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Metastasizing chondroid syringoma: case report
Pathology (1969), 1, pp. 77-81
METASTASIZING CHONDROl D SYR I NGOMA : CASE REPORT
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
L. R. MATZ,D. J. MCCULLYand B. A. R. STOKES Departments of Pathology, Royal Perth Hospital, and of Pathology and Surgery, University of Western Australia
Summary A case of metastasizing chondroid syringoma of the scalp is reported i n which the secondaries showed the characteristics of the primary tumour. We were unable to find a similar case report i n the literature. The lesion was well circumscribed and lobulated, with nests of epithelial cells at the periphery of the lobules and a relatively acellular chondroid or mucinous matrix centrally. It differed from the usual descriptions of chondroid syringoma by exhibiting cellular pleomorphism, mitoses and islands of tumour cells away from the main tumour mass. These features may be useful in assessing the potential behaviour of chondroid syringomas.
Hirsch & Helwig (1961) introduced the term chondroid syringoma for a tumour of sweat gland origin which previously had been called mixed tumour of skin, salivary gland type. These authors considered their term a more precise and descriptive one because of the invariable presence of sweat gland elements (syringoma) and cartilage-like material (chondroid). It is not a common tumour of skin, there being just over 300 cases reported in the literature (Rosborough, 1963). The commonest site of the lesion is on the head and neck, and recurrence of the tumour after excision is believed to occur if clearance is inadequate (Hirsch & Helwig, 1961). There are only 3 cases reported in which there has been evidence of malignancy (Hirsch & Helwig, 1961; Rosborough, 1963; Sharvill, 1962), and only one of these tumoms had metastasized (Rosborough, 1963). On account of this rarity of malignant change, we record the clinical course and pathological features of a fatal case of malignant chondroid syringoma with widespread metastases.
CASE HISTORY Clinical Record An 80-year old woman first presented with a large painful lump over the right parieto-occipital region of the scalp; it had been present, slowly increasing in size, for 5 years. Examination showed a firm, elevated irregular oval-shaped skin lesion, 5 x 3.5 x 2.5 cm., which was freely mobile. (Fig. 1). Regional lymph nodes were not enlarged. Radiographs of skull and chest were normal. The clinical diagnosis was sebaceous adenoma, and a wide local excision performed. The histological diagnosis was chondroid syringoma, with a comment on the unusual degree of cellular pleomorphism present.
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78
MATZ
et al.
FIG. 1 Primary lesion, right parieto-occipital region of scalp
Nine months later, a local recurrence was excised and it again showed the microscopical appearance of a chondroid syringoma: it was commented that the tumour could be malignant. After a further nine months, the patient returned to hospital with a fungating 8 >; 7 cm. mass attached to the underlying bone in the right occipital region of the scalp. A hard enlarged lymph node was present in the right posterior triangle of the neck. Radiographs showed erosion of the occipital squama of the skull beneath the lesion, and rounded opacities in the right lung. The enlarged cervical lymph node was excised, and metastatic chondroid syringoma found on histopathological examination. The scalp mass was infused locally with Methotrexate (Lederle), and later treated by X-ray therapy. No appreciable change occurred and she died 7 years after the tumour first appeared.
Necropsy Findings Over the vertex of the skull, there was an ulcerated mass 8 cm. in diameter which had eroded bone. Inferiorly and to the right, there was a large ulcerated metastasis, and many other subcutaneous metastases were present, particularly over the thorax and anterior abdominal wall. There were multiple metastases in both lungs : they were rounded, firm, white and up to 1 cm. in diameter, with a larger deposit at the M u m of the right lung. No significant abnormalities were found in the other organs and, in particular, the major salivary glands were normal.
