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Chondroid syringoma of the foot
TheFoot(l99414,
113-116
0 1994 Longman Group Ltd
Chondroid syringoma of the foot P. J. Anderson, M. A. Rahilly Department of Surgery, Eastern General Hospital, Edinburgh, UK and Department of Pathology, University of Edinburgh, Edinburgh, UK S UMMA R Y. Chondroid syringoma or mixed tumour of the skin is a relatively uncommon neoplasm thought to originate in sweat glands.’ They are usually found in the head and neck or trunk and have rarely been reported arising on the foot. 4 cases are presented which occurred on the plantar aspect of the foot. There was a history of trauma in 3 of the cases, and 1 recurred following excision. The number of specialities involved in the care of this group of patients is also highlighted. We can find only 6 previous cases of chondroid syringoma arising on the foot described in the world literature. We report on the clinicopathological features of a further 4 cases diagnosed in Edinburgh over a 9-year period.
referred by her general practitioner for an orthopaedic opinion. In this case, no history of trauma could be established. The lesion which measured 2 x 1.5 cm was excised under general anaesthesia. The cut surface showed firm light grey tissue. The patient was reviewed 5 months postoperatively with no evidence of recurrence.
CASE REPORTS Case 1 A 61-year-old woman with a IO-month history of a slowly enlarging blue swelling on the plantar aspect of her right hallux, was referred by her general practitioner for a general surgical opinion. There was a history of penetrative trauma 2 months before the patient first became aware of the lesion. At presentation the swelling was a discrete firm bluish nodule 1.5 x 1.5 cm with intact overlying skin. This was excised under local anaesthesia. The patient was reviewed 1 year postoperatively with no evidence of local recurrence.
Case 4 A 35-year-old man, HIV-positive through i.v. drug abuse, had a lo-year history of a swelling on the plantar aspect of his right foot. The lesion was blue in colour and tender and it measured 3 x 1.5 cm. He was referred by the infectious diseases clinic to a general surgeon. The patient was a construction worker and gave a history of several episodes of trauma to both feet. Excision had been attempted 4 years prior to this referral in an accident and emergency department, but the swelling had returned shortly afterwards. The lesion was completely excised under general anaesthesia. Unfortunately, the patient defaulted from follow-up.
Case 2 A 77-year-old woman with a 5-year history of a slowly growing bluish-black lesion on the plantar aspect of her left foot was referred by her general medical practitioner to a dermatology clinic. There was a history of local penetrating injury to the left foot 50 years previously. Radiography showed no evidence of a foreign body. Although she was placed on the waiting list for excision this was never performed due to her poor medical condition. At the time of referral, the lesion measured 2 x 1.5 cm. The patient died 5 years later of a myocardial infarction, and the diagnosis was established at post-mortem examination.
All 4 lesions showed similar histological features. They were located within the dermis and there was no connection to the epidermis (Fig. 1). Mixed tumour of the skin is characterized by a well circumscribed epithelial cell proliferation embedded in a distinctive connective tissue stroma and enclosed by a fibrous pseudocapsule. The tumour is made up of both large and small aggregates of cuboidal and polygonal epithelial cells, with cellular nests forming tubular lumina, and single epithelial cells dispersed
Case 3 An 82-year-old woman with an 8-month history of a swelling on the plantar aspect of her right foot was 113
114
The Foot
Fig. 1-A multilobulated magnification x 25.
chondroid
syringoma
located
within the dermis.
throughout a myxoid stroma. The stroma frequently shows oedematous and chondroid areas. The cells do not show significant nuclear pleomorphism and mitotic figures are rare. The 4 tumours contained readily identifiable hyaline cells. Hyaline cells have plasmacytoid features with eccentric nuclei and striking cytoplasmic eosinophilia (Fig. 2). They tend to be arranged in poorly cohesive sheets that lack any particular orientation. The presence of considerable amounts of haemosiderin in relation to thinwalled blood vessels indicates previous haemorrhage into stroma.
DISCUSSION
Chondroid syringoma or mixed tumour of skin is an uncommon neoplasm that has many structural resemblances to the mixed tumour of salivary glands1 The great majority of cases involve the skin of the head and neck1 and they are rarely located on the limbs and trunk. They are benign tumours and do not recur if surgical excision is complete. A true malignant cutaneous chondroid syringoma with metastases is extremely rare.’ We could find only 6 cases of chondroid syringoma affecting the skin of the feet described previously in the world literature.‘s3 This report describes the clinicopathological features of a
The overlying
epidermis
is normal.
