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Familial dermal cylindroma with involvement of the parotid gland
0007.1?26iX2/0308-0167$02.00
Brirlsh JournulqfPlas/ic Surgrr.v (1982)35, 167-170 c" 19X2The Trustees of British Association of Plastic Surgeons
Familial dermal cylindroma parotid gland
with involvement
of the
M. H. ALAWI, J. A. E. HOBBY and M. LESNA Wessex Centre for Plastic and Reconstructive Surgery, Odstock Hospital, Salisbury and the Department of Pathology, Royal Victoria Hospital, Bournemouth
Summary-The association of turban tumour with other dermal cylindromas in the parotid gland is very rare. Another case is reported in which the hereditary nature of the disease is discussed and certain aspects of the treatment and management of these cases are considered.
Multiple dermal cylindroma (turban tumour) is a familial autosomal dominant inherited disorder with variable penetration. The coexistence of dermal cylindromas with a similar tumour in the parotid gland is very rare (Reingold et al., 1977). Headington et al. (1977) considered the parotid neoplasm to be a form of basal cell adenoma or membranous basal cell adenoma. We wish to report in a male patient, the association of a large “turban turnour”, multiple dermal cylindromas developing over several decades and two types of benign parotid gland The mode of inheritance, neoplasm. histopathological features and aspects of treatment are considered.
Case report The patient, a ship’s stoker, born in 1912, first presented with a scalp lesion at the age of 27 years. Excision biopsy at that time showed the lesion to be a dermal cylindroma. In 1972, at the age of 60, further scalp lesions had appeared (Fig. 1). These were treated by full-thickness excision of the scalp and repair with split-thickness skin grafts to the pericranium. Subsequently cylindromas appeared in the skin of the neck, chest wall, external auditory meatus (Fig. 2) and the abdominal wall. In February 1980 he presented with multiple swellings in the left parotid gland (Fig. 3). There was no evidence of any facial nerve involvement. All the other salivary glands were normal on clinical examination. At operation total left parotidectomy servation of the facial nerve was performed.
with
pre-
Pathological findings The parotid gland included multiple nodules of tumour varying in size from microadenomatous foci forming in relation to the ducts (Fig. 4) to
Fig. 1 Multiple cylindromas
on the scalp and the face.
large masses 25mm in diameter. The lesions were not encapsulated but were clearly demarcated by thickened hyaline basement membrane. There were abundant intercellular hyaline deposits and the overall pattern of these tumours was indistinguishable from dermal cylindromas (Fig. 5). Many intercalated ducts showed reserve cell hyperplasia and 167
168
Fig. 2 meatus.
BRITISH
A
cylindroma
situated
in
the
Fig. 4 Parotid gland. Microadenomatous adenoma (cylindroma) adjacent to an pattern of the Normal salivary (Haematoxylin and eosin).
external
auditory
focus of basal cell intercalated duct. gland elsewhere.
Fig. 3 lobe.
A swelling
JOURNAL
OF PLASTIC
in the left parotid
SURGERY
gland just below the ear
changes in the ducts (left) Fig. 5 Parotid gland. Hyperplastic and prominent hyaline deposits among the small dark cells of the cylindroma. (Haematoxyhn and eosin).
FAMILIAL
DERMAL
CYLINDROMA
WITH
1NVOLVEMENT
oncocytic changes. One of the larger nodules in the parotid gland was a pleomorphic adenoma of the salivary gland closely adjacent but separate from a large membranous adenoma (Fig. 6). Following this operation there have been no recurrent tumours and the patient is well one year following parotidectomy. In an investigation of the hereditary pattern of involvement in our patient we discovered that several other members of the family had similar cylindromas on the scalp (Fig. 7).
Fig. 6 Parotid gland, Basal cell adenoma (cylindroma) with typical hyahne membranes delineating nodules of basaloid cells (upper half) and separated by a capsule and adipose tissue septum from a pleomorphic adenoma (lower half). (Haematoxylin and eosin).
FAMILY
TREE
PATIENT’S
CHILDREN WITH
ON SCALP
Fig. 7 The patient’s family tree. Those who presented tumours on the scalp are marked as positive (+ve).
