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Acantholytic squamous cell carcinoma of the oral cavity: a more aggressive entity?
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British Journal of Oral and Maxillofacial Surgery 47 (2009) 123–125
Short communication
Acantholytic squamous cell carcinoma of the oral cavity: a more aggressive entity? Cyrus J. Kerawala Department of Oral and Facial Surgery, North Hampshire Hospital, Aldermaston Road, Basingstoke, Hampshire, RG24 9NA, United Kingdom Accepted 25 April 2008 Available online 18 June 2008
Abstract Acantholytic squamous cell carcinoma is a histologically distinct variant of squamous cell carcinoma that arises most commonly in areas of the skin exposed to the sun. It is rare on mucosal surfaces of the upper aerodigestive tract, where there is some suggestion that it might behave more aggressively than conventional squamous cell carcinoma. This case which describes a 56-year-old patient presenting with Stage II disease of the tongue who succumbed to disease nine months after presentation despite aggressive multi-modality treatment adds weight to that argument. © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Acantholytic; Squamous cell carcinoma
Introduction Acantholytic (adenoid) squamous cell carcinoma (ASCC) is an uncommon, histologically distinct variant of squamous cell carcinoma (SCC). It is characterised by extensive acantholysis that results in pseudo-glandular or pseudo-vascular. It occurs most commonly in areas of the skin that are exposed to the sun, and was first recognised as a distinct entity in the 1940s.1 It has only rarely been documented in the mucosal upper aerodigestive tract.2–5 Its prognosis in the mucosal environment is controversial, with some reports suggesting more aggressive behaviour and poorer prognosis. This case adds weight to that argument.
Case report A 56-year-old man presented with a three-month history of a painful lesion on the right side of his tongue (Fig. 1). E-mail address: [email protected].
He was a non-smoker who drank fewer than two units of alcohol a week. Apart from a history of an aortic systolic murmur and depression that was treated with a selective serotonin re-uptake inhibitor he had maintained good health. An incisional biopsy confirmed an acantholytic variant of SCC with acantholysis of the malignant squamous cells producing pseudolumina that mimicked glandular differentiation (Fig. 2). This acantholytic change seemed to begin in the basal layer of the squamous epithelium. A staging computed tomogram (CT) from skull base to diaphragm was within normal limits. He had a right hemiglossectomy for what was clinically a T1 N0 lesion. His perioperative course was unremarkable. Subsequent histological examination showed a completely excised primary tumour 16 mm wide × 11 mm thick. Perineural infiltration was evident but there was no vascular invasion. The carcinoma was completely excised (margins >10 mm). Definitive histological examination of the excised specimen confirmed the preoperative diagnosis of an acantholytic invasive squamous cell carcinoma. Immunohistochemical and mucin stains excluded angiosarcoma and
0266-4356/$ – see front matter © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjoms.2008.04.018
124
C.J. Kerawala / British Journal of Oral and Maxillofacial Surgery 47 (2009) 123–125
Fig. 1. Clinical presentation.
adenocarcinoma, respectively. The proliferation rate of the tumour was high. None of the 31 lymph glands harvested from levels I–IV of the ipsilateral selective neck dissection were involved but in view of the histological variant in question and the aggressive local features, postoperative radiotherapy was given to the primary site. The patient was treated with a conformal CT plan 6 MV technique in two phases that gave a total of 60 Gy in 30 fractions over 40 days. Treatment began seven weeks postoperatively. The patient remained well until five months after the completion of radiotherapy when, at a routine review appointment, he complained of some difficulty swallowing. Although examination a month previously had been unremarkable he had developed evidence of bilateral cervical lymphadenopathy and recurrence at the primary site. Fibreoptic nasoendoscopy confirmed widespread laryngeal oedema. A subsequent CT showed an extensive soft tissue recurrence involving the floor of the mouth, submental area, suprahyoid musculature, bilateral carotid sheaths, and prevertebral soft tissues (Fig. 3). There was also widespread bilateral cervical lymphadenopathy with oedema throughout the neck. There was no significant mediastinal hilar or hilar lymphadenopathy.
