International Journal of Pediatric Otorhinolaryngology, 24 (1992) 269-274 0 1992 Elsevier Science Publishers B.V. All rights reserved 0165-5876/92/$05.00
269
PEDOT 00812
Endodermal Enrico
sinus (yolk sac) tumor of the parotid gland: a case report
Viva a, Faust0 Zorzi b, Giacomo Annibale Carla Baronchelli b and Maria Fausta a Department of Maxilla-Surgery,
‘, Stefania Bonetti b
Stefini
‘,
and b 1st Department of Pathology, Spedali Civili Brescia, and ’ Otorhinolaryngoiatric Section, Children’s Hospital Umberto I”, Brescia (Italy)
(Received 25 June 1991) (Revised version received 23 December 1991) (Accepted 24 December 1991)
Key words: Endodermal sinus tumor; Yolk sac tumor; Malignant salivary gland tumor
Abstract
Malignant salivary gland neoplasms in children are rare, most common being mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma and adenocarcinoma. Most germ-cell neoplasms of head and neck in children are teratomas. The authors report a case of endodermal sinus tumor (EST) of the parotid gland in a 2-year-old girl, which recurred after chemotherapy. The role of a-fetoprotein LWP) serum level as a helpful marker in differential diagnosis and in evaluating tumor progression is underlined.
Introduction
Malignant salivary gland neoplasms in children are rare [1,5,7]. They are reported in newborns and during the first year of life [15,20], but they are usually described in older children [3,16,27]. If vascular tumors are excluded, nearly 50% of all salivary gland neoplasms in children are malignant, with a frequency that is 2.5 time higher than in adults. The most common histological types are mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma and adenocarcinema [ll]. Tumors derived from organ rudiments are extremely rare in salivary glands [13,27,30]. One case of ‘embryonal carcinoma’ of the parotid gland was Correspondence
to: Dr. Enrico Viva, via Garzetta 28, 25060 Brescia, Italy.
270
Fig. 1. Neoformation
of the right parotid gland characterized by quick evolutions, covered by ealthy cutis. There is no paralysis of the facial nerve.
described by Donath in a lZyear-old boy, after reviewing a series of 2876 salivary gland tumors [91. Approximately 5% of all germinal cell neoplasms in children appear in the soft tissues of the head and neck [S]. In this topographic site they are principally teratomas, displaying a spectrum of tissue type and maturation; a proportion contains elements of endodermal sinus tumor (EST) either in pure form or admixed with teratomatous tumors. The authors report a case of endoderma1 sinus tumor arising in the parotid gland of a 2-year-old girl.
Case report
A 2-year-old girl presented with a painful lump on the right cheek dating from 1 month (Fig. 1). The lesion rapidly increased in volume and fever appeared. Ultrasonography and TC scan showed a large, 6 x 4 cm, quite defined mass in the right parotid region (Fig. 2). A total right parotidectomy with homolateral jugo-digastric lymphadenectomy and preservation of the facial nerve was performed. The high friabiiity of the tumor and the lack of a true capsule did not allow radical surgery. Intraoperative histology showed an unusual papillary adenocarcinoma. A cycle of radiotherapy was started; however, it was stopped at a dose of 2100 rads
Fig. 2. CT shows a voluminous
mass of the right parotid gland. Its structure contours are not cleary irregular.
is dishomogeneous
and the
due to the renewal of tumoral growth. Later, serum cy-fetoprotein (AFP) determination revealed a high level of the protein (83000 ng/ml>, while carcinoma embryonic antigen (CEA) and human chorionic gonadotropin (HCG) were normal. A final histological diagnosis of EST was suggested. In the meanwhile the girl went to the Institute Gustave Roussy for consultant hospitalization. A recurrence appeared in the original site and a chest X-ray revealed small nodules spread over both pulmonary fields. At this Institution chemotherapy was tried using the protocol ‘Teratome’. After an initial remission with neoplastic mass reduction, disappearance of the pulmonary nodules and a fall in AFP levels (12 ng/ml), starting from the seventh month of therapy, the tumor relapsed, accompanied by increasing AFP levels. The girl died from neoplastic progression 18 months after the therapy was started. An autopsy was not performed. Pathological specimens consisted of about 10 cc of friable, red-yellowish tissue fragments, 2 periparotid and 1 jugo-digastric lymph nodes. On histology the tumor showed a prevalent reticular pattern, characterized by an intercommunicating labyrinth of small glandular spaces. Somewhere else the tumor contained microcystic spaces with some papillary structure lined by neoplastic cells (Fig. 3). PAS-positive, diastase-resistant, intracellular and extracellular globules were present. In a few places ductal structures resembling residual salivary gland excretory ducts were recognized mingled within the neoplastic proliferation. The jugo-digastric lymph
Fig. 3. Irregular-tubular
spaces and a large papillary structure lined by a monostrate of cuboidal cells.
node showed massive metastatic localization. Immunohistochemical analysis revealed a focal AFP positivity in some neoplastic cell, while CEA, a,-antitrypsin MAT) and HCG were completely negative.