GLAND
169
Discussion The simultaneous occurrence of analogous adenomas of sweat glands and the parotid gland was first recorded by Headington ef ul. (1977). The origin of basal cell adenomas of the parotid from intercalated ducts has been postulated by Eversole (1971). Crain and Helwig (1961) believe that dermal cylindromas may also arise from the ducts, the oncocytic changes and the microadenomatous foci around the ducts support this theory. It is presumed that both these lesions have an eccrine duct origin and it seems possible that a single gene abnormality acting on ontogenetically similar stem cells may be responsible for the presence of tumours (hamartomas) in both the skin and the parotid skin the intercalated ducts and the eccrine ducts of skin belong to the same target organ. We would like to emphasise that the lesion described in this study must be clearly distinguished from adenoid carcinoma of the salivary glands and that despite their multifocality the parotid lesions described in our case did not show any evidence of invasive behaviour. The patient has been developing new dermal cylindromas over several decades, which is in keeping with observations in the published reports of inherited cylindromas. The first crop of lesions usually appear around the age of 25 years. A similar time scale was noted in the lesions affecting two of our patient’s sisters and his son. Relatives of patients with this abnormality may require genetic counselling. We are not aware that a specific chromosomal abnormality has been demonstrated in this condition. The carrier of the abnormal gene may not manifest the disease, with the result that the disease may skip a generation.
R. C. and Helwig, E. B. (1961). Dermal cyhndroma (dermal eccrine cyhndromd). American Journal q/ Clinical Pathology. 35, 504. Ever&e, L. R. (1971). Histogenic classification of salivary tumours. Archives of Pathology, 92, 433. Crain,
TUMOURS
PAROTID
References
NIECES & NEPHEW
+ve = PATIENT
OF THE
with
170 Headington, J. T., Batsakis, J. G., Beals, T. F., Campbell, T. E., Simmons, J. L. and Stone, W. D. (1977). Membranous basal cell adenoma of parotid gland, dermal cylindromas and trichoepitheliomas. Comparative histochemistry and ultra-structure. Cancer, 39, 2460. Reingold, I. M., Keashey, L. E. and Graham, J. H. (1977). Multicentric dermal-type cylindromas of the parotid glands in a patient with florid turban tumour. Cancer, 40, 1702.
BRITISH JOURNAL
OF PLASTIC SURGERY
The Authors M. G. Alawi, FRCSEQ, FRCSEng, Senior House Officer in Plastic Surgery. J. A. E. Hobby, BSc, FRCS, Consultant Plastic Surgeon, Wessex Centre for Plastic and Reconstructive Surgery, Odstock Hospital, Salisbury, Wiltshire. Milena Lesna, MD, LMSSA, MRC, Path, Consultant Histopathologist, Royal Victoria Hospital, Bournemouth, Dorset. Requests for reprints to: J. A. E. Hobby, BSc, FRCS, Consultant Plastic Surgeon, Wessex Centre for Plastic and Reconstructive Surgery, Odstock Hospital, Salisbury, Wiltshire.
Brirlsh JournulqfPlas/ic Surgrr.v (1982)35, 167-170 c" 19X2The Trustees of British Association of Plastic Surgeons
Familial dermal cylindroma parotid gland
with involvement
of the
M. H. ALAWI, J. A. E. HOBBY and M. LESNA Wessex Centre for Plastic and Reconstructive Surgery, Odstock Hospital, Salisbury and the Department of Pathology, Royal Victoria Hospital, Bournemouth
Summary-The association of turban tumour with other dermal cylindromas in the parotid gland is very rare. Another case is reported in which the hereditary nature of the disease is discussed and certain aspects of the treatment and management of these cases are considered.
Multiple dermal cylindroma (turban tumour) is a familial autosomal dominant inherited disorder with variable penetration. The coexistence of dermal cylindromas with a similar tumour in the parotid gland is very rare (Reingold et al., 1977). Headington et al. (1977) considered the parotid neoplasm to be a form of basal cell adenoma or membranous basal cell adenoma. We wish to report in a male patient, the association of a large “turban turnour”, multiple dermal cylindromas developing over several decades and two types of benign parotid gland The mode of inheritance, neoplasm. histopathological features and aspects of treatment are considered.