Fig. 2. Biopsy showing acantholysis of the malignant squamous cells and pseudolumina (haematoxylin and eosin, original magnification × 100).
Fig. 3. Sagittal computed tomogram showing the extent of locoregional recurrent disease.
Soon after the CT the patient was admitted for control of increasing stridor. A combination of intravenous steroids, adrenalin nebulisers, and heliox stabilised matters briefly, but he died of locoregional recurrence nine months after his initial presentation. Discussion Acantholytic SCC is a well-recognised variant of squamous cell carcinoma that was first described in 1947.1 In his initial report Lever referred to the neoplasm as an adenocanthoma of sweat glands, but in subsequent studies it became evident that the tumour was a distinct variant of squamous cell carcinoma of non-eccrine origin. Although it commonly occurs in areas of the skin exposed to the sun it has only rarely been seen in mucosal sites such as the lip, oral cavity, nasopharynx, larynx, and tongue. The first reported case of the latter site was in 1977.6 The differential diagnosis includes adeno-squamous carcinoma, adenoid cystic carcinoma, metastatic adenocarcinoma, mucoepidermoid carcinoma and basiloid squamous cell carcinoma. Histological criteria for diagnosing ASCC include a conventional squamous cell component (usually well-differentiated) with pseudolumina containing single or grouped acantholytic and dyskeratotic epithelial cells or cellular debris under the squamous neoplastic component. This non-solid component usually shows an alveolar pattern or a pseudoglandular arrangement. Clear cells and a chronic inflammatory infiltrate are common. A spindle cell or desmoplastic reaction may also be present. ASCC arising in sun-damaged areas of the skin seems to have a slightly greater risk of recurrence and metastases
C.J. Kerawala / British Journal of Oral and Maxillofacial Surgery 47 (2009) 123–125
than conventional squamous cell carcinoma. Its prognosis in mucosal sites is, however, controversial. Though a good prognosis has been described in most cases, these arise in areas such as the lip, where tumours are easy to see and as a result often present early.7 In other sites such as the larynx there is a suggestion of more aggressive behaviour with higher rates of regional lymphadenopathy and recurrence.3,4 Gonz´alez-Vela et al. reported six patients with upper aerodigestive ASCC, four of whom developed recurrent tumours a mean of 15 months aftertreatment (range 2–30). All four died of their disease. The number of reported confirmed cases of upper aerodigestive ASCC is small. At sites other than the lip there is a suggestion that they are more aggressive than conventional squamous cell carcinomas, although there are fewer than half a dozen reported cases arising in the tongue. The focus of this report is to highlight the possible poorer prognosis of acantholytic SCC compared with that of squamous cell carcinoma, and to prompt clinicians to consider a more aggressive multidisciploinary treatment for these patients.
125
References 1. Lever WF. Adenocanthoma of sweat glands: carcinoma of sweat glands with glandular and epidermal elements. Report of 4 cases. Arch Dermatol Syphilol 1947;56:157–71. 2. Johnson WC, Helwig EB. Adenoid squamous cell carcinoma (adenoacanthoma). A clinicopathologic study of 155 patients. Cancer 1966;19:1639–50. 3. Zaatari GS, Santoianni RA. Adenoid squamous cell carcinoma of the nasopharynx and neck region. Arch Pathol Lab Med 1986;110:542– 6. 4. Gonz´alez-Vela MC, Val-Bernal JF, Mayorga M, Zaldumbide L, B´ascones M. Adenoid squamous cell carcinoma of the larynx: an uncommon histological variant of squamous cell carcinoma. APMIS 2006;114:470– 3. 5. Ferlito A, Devaney KO, Rinaldo A, Milroy CM, Carbone A. Mucosal adenoid squamous cell carcinoma of the head and neck. Ann Otol Rhinol Laryngol 1996;105:409–13. 6. Goldman RL, Klein HZ, Sung M. Adenoid squamous cell carcinoma of the oral cavity: report of the first case arising in the tongue. Arch Otolaryngol 1977;103:496–8. 7. Jones AC, Freedman PD, Kerpel SM. Oral adenoid squamous cell carcinoma: a report of three cases and review of the literature. J Oral Maxillofac Surg 1993;51:676–81.