Histological Findings The primary tumour was circumscribed with peripheral iobulation and consisted of epithelial cells and a large amount of basophilic chondroid or mucinous matrix in which there were areas of degeneration. The epithelial cells tended to be grouped into nests at the periphery of an area of lobulation and showed differentiation into tubulo-alveolar or ductular structures
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METASTASIZING CHONDROID SYRINGOMA
79
FIG.2 Primary lesion showing pseudo-encapsulationand lobulation. Tumour composed of peripheral tubulo-alveolar structures with gradual thinning of cells and increase in mucoid matrix toward the centre. H & E X35
(Fig. 2). Towards the centre of a lobule, the cells thinned out into cords or single cells, so that centrally there were relatively acellular areas with large amounts of matrix. Most of the epithelial cells had a pale vesicular nucleus and a moderate amount of eosinophilic cytoplasm, but many others were pleomorphic showing variations in size, shape and cytoplasmic staining, as well as bizarre multinucleated cells, occasional mitoses, and degenerating individual cells. Vacuolated cells were also present. PAS and PAS-diastase demonstrated glycogen in the cells, especially in the centre of the lobules. Many cells contained fine granular lipid (Oil red 0). In several areas, islands of tumour cells had separated from the main mass. The matrix gave a strongly positive reaction with alcian blue and alcian green, a moderately positive reaction with Southgate’s muci-carmine, and a weakly positive PAS reaction. Reticulin fibres were plentiful, but no elastic fibres were seen. The appearances of the recurrent tumour and metastases were similar to the primary tumour, but there was less tendency to form tubulo-alveolar or ductular structures and a considerable increase in pleomorphism (Fig. 3). Other minor differences were less glycogen in the central cells of the lobules and increased reticulin in the matrix.
DISCUSSION The situation of the primary lesion on the scalp is consistent with the diagnosis of chondroid syringoma and excludes the possibility of a mixed tumour of heterotopic salivary gland tissue. T h e m m ~ & g ~ f a o r m a l f n a j osalivary r glands excludes metastasis from a mixed salivary gland tumour. The histological findings of an admixture of epithelial elements showing some differentiation into tubulo-alveolar or ductular structures and chondroid or myxoid matrix, is in accordance with previous descriptions of chondroid syringoma. Hirsch & Helwig (1961)
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
80
MATZ e t
al.
FIG. 3 Skin metastasis with features similar to primary tumour but showing more pleomorphism. H & E 124 \%
described an increased amount of glycogen in the cells of the central areas of the lobules. This was also present in our material in the primary tumour and may be a distinctive feature of chondroid syringomas. In retrospect, there were certain histopathological features of this primary tumour that are not described in benign chondroid syringoma which are worth emphasizing with regard to malignant potential. These features were cellular pleomorphism (Fig. 4), mitoses and islands of turnour cells separate from the main tumour. In the 3 cases of malignant chondroid syringoma in the literature, cellular pleomorphism was not reported. Mitoses were present in only one case, but islands of tumour cells separate from the main tumour were present in either the primary or recurrent lesions in all 3 cases. In the first case (Hirsch & Helwig, 1961) the diagnosis was morphologically based, there being no recurrence at the time of reporting. The second case ( Sharvill, 1962) recurred locally with histological features typical of carcinomatous invasion of the surrounding adipose tissue. The third (Rosborough, 1963) developed a metastasis in the regional lymph node and is of interest in that the histological appearances of the nodes were those of a clear or squamous cell carcinoma and had none of the traits of the primary lesion. Our case, then, is of added significance as it is the first to be reported in which the metastases retained the characteristics of the primary tumour. The clinical history of a slowly enlarging mass in the skin over a period of 5 years, followed by rapid spread with downhill course after surgical intervention, is typical of sweat gland carcinomas (Smith, 1955). In a review of 21 cases of metastasizing carcinoma of sweat glands Smith found the average interval between noticing the lesion and presenting for treatment was 11 years. Most cases dying of metastatic sweat gland carcinoma do so within 4 years of primary excision (Paine, 1965). ACKNOWLEDGEMENTS The authors thank Mr H. Upenieks of the Department of Medical Illustration, University of Western Australia, for preparation of the photographs.
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
METASTASIZING CHONDROID SYRINGOMA
81
FIG. 4 Area of pleomorphism in primary tumour which should suggest potentially malignant behaviour. H & E X 124
References HIRSCH, P. & HELWIG, E. B. (1961): Chondroid syringoma. Arch. Derm. 84, 835-847. PAINE,C. H. (1965): Very late recurrence of a previously excised sweat gland carcinoma : case report with review of the literature. Brit. 3. Cancer 19, 263-267. ROSBOROUGH, D. (1963): Malignant mixed tumours of the skin. Brit. 3. Surg. 50, 697-699.
SHARVILL, D. E. (1962) : Mixed-saavary tvpe tumour of the skin with malignant fecurrence. Brit. 3. Derrn. 14, 103-104. SMITH, C. C. K. (1955):Metastasizing carcinoma of the sweat-glands. Brit. 3. Surg. 43, 80-84. STOUT, A. P. & GOWN, J. G. (1959): Mixed turnours of the skin of the salivary gland type. Cancer (Philad.) 12, 537-543.
