virginia C.

Carbone, Antonino

Grigoletto, Eligio

Volpe, Rachele

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The Journal of Laryngology and OtologyJune 1983. Vol. 97. pp. 553-556Botryoid rhabdomyosarcoma of the nasopharynxbyVIRGINIA C. CANALE,* RACHELE Vou>E,t ANTONINO CARBONE! and ELIGIOGRIGOLETTO* (Pordenone, and Genova, Italy)IntroductionBotryoid rhabdomyosarcoma is a common soft-tissue sarcoma of children, the mostfrequent presentation being in the genito-urinary tract A less frequent site is the head andneck region involving the pharynx, larynx, maxillary sinus and middle ear (Ragab et al,1977).Rhabdomyosarcomas of the head and neck are generally associated with a poorprognosis because of their late diagnosis, inoperability and local recurrences involvingespecially the base of the skull and meninges. Better definition of the stage of the disease,histology and treatment by the various modern modalities of combined surgery,radiotherapy (RT) and chemotherapy (CT) have significantly altered the prognosis andsurvival in patients with these tumours, with two-year disease-free survival rates reportedas 85 per cent, 83 per cent, 50 per cent, 22 per cent and 6 per cent with Stages I, IIA, IIB,IILA and IIIB disease respectively (Green and JafFe, 1978). However, no prognosticcorrelation or distinction in survival rates has been reported between the botryoid andother variants of embryonal rhabdomyosarcomas of the nasopharynx.The purpose of this paper is to illustrate a case of Stage III (according to the IntergroupRhabdomyosarcoma Study) botryoid rhabdomyosarcoma of the nasopharynx associatedwith a four-and-a-half-year disease-free survival after combined radio-chemotherapeutictreatmentCase Presentation I n i t i a l haematological, biochemical andA \2Yi-year-old Italian female was admitted to urinary evaluations, including a 24-hour VMAour hospital in January 1974 at six years of age determination, were within normal limits. A chestwith signs of nasal obstruction and sinusitis of X-ray was normal. Sinus X-rays and tomogramsincreasing intensity during the two months of the upper airways demonstrated opaquepreceding hospitalizatioa No other systemic masses occupying the nasal fossae with extensionsymptoms were evident Since the age of six into the right maxillary sinus. Histologicalmonths, the child had experienced frequent examination of a nasopharyngeal mass biopsyepisodes of mucopurulent rhinitis and asthmatic revealed embryonal rhabdomyosarcoma.bronchitis. ENT examination revealed No further surgery was undertaken and themucopurulent material in both nasal fossae, patient was treated with 6,000 rads 60cobaltseveral sessile polypoid masses occupying the radiotherapy (TCT) in 30 sessions to thenasopharynx, and bilateral diffuse adenopathy of nasopharynx and maxillary sinuses, with greaterthe submandibular and lateral cervical lymph than 75 per cent reduction of the tumour masses,nodes. No other signs of metastatic disease were Post-TCT, the patient did well until Januaryapparent 1975, when one right and one left enlarged, firm,* Division of Oncology and Radiotherapy, Ospedale Civile, Via Montereale, 33170, Pordenone, Italy.t Department of Pathology, Istituto Scientifico per Lo Studio e La Cura di Tumore, Istituto di Oncologiadell'Universita, Viale Benedette X V , 10, 16132, Genova, Italy.553of use, available at https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215100094561Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 14:43:05, subject to the Cambridge Core terms554 V. C. CANALE, R. VOLPE, A. CARBONE AND E. GRIGOLETTOfixed submandibular lymph nodes were noted,measuring 2X1.5 cm. and 6X6.5 cm. respec-tively. Re-evaluation studies, including a bonemarrow aspiration, lumbar puncture andpulmonary tomograms, were all negative exceptfor an opaciflcation of the soft tissue and a mildcompression of the left hypopharynx. A leftlymph node biopsy histologically confirmed thepresence of metastasis. CT with five daily dosesof Adriamycin (10 mg./m2.) was administeredfollowed by 4,000 rads TCT in 20 sessions toboth antero-lateral lymph node chains.From July to December 1975, following thecompletion of TCT, the patient was treated withsix-monthly cycles of intravenous CT accordingto Gottlieb et al. (1972): Adriamycin 50 mg.Day 1, Cyclophosphamide 500 mg. Day 1,Vincristine 1.2 mg. Days 