British Journal of Oral and Maxillofacial Surgery 47 (2009) 123–125
Short communication
Acantholytic squamous cell carcinoma of the oral cavity: a more aggressive entity? Cyrus J. Kerawala Department of Oral and Facial Surgery, North Hampshire Hospital, Aldermaston Road, Basingstoke, Hampshire, RG24 9NA, United Kingdom Accepted 25 April 2008 Available online 18 June 2008
Abstract Acantholytic squamous cell carcinoma is a histologically distinct variant of squamous cell carcinoma that arises most commonly in areas of the skin exposed to the sun. It is rare on mucosal surfaces of the upper aerodigestive tract, where there is some suggestion that it might behave more aggressively than conventional squamous cell carcinoma. This case which describes a 56-year-old patient presenting with Stage II disease of the tongue who succumbed to disease nine months after presentation despite aggressive multi-modality treatment adds weight to that argument. © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Acantholytic; Squamous cell carcinoma
Introduction Acantholytic (adenoid) squamous cell carcinoma (ASCC) is an uncommon, histologically distinct variant of squamous cell carcinoma (SCC). It is characterised by extensive acantholysis that results in pseudo-glandular or pseudo-vascular. It occurs most commonly in areas of the skin that are exposed to the sun, and was first recognised as a distinct entity in the 1940s.1 It has only rarely been documented in the mucosal upper aerodigestive tract.2–5 Its prognosis in the mucosal environment is controversial, with some reports suggesting more aggressive behaviour and poorer prognosis. This case adds weight to that argument.
Case report A 56-year-old man presented with a three-month history of a painful lesion on the right side of his tongue (Fig. 1). E-mail address: [email protected].
He was a non-smoker who drank fewer than two units of alcohol a week. Apart from a history of an aortic systolic murmur and depression that was treated with a selective serotonin re-uptake inhibitor he had maintained good health. An incisional biopsy confirmed an acantholytic variant of SCC with acantholysis of the malignant squamous cells producing pseudolumina that mimicked glandular differentiation (Fig. 2). This acantholytic change seemed to begin in the basal layer of the squamous epithelium. A staging computed tomogram (CT) from skull base to diaphragm was within normal limits. He had a right hemiglossectomy for what was clinically a T1 N0 lesion. His perioperative course was unremarkable. Subsequent histological examination showed a completely excised primary tumour 16 mm wide × 11 mm thick. Perineural infiltration was evident but there was no vascular invasion. The carcinoma was completely excised (margins >10 mm). Definitive histological examination of the excised specimen confirmed the preoperative diagnosis of an acantholytic invasive squamous cell carcinoma. Immunohistochemical and mucin stains excluded angiosarcoma and
0266-4356/$ – see front matter © 2008 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjoms.2008.04.018
124
C.J. Kerawala / British Journal of Oral and Maxillofacial Surgery 47 (2009) 123–125
Fig. 1. Clinical presentation.
adenocarcinoma, respectively. The proliferation rate of the tumour was high. None of the 31 lymph glands harvested from levels I–IV of the ipsilateral selective neck dissection were involved but in view of the histological variant in question and the aggressive local features, postoperative radiotherapy was given to the primary site. The patient was treated with a conformal CT plan 6 MV technique in two phases that gave a total of 60 Gy in 30 fractions over 40 days. Treatment began seven weeks postoperatively. The patient remained well until five months after the completion of radiotherapy when, at a routine review appointment, he complained of some difficulty swallowing. Although examination a month previously had been unremarkable he had developed evidence of bilateral cervical lymphadenopathy and recurrence at the primary site. Fibreoptic nasoendoscopy confirmed widespread laryngeal oedema. A subsequent CT showed an extensive soft tissue recurrence involving the floor of the mouth, submental area, suprahyoid musculature, bilateral carotid sheaths, and prevertebral soft tissues (Fig. 3). There was also widespread bilateral cervical lymphadenopathy with oedema throughout the neck. There was no significant mediastinal hilar or hilar lymphadenopathy.
Fig. 2. Biopsy showing acantholysis of the malignant squamous cells and pseudolumina (haematoxylin and eosin, original magnification × 100).