METASTASIZING CHONDROl D SYR I NGOMA : CASE REPORT
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
L. R. MATZ,D. J. MCCULLYand B. A. R. STOKES Departments of Pathology, Royal Perth Hospital, and of Pathology and Surgery, University of Western Australia
Summary A case of metastasizing chondroid syringoma of the scalp is reported i n which the secondaries showed the characteristics of the primary tumour. We were unable to find a similar case report i n the literature. The lesion was well circumscribed and lobulated, with nests of epithelial cells at the periphery of the lobules and a relatively acellular chondroid or mucinous matrix centrally. It differed from the usual descriptions of chondroid syringoma by exhibiting cellular pleomorphism, mitoses and islands of tumour cells away from the main tumour mass. These features may be useful in assessing the potential behaviour of chondroid syringomas.
Hirsch & Helwig (1961) introduced the term chondroid syringoma for a tumour of sweat gland origin which previously had been called mixed tumour of skin, salivary gland type. These authors considered their term a more precise and descriptive one because of the invariable presence of sweat gland elements (syringoma) and cartilage-like material (chondroid). It is not a common tumour of skin, there being just over 300 cases reported in the literature (Rosborough, 1963). The commonest site of the lesion is on the head and neck, and recurrence of the tumour after excision is believed to occur if clearance is inadequate (Hirsch & Helwig, 1961). There are only 3 cases reported in which there has been evidence of malignancy (Hirsch & Helwig, 1961; Rosborough, 1963; Sharvill, 1962), and only one of these tumoms had metastasized (Rosborough, 1963). On account of this rarity of malignant change, we record the clinical course and pathological features of a fatal case of malignant chondroid syringoma with widespread metastases.
CASE HISTORY Clinical Record An 80-year old woman first presented with a large painful lump over the right parieto-occipital region of the scalp; it had been present, slowly increasing in size, for 5 years. Examination showed a firm, elevated irregular oval-shaped skin lesion, 5 x 3.5 x 2.5 cm., which was freely mobile. (Fig. 1). Regional lymph nodes were not enlarged. Radiographs of skull and chest were normal. The clinical diagnosis was sebaceous adenoma, and a wide local excision performed. The histological diagnosis was chondroid syringoma, with a comment on the unusual degree of cellular pleomorphism present.
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
78
MATZ
et al.
FIG. 1 Primary lesion, right parieto-occipital region of scalp
Nine months later, a local recurrence was excised and it again showed the microscopical appearance of a chondroid syringoma: it was commented that the tumour could be malignant. After a further nine months, the patient returned to hospital with a fungating 8 >; 7 cm. mass attached to the underlying bone in the right occipital region of the scalp. A hard enlarged lymph node was present in the right posterior triangle of the neck. Radiographs showed erosion of the occipital squama of the skull beneath the lesion, and rounded opacities in the right lung. The enlarged cervical lymph node was excised, and metastatic chondroid syringoma found on histopathological examination. The scalp mass was infused locally with Methotrexate (Lederle), and later treated by X-ray therapy. No appreciable change occurred and she died 7 years after the tumour first appeared.
Necropsy Findings Over the vertex of the skull, there was an ulcerated mass 8 cm. in diameter which had eroded bone. Inferiorly and to the right, there was a large ulcerated metastasis, and many other subcutaneous metastases were present, particularly over the thorax and anterior abdominal wall. There were multiple metastases in both lungs : they were rounded, firm, white and up to 1 cm. in diameter, with a larger deposit at the M u m of the right lung. No significant abnormalities were found in the other organs and, in particular, the major salivary glands were normal.
Histological Findings The primary tumour was circumscribed with peripheral iobulation and consisted of epithelial cells and a large amount of basophilic chondroid or mucinous matrix in which there were areas of degeneration. The epithelial cells tended to be grouped into nests at the periphery of an area of lobulation and showed differentiation into tubulo-alveolar or ductular structures
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
METASTASIZING CHONDROID SYRINGOMA
79
FIG.2 Primary lesion showing pseudo-encapsulationand lobulation. Tumour composed of peripheral tubulo-alveolar structures with gradual thinning of cells and increase in mucoid matrix toward the centre. H & E X35
(Fig. 2). Towards the centre of a lobule, the cells thinned out into cords or single cells, so that centrally there were relatively acellular areas with large amounts of matrix. Most of the epithelial cells had a pale vesicular nucleus and a moderate amount of eosinophilic cytoplasm, but many others were pleomorphic showing variations in size, shape and cytoplasmic staining, as well as bizarre multinucleated cells, occasional mitoses, and degenerating individual cells. Vacuolated cells were also present. PAS and PAS-diastase demonstrated glycogen in the cells, especially in the centre of the lobules. Many cells contained fine granular lipid (Oil red 0). In several areas, islands of tumour cells had separated from the main mass. The matrix gave a strongly positive reaction with alcian blue and alcian green, a moderately positive reaction with Southgate’s muci-carmine, and a weakly positive PAS reaction. Reticulin fibres were plentiful, but no elastic fibres were seen. The appearances of the recurrent tumour and metastases were similar to the primary tumour, but there was less tendency to form tubulo-alveolar or ductular structures and a considerable increase in pleomorphism (Fig. 3). Other minor differences were less glycogen in the central cells of the lobules and increased reticulin in the matrix.