Discussion ESTs are malignant germ-cell neoplasms. Their usual presentation is the testis and ovary. Extragonadal sites are rare, but well known. They have been described in anterior mediastinum [121, sacro-coccygeal region [lo], retroperitoneum and, less frequently, orbit [18,26], thymus [22], head and neck [8,171, liver [14,231, stomach [21], vulva and clitoris [29,4], vagina [61, cervix of the uterus [6], endometrium [24], urinary bladder [281, prostate [21 and pineal region. Salivary-gland localization of ESTs and other germ-cell neoplasms with extraembryonal differentiation seems so far unknown, even though teratomas have been described in the soft tissue of the head and neck. Differential diagnosis with adenocarcinoma should be considered in the absence of serum APP determination, especially when the more characteristic morphological patterns of EST are not prevalent. AFP serum level has been a marker of the neoplasm and also a clear indicator of the response to antiblastic therapy; a decreasing AFP level anticipates neoplastic mass reduction and the disappearance of pulmonary metas-
273
tases, whereas a new increase markes the neoplastic relapse. Like EST in gonadal and other extragonadal sites, the salivary gland localization also appears to be very aggressive, with a short survival time and a very short disease-free interval.
References 1 Baker S.R. and Malone B., Salivary gland malignancies in children, Cancer, 55 (1985) 1730-1736. 2 Benson R.C., Segura J.W. and Carney J.A., Primary yolk sac (endodermal sinus) tumor of the prostate, Cancer, 41 (1978) 1395-1398. 3 Byers M.R., Piorkowski R. and Luna M.A., Malignant parotid tumors in patients under 20 years of age, Arch. Otolaryngol., 110 (1984) 232-235. 4 Castaldo T.W., Petrilli ES., Ballon S.C., Voet R.L., Lagasse L.D. and Lubens R., Endodermal sinus tumor of the clitoris, Gynecol. Oncol., 9 (1980) 376-380. 5 Castro E.B., Huvos A.G., Strong E.W. and Foote F.W., Jr., Tumors of the major salivary gland in children, Cancer, 29 (1972) 312-317. 6 Copeland L.J., Sneige N., Ordonez N.G. et al., Endodermal sinus tumor of the vagina and cervix. Cancer, 55 (1985) 2558-2565. 7 Dahlquist A. and Gstberg, Malignant salivary gland tumors in children, Acta Otolaryngol.. 94 (1982) 175-179. 8 Denher L.P., Mills A., Talerman A., Billman G.F., Krous H.F. and Plaz C.E., Germ-cell neoplasms of head and neck: a pathologic spectrum of teratomatous and endodermal sinus tumors, Hum. Pathol., 21 (1990) 309-318. 9 Donath K, Seifert G. and Lentrodt J., The embryonal carcinoma of the parotid gland: a rare example of an embryonal tumor, Virch. Arch. (A), 403 (1984) 425-440. 10 Ein S., Mancer K. and Adeyemi D., Malignant sacrococcygeal teratoma-endodermal sinus yolk sac tumor in infants and children: a 32 years review, J. Pediatr. Surg., 20 (1985) 473-476. 11 Evenson J.W. and Cawson R.A., Salivary gland tumors: a review of 2410 cases with particular reference to histological types, sites, age and sex distribution, J. Pathol., 146 (1985) 51-58. I2 Gooneratne S., Keh P., Sreekanth S., Recant W. and Talerman A., Anterior mediastinal endodermal sinus (yolk sac) tumor in a female infant, Cancer, 56 (1985) 1430-1433. 13 Harris M.D., MC Keever P. and Robertson J.M., Congenital tumors of the salivary gland: a case report and review, Histopathology, 17 (1990) 155-157. 14 Hart W.R., Primary endodermal sinus (yolk sac) tumor of the liver, Cancer, 35 (1975) 1453-1458. 15 Hendrick S.W., Mucoepidermoid cancer of the parotid gland in a one-year-old child. Am. J. Surg.. 108 (1964) 907-909. 16 Kauffman S.L. and Stout A.P. Tumor of the major salivary glands in children, Cancer, 16 (1963) 1317-1331. 17 Lack E.E.. Extragonadal germ cell tumors of the head and neck region: review of 16 cases. Hum. Pathol., 16 (1985) 56-64. 18 Margo C.E.. Folgerg R., Zimmerman L.E. and Sesterhenn LA., Endodermal sinus tumor (yolk sac tumor) of the orbit, Ophthalmology, 90 (1983) 1426-1432. 19 Marlow J.F. and Hora J.F., Parotid mucoepidermoid carcinoma in children, Laryngoscope. 7X (1968) 68-72. 20 M&night H.A., Malignant parotid tumor in the newborn, Am. J. Surg., 45 (1939) 128-130. 21 Motoyama, T., Saito K., lwafuchi M. and Watanabe H.. Endodermal sinus tumor of the stomach, Acta Pathol. Jpn., 35 (1985) 497-505. 22 Nakagawa H., Sonoda T., Shirono S., Shinohara S., Tanaka T. and Tanaka S.A case of endodermal sinus tumor of the thymus, Rinsho Hoshasen, 26 (1981) 395-398. 23 Narita T., Moriyama Y. and Ito Y., Endodermal sinus (yolk sac) tumor of the liver: a case report and review of the literature, J. Pathol., 155 (1988) 41-47.