H and E stain. Original
further 4 cases diagnosed in Edinburgh between 1984 and 1992. Clinically, chondroid syringoma usually appears as a solitary, non-ulcerated firm subcutaneous nodule, which may (as in 3 of these cases) be darker in colour than surrounding soft tissue. All of these cases occurred on the plantar aspect of the foot. This finding is consistent with the report that this is the most common site for soft tissue neoplasia of the foot and that 50% of all soft tissue swellings here are benign or malignant tumours.4 3 of our cases were originally seen by their general practitioner, but clinicians from several specialities were involved in their assessment. The preoperative clinical diagnoses were: implantation dermoid cyst (case 1); subcutaneous ganglion (cases 2 and 4); and no provisional diagnosis was proferred for case 3. It is noteworthy that the surgeon who operated on the HIV-positive patient raised the possibility of Kaposi’s sarcoma, due to the vascular appearance of the nodule. Cutaneous chondroid syringoma also remains an intriguing lesion for pathologists. The most useful diagnostic features are low power circumscription and the biphasic nature of the tumour, with nests of regular cuboidal epithelial cells surrounded by abundant myxoid matrix. The stroma is reactive with Alcian Blue and metachromasia appears with
Chondroid
Fig. 2- -Typical nests of hyaline magnificalion x 400.
cells. Note their plasmacytoid
character
Toluidine Blue, indicating that it is rich in mucopolysaccharides. The hyaline or plasmacytoid cell is an indicator of myoepithelial differentiation and is a valuable diagnostic feature of mixed cell tumours, both of skin and of salivary glands5 Electron microscopy of hyaline cells shows that the entire cytoplasm is filled with a myofibrillar meshwork, related to myoepithelial differentiation.5 An important differential diagnosis, both for the clinician and the pathologist, is that of malignant melanoma. A fine needle aspirate of a chondroid syringoma may contain cytologically fairly uniform cells with eosinophilic cytoplasm and background pigmentation and thus present a challenging diagnosis. Histologically, the lobulation, normal epidermis, lack of significant nuclear pleomorphism and prominent nucleoli, together with a low mitotic rate, would all militate against a diagnosis of malignant melanoma. Immunohistochemistry may aid the diagnostic process but it must be interpreted with caution, because the SlOO antigen expressed in malignant melanoma is also positive in chondroid syringoma (reflecting myoepithelial differentiation). However, the melanoma specific antigen HMB 45 is not expressed in cutaneous chondroid syringoma. Local recurrences of chondroid syringoma are attributed to incomplete excision, which may easily
(arrow)
and tendency
syringoma
to lack cohesion.
of the foot
115
H and F. stain. Original
occur because of lobulation of the tumour. Trauma prior to the development of cutaneous mixed tumours is considered to be rare.i The role of trauma in tumourigenesis is difficult to evaluate and must remain tenuous, although prior injury was documented in 3 of our cases. In conclusion, we have described 4 cases of benign chondroid syringoma occurring on the plantar aspect of the foot. All the cases were diagnosed in Edinburgh over a 9-year period. This cluster of cases would question the apparent extreme rarity of this tumour in this anatomical location, as suggested in the literature, and we speculate that this may be due to the variety of clinical specialities involved in their management, as these cases highlight. Acknowledgements We should like to thank Mr D. Lee. Mr J. M. T. Griffiths Mr C. Court-Brown for permission to present their patients.
References 1. Hirsch P, Helwig E. Chondroid
syringoma: mixed tumours of skin, salivary gland type. Arch Dermatol 1961; 84: 835-846. 2. Matz I R, McCully D J. Stokes B A R. Metastasisng chondroid syringoma: case report. Pathology 1969: 1: 77781.
and
116 The Foot 3. Mambo N. Hyaline cells in a benign chondroid syringoma. Am J Dermatopathol 1984; 6: 265-272. 4. Craigen M A C, El Gawad M A, Anderson E G. Soft tissue swelling of the foot. The Foot 1991; 1: 113-116. 5. Lomax-Smith J D, Azzopardi J G. The hyaline cell: a distinctive feature of ‘mixed’ salivary tumours. Histopathology 1978; 2: 77-92.
The authors P. J. Anderson FDS, FRCS Department of Surgery Eastern General Hospital Edinburgh UK
M. A. RahiUy MRCPath Department of Pathology University of Edinburgh Edinburgh UK Correspondence to P. J. Anderson, Department of Plastic Surgery, Mount Vernon Hospital, Northwood, Middlesex, UK.