DISCUSSION The situation of the primary lesion on the scalp is consistent with the diagnosis of chondroid syringoma and excludes the possibility of a mixed tumour of heterotopic salivary gland tissue. T h e m m ~ & g ~ f a o r m a l f n a j osalivary r glands excludes metastasis from a mixed salivary gland tumour. The histological findings of an admixture of epithelial elements showing some differentiation into tubulo-alveolar or ductular structures and chondroid or myxoid matrix, is in accordance with previous descriptions of chondroid syringoma. Hirsch & Helwig (1961)
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
80
MATZ e t
al.
FIG. 3 Skin metastasis with features similar to primary tumour but showing more pleomorphism. H & E 124 \%
described an increased amount of glycogen in the cells of the central areas of the lobules. This was also present in our material in the primary tumour and may be a distinctive feature of chondroid syringomas. In retrospect, there were certain histopathological features of this primary tumour that are not described in benign chondroid syringoma which are worth emphasizing with regard to malignant potential. These features were cellular pleomorphism (Fig. 4), mitoses and islands of turnour cells separate from the main tumour. In the 3 cases of malignant chondroid syringoma in the literature, cellular pleomorphism was not reported. Mitoses were present in only one case, but islands of tumour cells separate from the main tumour were present in either the primary or recurrent lesions in all 3 cases. In the first case (Hirsch & Helwig, 1961) the diagnosis was morphologically based, there being no recurrence at the time of reporting. The second case ( Sharvill, 1962) recurred locally with histological features typical of carcinomatous invasion of the surrounding adipose tissue. The third (Rosborough, 1963) developed a metastasis in the regional lymph node and is of interest in that the histological appearances of the nodes were those of a clear or squamous cell carcinoma and had none of the traits of the primary lesion. Our case, then, is of added significance as it is the first to be reported in which the metastases retained the characteristics of the primary tumour. The clinical history of a slowly enlarging mass in the skin over a period of 5 years, followed by rapid spread with downhill course after surgical intervention, is typical of sweat gland carcinomas (Smith, 1955). In a review of 21 cases of metastasizing carcinoma of sweat glands Smith found the average interval between noticing the lesion and presenting for treatment was 11 years. Most cases dying of metastatic sweat gland carcinoma do so within 4 years of primary excision (Paine, 1965). ACKNOWLEDGEMENTS The authors thank Mr H. Upenieks of the Department of Medical Illustration, University of Western Australia, for preparation of the photographs.
Pathology Downloaded from informahealthcare.com by Nyu Medical Center on 06/02/15 For personal use only.
METASTASIZING CHONDROID SYRINGOMA
81
FIG. 4 Area of pleomorphism in primary tumour which should suggest potentially malignant behaviour. H & E X 124
References HIRSCH, P. & HELWIG, E. B. (1961): Chondroid syringoma. Arch. Derm. 84, 835-847. PAINE,C. H. (1965): Very late recurrence of a previously excised sweat gland carcinoma : case report with review of the literature. Brit. 3. Cancer 19, 263-267. ROSBOROUGH, D. (1963): Malignant mixed tumours of the skin. Brit. 3. Surg. 50, 697-699.
SHARVILL, D. E. (1962) : Mixed-saavary tvpe tumour of the skin with malignant fecurrence. Brit. 3. Derrn. 14, 103-104. SMITH, C. C. K. (1955):Metastasizing carcinoma of the sweat-glands. Brit. 3. Surg. 43, 80-84. STOUT, A. P. & GOWN, J. G. (1959): Mixed turnours of the skin of the salivary gland type. Cancer (Philad.) 12, 537-543.