214 24 Pileri S., Martinelli G., Serra L. and Bazzocchi F. Endodermal sinus tumor arising in the endometrium, Obstet. Gynecol., 56 (1980) 391-396. 2.5 Rose P.E. and Howard E.R., Congenital teratoma of submandibular gland, J. Pediatr. Surg., 17 (1982) 414-416. 26 Roth L., Yolk sac tumor of primary orbital localization, Ann. Pediatr., 31 (1984) 693-695. 27 Schuller D.E. and MC Cabe B.F., Salivary gland neoplasm in children, Otolaryngol. Clin. North Am., 10 (1977) 399-412. 28 Taylor G., Jordan M., Churchill B. and Mancer K., Yolk sac tumor of the bladder, J. Urol., 129 (1983) 591-594. 29 Ungerleider R.S., Donaldson S.S., Warnke R.A. and Wilbur J.R., Endodermal sinus tumor: the Stanford experience and the first reported case arising in the vulva, Cancer, 41 (1978) 1627-1634. 30 Vawter G.F. and Tefft M., Congenital tumors of the parotid gland. Arch. Pathol., 82 (1966) 242-245.
269
PEDOT 00812
Endodermal Enrico
sinus (yolk sac) tumor of the parotid gland: a case report
Viva a, Faust0 Zorzi b, Giacomo Annibale Carla Baronchelli b and Maria Fausta a Department of Maxilla-Surgery,
‘, Stefania Bonetti b
Stefini
‘,
and b 1st Department of Pathology, Spedali Civili Brescia, and ’ Otorhinolaryngoiatric Section, Children’s Hospital Umberto I”, Brescia (Italy)
(Received 25 June 1991) (Revised version received 23 December 1991) (Accepted 24 December 1991)
Key words: Endodermal sinus tumor; Yolk sac tumor; Malignant salivary gland tumor
Abstract
Malignant salivary gland neoplasms in children are rare, most common being mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma and adenocarcinoma. Most germ-cell neoplasms of head and neck in children are teratomas. The authors report a case of endodermal sinus tumor (EST) of the parotid gland in a 2-year-old girl, which recurred after chemotherapy. The role of a-fetoprotein LWP) serum level as a helpful marker in differential diagnosis and in evaluating tumor progression is underlined.
Introduction
Malignant salivary gland neoplasms in children are rare [1,5,7]. They are reported in newborns and during the first year of life [15,20], but they are usually described in older children [3,16,27]. If vascular tumors are excluded, nearly 50% of all salivary gland neoplasms in children are malignant, with a frequency that is 2.5 time higher than in adults. The most common histological types are mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma and adenocarcinema [ll]. Tumors derived from organ rudiments are extremely rare in salivary glands [13,27,30]. One case of ‘embryonal carcinoma’ of the parotid gland was Correspondence
to: Dr. Enrico Viva, via Garzetta 28, 25060 Brescia, Italy.
270
Fig. 1. Neoformation
of the right parotid gland characterized by quick evolutions, covered by ealthy cutis. There is no paralysis of the facial nerve.
described by Donath in a lZyear-old boy, after reviewing a series of 2876 salivary gland tumors [91. Approximately 5% of all germinal cell neoplasms in children appear in the soft tissues of the head and neck [S]. In this topographic site they are principally teratomas, displaying a spectrum of tissue type and maturation; a proportion contains elements of endodermal sinus tumor (EST) either in pure form or admixed with teratomatous tumors. The authors report a case of endoderma1 sinus tumor arising in the parotid gland of a 2-year-old girl.