113-116
0 1994 Longman Group Ltd
Chondroid syringoma of the foot P. J. Anderson, M. A. Rahilly Department of Surgery, Eastern General Hospital, Edinburgh, UK and Department of Pathology, University of Edinburgh, Edinburgh, UK S UMMA R Y. Chondroid syringoma or mixed tumour of the skin is a relatively uncommon neoplasm thought to originate in sweat glands.’ They are usually found in the head and neck or trunk and have rarely been reported arising on the foot. 4 cases are presented which occurred on the plantar aspect of the foot. There was a history of trauma in 3 of the cases, and 1 recurred following excision. The number of specialities involved in the care of this group of patients is also highlighted. We can find only 6 previous cases of chondroid syringoma arising on the foot described in the world literature. We report on the clinicopathological features of a further 4 cases diagnosed in Edinburgh over a 9-year period.
referred by her general practitioner for an orthopaedic opinion. In this case, no history of trauma could be established. The lesion which measured 2 x 1.5 cm was excised under general anaesthesia. The cut surface showed firm light grey tissue. The patient was reviewed 5 months postoperatively with no evidence of recurrence.
CASE REPORTS Case 1 A 61-year-old woman with a IO-month history of a slowly enlarging blue swelling on the plantar aspect of her right hallux, was referred by her general practitioner for a general surgical opinion. There was a history of penetrative trauma 2 months before the patient first became aware of the lesion. At presentation the swelling was a discrete firm bluish nodule 1.5 x 1.5 cm with intact overlying skin. This was excised under local anaesthesia. The patient was reviewed 1 year postoperatively with no evidence of local recurrence.
Case 4 A 35-year-old man, HIV-positive through i.v. drug abuse, had a lo-year history of a swelling on the plantar aspect of his right foot. The lesion was blue in colour and tender and it measured 3 x 1.5 cm. He was referred by the infectious diseases clinic to a general surgeon. The patient was a construction worker and gave a history of several episodes of trauma to both feet. Excision had been attempted 4 years prior to this referral in an accident and emergency department, but the swelling had returned shortly afterwards. The lesion was completely excised under general anaesthesia. Unfortunately, the patient defaulted from follow-up.
Case 2 A 77-year-old woman with a 5-year history of a slowly growing bluish-black lesion on the plantar aspect of her left foot was referred by her general medical practitioner to a dermatology clinic. There was a history of local penetrating injury to the left foot 50 years previously. Radiography showed no evidence of a foreign body. Although she was placed on the waiting list for excision this was never performed due to her poor medical condition. At the time of referral, the lesion measured 2 x 1.5 cm. The patient died 5 years later of a myocardial infarction, and the diagnosis was established at post-mortem examination.
All 4 lesions showed similar histological features. They were located within the dermis and there was no connection to the epidermis (Fig. 1). Mixed tumour of the skin is characterized by a well circumscribed epithelial cell proliferation embedded in a distinctive connective tissue stroma and enclosed by a fibrous pseudocapsule. The tumour is made up of both large and small aggregates of cuboidal and polygonal epithelial cells, with cellular nests forming tubular lumina, and single epithelial cells dispersed
Case 3 An 82-year-old woman with an 8-month history of a swelling on the plantar aspect of her right foot was 113
114
The Foot
Fig. 1-A multilobulated magnification x 25.
chondroid
syringoma
located
within the dermis.
throughout a myxoid stroma. The stroma frequently shows oedematous and chondroid areas. The cells do not show significant nuclear pleomorphism and mitotic figures are rare. The 4 tumours contained readily identifiable hyaline cells. Hyaline cells have plasmacytoid features with eccentric nuclei and striking cytoplasmic eosinophilia (Fig. 2). They tend to be arranged in poorly cohesive sheets that lack any particular orientation. The presence of considerable amounts of haemosiderin in relation to thinwalled blood vessels indicates previous haemorrhage into stroma.
DISCUSSION
Chondroid syringoma or mixed tumour of skin is an uncommon neoplasm that has many structural resemblances to the mixed tumour of salivary glands1 The great majority of cases involve the skin of the head and neck1 and they are rarely located on the limbs and trunk. They are benign tumours and do not recur if surgical excision is complete. A true malignant cutaneous chondroid syringoma with metastases is extremely rare.’ We could find only 6 cases of chondroid syringoma affecting the skin of the feet described previously in the world literature.‘s3 This report describes the clinicopathological features of a
The overlying
epidermis
is normal.