Fig. 3. Sagittal computed tomogram showing the extent of locoregional recurrent disease.
Soon after the CT the patient was admitted for control of increasing stridor. A combination of intravenous steroids, adrenalin nebulisers, and heliox stabilised matters briefly, but he died of locoregional recurrence nine months after his initial presentation. Discussion Acantholytic SCC is a well-recognised variant of squamous cell carcinoma that was first described in 1947.1 In his initial report Lever referred to the neoplasm as an adenocanthoma of sweat glands, but in subsequent studies it became evident that the tumour was a distinct variant of squamous cell carcinoma of non-eccrine origin. Although it commonly occurs in areas of the skin exposed to the sun it has only rarely been seen in mucosal sites such as the lip, oral cavity, nasopharynx, larynx, and tongue. The first reported case of the latter site was in 1977.6 The differential diagnosis includes adeno-squamous carcinoma, adenoid cystic carcinoma, metastatic adenocarcinoma, mucoepidermoid carcinoma and basiloid squamous cell carcinoma. Histological criteria for diagnosing ASCC include a conventional squamous cell component (usually well-differentiated) with pseudolumina containing single or grouped acantholytic and dyskeratotic epithelial cells or cellular debris under the squamous neoplastic component. This non-solid component usually shows an alveolar pattern or a pseudoglandular arrangement. Clear cells and a chronic inflammatory infiltrate are common. A spindle cell or desmoplastic reaction may also be present. ASCC arising in sun-damaged areas of the skin seems to have a slightly greater risk of recurrence and metastases
C.J. Kerawala / British Journal of Oral and Maxillofacial Surgery 47 (2009) 123–125
than conventional squamous cell carcinoma. Its prognosis in mucosal sites is, however, controversial. Though a good prognosis has been described in most cases, these arise in areas such as the lip, where tumours are easy to see and as a result often present early.7 In other sites such as the larynx there is a suggestion of more aggressive behaviour with higher rates of regional lymphadenopathy and recurrence.3,4 Gonz´alez-Vela et al. reported six patients with upper aerodigestive ASCC, four of whom developed recurrent tumours a mean of 15 months aftertreatment (range 2–30). All four died of their disease. The number of reported confirmed cases of upper aerodigestive ASCC is small. At sites other than the lip there is a suggestion that they are more aggressive than conventional squamous cell carcinomas, although there are fewer than half a dozen reported cases arising in the tongue. The focus of this report is to highlight the possible poorer prognosis of acantholytic SCC compared with that of squamous cell carcinoma, and to prompt clinicians to consider a more aggressive multidisciploinary treatment for these patients.
125
References 1. Lever WF. Adenocanthoma of sweat glands: carcinoma of sweat glands with glandular and epidermal elements. Report of 4 cases. Arch Dermatol Syphilol 1947;56:157–71. 2. Johnson WC, Helwig EB. Adenoid squamous cell carcinoma (adenoacanthoma). A clinicopathologic study of 155 patients. Cancer 1966;19:1639–50. 3. Zaatari GS, Santoianni RA. Adenoid squamous cell carcinoma of the nasopharynx and neck region. Arch Pathol Lab Med 1986;110:542– 6. 4. Gonz´alez-Vela MC, Val-Bernal JF, Mayorga M, Zaldumbide L, B´ascones M. Adenoid squamous cell carcinoma of the larynx: an uncommon histological variant of squamous cell carcinoma. APMIS 2006;114:470– 3. 5. Ferlito A, Devaney KO, Rinaldo A, Milroy CM, Carbone A. Mucosal adenoid squamous cell carcinoma of the head and neck. Ann Otol Rhinol Laryngol 1996;105:409–13. 6. Goldman RL, Klein HZ, Sung M. Adenoid squamous cell carcinoma of the oral cavity: report of the first case arising in the tongue. Arch Otolaryngol 1977;103:496–8. 7. Jones AC, Freedman PD, Kerpel SM. Oral adenoid squamous cell carcinoma: a report of three cases and review of the literature. J Oral Maxillofac Surg 1993;51:676–81.