Case report
A 2-year-old girl presented with a painful lump on the right cheek dating from 1 month (Fig. 1). The lesion rapidly increased in volume and fever appeared. Ultrasonography and TC scan showed a large, 6 x 4 cm, quite defined mass in the right parotid region (Fig. 2). A total right parotidectomy with homolateral jugo-digastric lymphadenectomy and preservation of the facial nerve was performed. The high friabiiity of the tumor and the lack of a true capsule did not allow radical surgery. Intraoperative histology showed an unusual papillary adenocarcinoma. A cycle of radiotherapy was started; however, it was stopped at a dose of 2100 rads
Fig. 2. CT shows a voluminous
mass of the right parotid gland. Its structure contours are not cleary irregular.
is dishomogeneous
and the
due to the renewal of tumoral growth. Later, serum cy-fetoprotein (AFP) determination revealed a high level of the protein (83000 ng/ml>, while carcinoma embryonic antigen (CEA) and human chorionic gonadotropin (HCG) were normal. A final histological diagnosis of EST was suggested. In the meanwhile the girl went to the Institute Gustave Roussy for consultant hospitalization. A recurrence appeared in the original site and a chest X-ray revealed small nodules spread over both pulmonary fields. At this Institution chemotherapy was tried using the protocol ‘Teratome’. After an initial remission with neoplastic mass reduction, disappearance of the pulmonary nodules and a fall in AFP levels (12 ng/ml), starting from the seventh month of therapy, the tumor relapsed, accompanied by increasing AFP levels. The girl died from neoplastic progression 18 months after the therapy was started. An autopsy was not performed. Pathological specimens consisted of about 10 cc of friable, red-yellowish tissue fragments, 2 periparotid and 1 jugo-digastric lymph nodes. On histology the tumor showed a prevalent reticular pattern, characterized by an intercommunicating labyrinth of small glandular spaces. Somewhere else the tumor contained microcystic spaces with some papillary structure lined by neoplastic cells (Fig. 3). PAS-positive, diastase-resistant, intracellular and extracellular globules were present. In a few places ductal structures resembling residual salivary gland excretory ducts were recognized mingled within the neoplastic proliferation. The jugo-digastric lymph
Fig. 3. Irregular-tubular
spaces and a large papillary structure lined by a monostrate of cuboidal cells.
node showed massive metastatic localization. Immunohistochemical analysis revealed a focal AFP positivity in some neoplastic cell, while CEA, a,-antitrypsin MAT) and HCG were completely negative.
Discussion ESTs are malignant germ-cell neoplasms. Their usual presentation is the testis and ovary. Extragonadal sites are rare, but well known. They have been described in anterior mediastinum [121, sacro-coccygeal region [lo], retroperitoneum and, less frequently, orbit [18,26], thymus [22], head and neck [8,171, liver [14,231, stomach [21], vulva and clitoris [29,4], vagina [61, cervix of the uterus [6], endometrium [24], urinary bladder [281, prostate [21 and pineal region. Salivary-gland localization of ESTs and other germ-cell neoplasms with extraembryonal differentiation seems so far unknown, even though teratomas have been described in the soft tissue of the head and neck. Differential diagnosis with adenocarcinoma should be considered in the absence of serum APP determination, especially when the more characteristic morphological patterns of EST are not prevalent. AFP serum level has been a marker of the neoplasm and also a clear indicator of the response to antiblastic therapy; a decreasing AFP level anticipates neoplastic mass reduction and the disappearance of pulmonary metas-
273
tases, whereas a new increase markes the neoplastic relapse. Like EST in gonadal and other extragonadal sites, the salivary gland localization also appears to be very aggressive, with a short survival time and a very short disease-free interval.