H and E stain. Original
further 4 cases diagnosed in Edinburgh between 1984 and 1992. Clinically, chondroid syringoma usually appears as a solitary, non-ulcerated firm subcutaneous nodule, which may (as in 3 of these cases) be darker in colour than surrounding soft tissue. All of these cases occurred on the plantar aspect of the foot. This finding is consistent with the report that this is the most common site for soft tissue neoplasia of the foot and that 50% of all soft tissue swellings here are benign or malignant tumours.4 3 of our cases were originally seen by their general practitioner, but clinicians from several specialities were involved in their assessment. The preoperative clinical diagnoses were: implantation dermoid cyst (case 1); subcutaneous ganglion (cases 2 and 4); and no provisional diagnosis was proferred for case 3. It is noteworthy that the surgeon who operated on the HIV-positive patient raised the possibility of Kaposi’s sarcoma, due to the vascular appearance of the nodule. Cutaneous chondroid syringoma also remains an intriguing lesion for pathologists. The most useful diagnostic features are low power circumscription and the biphasic nature of the tumour, with nests of regular cuboidal epithelial cells surrounded by abundant myxoid matrix. The stroma is reactive with Alcian Blue and metachromasia appears with
Chondroid
Fig. 2- -Typical nests of hyaline magnificalion x 400.
cells. Note their plasmacytoid
character
Toluidine Blue, indicating that it is rich in mucopolysaccharides. The hyaline or plasmacytoid cell is an indicator of myoepithelial differentiation and is a valuable diagnostic feature of mixed cell tumours, both of skin and of salivary glands5 Electron microscopy of hyaline cells shows that the entire cytoplasm is filled with a myofibrillar meshwork, related to myoepithelial differentiation.5 An important differential diagnosis, both for the clinician and the pathologist, is that of malignant melanoma. A fine needle aspirate of a chondroid syringoma may contain cytologically fairly uniform cells with eosinophilic cytoplasm and background pigmentation and thus present a challenging diagnosis. Histologically, the lobulation, normal epidermis, lack of significant nuclear pleomorphism and prominent nucleoli, together with a low mitotic rate, would all militate against a diagnosis of malignant melanoma. Immunohistochemistry may aid the diagnostic process but it must be interpreted with caution, because the SlOO antigen expressed in malignant melanoma is also positive in chondroid syringoma (reflecting myoepithelial differentiation). However, the melanoma specific antigen HMB 45 is not expressed in cutaneous chondroid syringoma. Local recurrences of chondroid syringoma are attributed to incomplete excision, which may easily
(arrow)
and tendency
syringoma
to lack cohesion.
of the foot
115
H and F. stain. Original
occur because of lobulation of the tumour. Trauma prior to the development of cutaneous mixed tumours is considered to be rare.i The role of trauma in tumourigenesis is difficult to evaluate and must remain tenuous, although prior injury was documented in 3 of our cases. In conclusion, we have described 4 cases of benign chondroid syringoma occurring on the plantar aspect of the foot. All the cases were diagnosed in Edinburgh over a 9-year period. This cluster of cases would question the apparent extreme rarity of this tumour in this anatomical location, as suggested in the literature, and we speculate that this may be due to the variety of clinical specialities involved in their management, as these cases highlight. Acknowledgements We should like to thank Mr D. Lee. Mr J. M. T. Griffiths Mr C. Court-Brown for permission to present their patients.
References 1. Hirsch P, Helwig E. Chondroid
syringoma: mixed tumours of skin, salivary gland type. Arch Dermatol 1961; 84: 835-846. 2. Matz I R, McCully D J. Stokes B A R. Metastasisng chondroid syringoma: case report. Pathology 1969: 1: 77781.
and
116 The Foot 3. Mambo N. Hyaline cells in a benign chondroid syringoma. Am J Dermatopathol 1984; 6: 265-272. 4. Craigen M A C, El Gawad M A, Anderson E G. Soft tissue swelling of the foot. The Foot 1991; 1: 113-116. 5. Lomax-Smith J D, Azzopardi J G. The hyaline cell: a distinctive feature of ‘mixed’ salivary tumours. Histopathology 1978; 2: 77-92.
The authors P. J. Anderson FDS, FRCS Department of Surgery Eastern General Hospital Edinburgh UK
M. A. RahiUy MRCPath Department of Pathology University of Edinburgh Edinburgh UK Correspondence to P. J. Anderson, Department of Plastic Surgery, Mount Vernon Hospital, Northwood, Middlesex, UK.