1 and 5 and DTIC300 mg. Days 1 to 5. The patient tolerated CTwith no complications. A right maxillary sinusbiopsy in February 1976 revealed no evidence ofmalignancy.The patient has maintained a four-and-a-half-year complete remission since suspension of CTor a six- and-a-half-year survival since diagnosis.Consequences of RT include partial atrophy ofthe nasal and nasopharyngeal mucosa, multipledental caries and enamel dysplasia, minimalatrophy of the maxillary bones, and bilateralpersistent opaciflcation of the maxillary sinuses.Pathological FindingsGrossly, the initial biopsy from the naso-pharyngeal tumour appeared as a translucent,gelatinous, grape-like mass. This 3X2 cm.polypoid mass was soft, shiny and partiallyulcerated.Microscopically, the neoplasm was primarilycomposed of immature cells with a loosestructure. The tumour showed a relatively denselayer of less differentiated, round or spindlerhabdomyoblastic cells of small and medium size,three to five cells thick, with many mitoses. Thesurface layer was covered with benign surfaceepithelial cells of the respiratory mucosa(Fig. 1). Immediately beneath the layer ofrhabdomyoblasts, there was abundant myxoidloose tissue containing small blood vessels,stellate cells and several small rounded andelongated cells with acidophilic cytoplasmcontaining two or more nucleoli and a moderateamount of mitotic activity. Features suggestive ofcross-striation could not be observed in theircytoplasm.»» . . . • - •• . » ** *FIG. 1Botryoid rhabdomyosarcoma. Thesubepithelial zone contains embryonalrhabdomyoblasts as well as myxoid loosetissue. Haematoxylin and Eosin XI30.of use, available at https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215100094561Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 14:43:05, subject to the Cambridge Core termsCLINICAL RECORDS 555The histologic diagnosis was embryonalrhabdomyosarcoma of loose or botryoid typeaccording to the International Society ofPediatric Oncology (SIOP) classification(Gerard-Marchant et al, 1978). The histologicexamination of a lymph node biopsy performedone year after the initial diagnosis revealedunequivocal metastatic lesions by embryonalrhabdomyosarcoma, whereas the control bonemarrow aspirates and spinal fluid cytologicalexaminations were always negative.DiscussionThe recognition of sarcoma botryoides asa distinct clinical and pathological entity, andthe description of alveolar rhabdomyosarcomacomplete the frequently-used grouping of±abdomyosarcomas into embryonal, botryoid,alveolar and pleomorphic types (Horn andEnterline, 1958). However, the histologicclassification adopted by SIOP differentiated twomain types of rhabdomyosarcomas: embryonaland adult (Gerard-Marchant et al, 1978).The former included the loose (or botryoid),dense, myoblastic and alveolar (Riopelle's)rhabdomyosarcomas.Embryonal rhabdomyosarcoma is a commonsoft-tissue sarcoma of children with a median ageincidence of five years (Green and Jaffe, 1978).Although this tumour can develop in any area ofthe body, the botryoid variant occurs near themucosal surface (Stout and Lattes, 1967).Clinical manifestations of tumours of thenasopharynx and paranasal sinuses include localpain and swelling, nasal obstruction, nasaldischarge, epistaxis and sinusitis. The overallincidence of positive regional lymph nodes atpresentation for rhabdomyosarcomas of the headand neck is estimated to be 3 per cent (Green andJaffe, 1978). Local recurrences with directmeningeal extension or distant metastases to thelymph nodes, lungs, bones, liver and bone marrowtend to develop within an average of 7.6 months,with approximately 70 per cent occurring withinone year from diagnosis (Ragab et al, 1977).The value of combined surgery, CT and RT hasbeen clearly demonstrated in several studies(Ghavimi et al, 1975; Donaldson et al, 1973;Mauer et al, 1977). Surgical excision, wheneverpossible, is recommended for head and necktumours but should not involve major loss offunction or cosmetic deformity. Currentrecommendations for RT are 5,000-6,000 radsTCT to the primary tumour, resulting in localcontrol in 80-90 per cent of non-orbital head andneck tumours (Ragab etal, 1977). CombinationCT with VAC and RT have proved extremelyefficacious, especially in inoperable tumours.Donaldson et al. (1973) achieved a local controlrate of 89 per cent Of these patients, 