References 1 Baker S.R. and Malone B., Salivary gland malignancies in children, Cancer, 55 (1985) 1730-1736. 2 Benson R.C., Segura J.W. and Carney J.A., Primary yolk sac (endodermal sinus) tumor of the prostate, Cancer, 41 (1978) 1395-1398. 3 Byers M.R., Piorkowski R. and Luna M.A., Malignant parotid tumors in patients under 20 years of age, Arch. Otolaryngol., 110 (1984) 232-235. 4 Castaldo T.W., Petrilli ES., Ballon S.C., Voet R.L., Lagasse L.D. and Lubens R., Endodermal sinus tumor of the clitoris, Gynecol. Oncol., 9 (1980) 376-380. 5 Castro E.B., Huvos A.G., Strong E.W. and Foote F.W., Jr., Tumors of the major salivary gland in children, Cancer, 29 (1972) 312-317. 6 Copeland L.J., Sneige N., Ordonez N.G. et al., Endodermal sinus tumor of the vagina and cervix. Cancer, 55 (1985) 2558-2565. 7 Dahlquist A. and Gstberg, Malignant salivary gland tumors in children, Acta Otolaryngol.. 94 (1982) 175-179. 8 Denher L.P., Mills A., Talerman A., Billman G.F., Krous H.F. and Plaz C.E., Germ-cell neoplasms of head and neck: a pathologic spectrum of teratomatous and endodermal sinus tumors, Hum. Pathol., 21 (1990) 309-318. 9 Donath K, Seifert G. and Lentrodt J., The embryonal carcinoma of the parotid gland: a rare example of an embryonal tumor, Virch. Arch. (A), 403 (1984) 425-440. 10 Ein S., Mancer K. and Adeyemi D., Malignant sacrococcygeal teratoma-endodermal sinus yolk sac tumor in infants and children: a 32 years review, J. Pediatr. Surg., 20 (1985) 473-476. 11 Evenson J.W. and Cawson R.A., Salivary gland tumors: a review of 2410 cases with particular reference to histological types, sites, age and sex distribution, J. Pathol., 146 (1985) 51-58. I2 Gooneratne S., Keh P., Sreekanth S., Recant W. and Talerman A., Anterior mediastinal endodermal sinus (yolk sac) tumor in a female infant, Cancer, 56 (1985) 1430-1433. 13 Harris M.D., MC Keever P. and Robertson J.M., Congenital tumors of the salivary gland: a case report and review, Histopathology, 17 (1990) 155-157. 14 Hart W.R., Primary endodermal sinus (yolk sac) tumor of the liver, Cancer, 35 (1975) 1453-1458. 15 Hendrick S.W., Mucoepidermoid cancer of the parotid gland in a one-year-old child. Am. J. Surg.. 108 (1964) 907-909. 16 Kauffman S.L. and Stout A.P. Tumor of the major salivary glands in children, Cancer, 16 (1963) 1317-1331. 17 Lack E.E.. Extragonadal germ cell tumors of the head and neck region: review of 16 cases. Hum. Pathol., 16 (1985) 56-64. 18 Margo C.E.. Folgerg R., Zimmerman L.E. and Sesterhenn LA., Endodermal sinus tumor (yolk sac tumor) of the orbit, Ophthalmology, 90 (1983) 1426-1432. 19 Marlow J.F. and Hora J.F., Parotid mucoepidermoid carcinoma in children, Laryngoscope. 7X (1968) 68-72. 20 M&night H.A., Malignant parotid tumor in the newborn, Am. J. Surg., 45 (1939) 128-130. 21 Motoyama, T., Saito K., lwafuchi M. and Watanabe H.. Endodermal sinus tumor of the stomach, Acta Pathol. Jpn., 35 (1985) 497-505. 22 Nakagawa H., Sonoda T., Shirono S., Shinohara S., Tanaka T. and Tanaka S.A case of endodermal sinus tumor of the thymus, Rinsho Hoshasen, 26 (1981) 395-398. 23 Narita T., Moriyama Y. and Ito Y., Endodermal sinus (yolk sac) tumor of the liver: a case report and review of the literature, J. Pathol., 155 (1988) 41-47.
214 24 Pileri S., Martinelli G., Serra L. and Bazzocchi F. Endodermal sinus tumor arising in the endometrium, Obstet. Gynecol., 56 (1980) 391-396. 2.5 Rose P.E. and Howard E.R., Congenital teratoma of submandibular gland, J. Pediatr. Surg., 17 (1982) 414-416. 26 Roth L., Yolk sac tumor of primary orbital localization, Ann. Pediatr., 31 (1984) 693-695. 27 Schuller D.E. and MC Cabe B.F., Salivary gland neoplasm in children, Otolaryngol. Clin. North Am., 10 (1977) 399-412. 28 Taylor G., Jordan M., Churchill B. and Mancer K., Yolk sac tumor of the bladder, J. Urol., 129 (1983) 591-594. 29 Ungerleider R.S., Donaldson S.S., Warnke R.A. and Wilbur J.R., Endodermal sinus tumor: the Stanford experience and the first reported case arising in the vulva, Cancer, 41 (1978) 1627-1634. 30 Vawter G.F. and Tefft M., Congenital tumors of the parotid gland. Arch. Pathol., 82 (1966) 242-245.