74 per centwere free of disease two to seven years later, witha median disease-free interval of two years eightmonths.Several factors, including extent of disease,site, histologic type and age modify the prognosis(Pratt etal, 1978). Rhabdomyosarcomas of thehead and neck, excluding the orbit, are presentlyassociated with a 68-75 per cent disease-freesurvival (Green and Jaffe, 1978). The bestsurvivals regarding histologic type have beenreported with sarcoma botryoides (site notspecified), although in the National Rhabdomyo-sarcoma Study, the pathologic type did not affectthe survival rate (Ragab et al, 1977). Childrenunder seven years of age have a significantlybetter survival than older children due to lessextensive disease at the time of diagnosis, lowerrates of recurrence and a more favourablehistologic type.Our patient appears to have met all thesefavourable criteria and therefore the long-termdisease-free survival was expected but still note-worthy since it is above the median reported in theliterature for the stage of the disease. This may bedue in part to its histologic type and in part to thecombined therapy employed. We suggest thathistologic type and prognosis of rhabdomyo-sarcomas of the nasopharynx may be bettercorrelated in future studies of patients.SummaryA case report of a Stage III botryoidrhabdomyosarcoma of the nasopharynx associatedwith a six-and-a-half-year survival is presented.Treatment consisted of surgery, radiotherapy(6,000 rads TCT) to the nasopharynx andmaxillary sinuses bilaterally, and six cycles ofpolychemotherapy with Vincristine, Adriamycin,Cyclophosphamide and DTIC, without majorloss of function or cosmetic deformity. Thehistology of the lesion is discussed with referenceto recent classification and prognosis. Theauthors suggest that the histological typeand prognosis of rhabdomyosarcoma of thenasopharynx in children may be better correlatedin future studies.of use, available at https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215100094561Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 14:43:05, subject to the Cambridge Core terms556 V. C. CANALE, R. VOLPE, A. CARBONE AND E. GRIGOLETTOReferencesDONALDSON, S. S., CASTRO, J. R, WILBUR, J. R, andJESSER,R H. JR. (1973) Rhabdomyosarcomaofheadandneck in children. Cancer, 31: 26.GERARD-MARCHANT, R, MARSDEN, H. B., and VAN UNNIK, A. J. M. (1978) Histological classification ofrhabdomyosarcomas. In Abstracts, Xth Meeting of the International Society of Pediatric Oncology (SIOP),Brussels, Belgium. 20-23 September, p. 13.GHAVIMI, P., EXELBY, P. R., and D'ANGIO, G. I. (1975) Multidisciplinary treatment of embryonalrhabdomyosarcoma in children. Cancer, 35: 677.GOTTLIEB, J. A., BAKER, L. H., and QUAGLIANA, J. M. (1972) III. Chemotherapy of sarcomas with a combinationof Adriamycin and dimethyl triazeno imidazole carboxamide. Cancer, 30: 1632.GREEN, D. M., and JAFFE, N. (1978) Progress and controversy in the treatment of childhood rhabdomyosarcoma.Cancer Treatment Reports, 5: 7.HORN, R C. JR., and ENTERLINE, H. T. (1958) Rhabdomyosarcoma: a clinicopathological study and classificationof 39 cases. Cancer, 11: 181.MAUER, H. M., MOON, T., and DONALDSON, M. (1977) Preliminary results of the Intergroup RhabdomyosarcomaStudy (IRS). In Management of Primary Bone and Soft Tissue Tumors. Year Book Medical Publishers, Inc.,p. 317.PRATT, C. B., SMITH, J. W., and WOERNER, S. (1978) Factors leading to delay in the diagnosis and affectingsurvival of children with head and neck rhabdomyosarcoma. Pediatrics, 61: 30.RAGAB, A. A., PHELAN, E. T , and RAZEK, A. A. (1977) Malignant tumors of the soft tissues. Clinical PediatricOncology. Eds. W. W. Sutow, T. J. Vietti, and D. J. Femback. The C. V. Mosby Co., St Louis, p. 569.STOUT, A. P., and LATTES, R (1967) Tumors of the soft tissues. Atlas Tumor Pathology. 2nd. Series, Fasc. 1.Armed Forces Institute of Pathology, Washington, DC, p. 138.Address for reprints:Dr. V. C. Canale,Divisione di Radioterapia e Oncologia Medica,Ospedale Civile di Pordenone,Via Montereale,33170,Pordenone,Italia.of use, available at https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S0022215100094561Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 14:43:05, subject to the Cambridge Core terms

Botryoid rhabdomyosarcoma of the nasopharynx

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