Case report The patient, a ship’s stoker, born in 1912, first presented with a scalp lesion at the age of 27 years. Excision biopsy at that time showed the lesion to be a dermal cylindroma. In 1972, at the age of 60, further scalp lesions had appeared (Fig. 1). These were treated by full-thickness excision of the scalp and repair with split-thickness skin grafts to the pericranium. Subsequently cylindromas appeared in the skin of the neck, chest wall, external auditory meatus (Fig. 2) and the abdominal wall. In February 1980 he presented with multiple swellings in the left parotid gland (Fig. 3). There was no evidence of any facial nerve involvement. All the other salivary glands were normal on clinical examination. At operation total left parotidectomy servation of the facial nerve was performed.
with
pre-
Pathological findings The parotid gland included multiple nodules of tumour varying in size from microadenomatous foci forming in relation to the ducts (Fig. 4) to
Fig. 1 Multiple cylindromas
on the scalp and the face.
large masses 25mm in diameter. The lesions were not encapsulated but were clearly demarcated by thickened hyaline basement membrane. There were abundant intercellular hyaline deposits and the overall pattern of these tumours was indistinguishable from dermal cylindromas (Fig. 5). Many intercalated ducts showed reserve cell hyperplasia and 167
168
Fig. 2 meatus.
BRITISH
A
cylindroma
situated
in
the
Fig. 4 Parotid gland. Microadenomatous adenoma (cylindroma) adjacent to an pattern of the Normal salivary (Haematoxylin and eosin).
external
auditory
focus of basal cell intercalated duct. gland elsewhere.
Fig. 3 lobe.
A swelling
JOURNAL
OF PLASTIC
in the left parotid
SURGERY
gland just below the ear
changes in the ducts (left) Fig. 5 Parotid gland. Hyperplastic and prominent hyaline deposits among the small dark cells of the cylindroma. (Haematoxyhn and eosin).
FAMILIAL
DERMAL
CYLINDROMA
WITH
1NVOLVEMENT
oncocytic changes. One of the larger nodules in the parotid gland was a pleomorphic adenoma of the salivary gland closely adjacent but separate from a large membranous adenoma (Fig. 6). Following this operation there have been no recurrent tumours and the patient is well one year following parotidectomy. In an investigation of the hereditary pattern of involvement in our patient we discovered that several other members of the family had similar cylindromas on the scalp (Fig. 7).
Fig. 6 Parotid gland, Basal cell adenoma (cylindroma) with typical hyahne membranes delineating nodules of basaloid cells (upper half) and separated by a capsule and adipose tissue septum from a pleomorphic adenoma (lower half). (Haematoxylin and eosin).
FAMILY
TREE
PATIENT’S
CHILDREN WITH
ON SCALP
Fig. 7 The patient’s family tree. Those who presented tumours on the scalp are marked as positive (+ve).
GLAND
169
Discussion The simultaneous occurrence of analogous adenomas of sweat glands and the parotid gland was first recorded by Headington ef ul. (1977). The origin of basal cell adenomas of the parotid from intercalated ducts has been postulated by Eversole (1971). Crain and Helwig (1961) believe that dermal cylindromas may also arise from the ducts, the oncocytic changes and the microadenomatous foci around the ducts support this theory. It is presumed that both these lesions have an eccrine duct origin and it seems possible that a single gene abnormality acting on ontogenetically similar stem cells may be responsible for the presence of tumours (hamartomas) in both the skin and the parotid skin the intercalated ducts and the eccrine ducts of skin belong to the same target organ. We would like to emphasise that the lesion described in this study must be clearly distinguished from adenoid carcinoma of the salivary glands and that despite their multifocality the parotid lesions described in our case did not show any evidence of invasive behaviour. The patient has been developing new dermal cylindromas over several decades, which is in keeping with observations in the published reports of inherited cylindromas. The first crop of lesions usually appear around the age of 25 years. A similar time scale was noted in the lesions affecting two of our patient’s sisters and his son. Relatives of patients with this abnormality may require genetic counselling. We are not aware that a specific chromosomal abnormality has been demonstrated in this condition. The carrier of the abnormal gene may not manifest the disease, with the result that the disease may skip a generation.
R. C. and Helwig, E. B. (1961). Dermal cyhndroma (dermal eccrine cyhndromd). American Journal q/ Clinical Pathology. 35, 504. Ever&e, L. R. (1971). Histogenic classification of salivary tumours. Archives of Pathology, 92, 433. Crain,
TUMOURS
PAROTID
References
NIECES & NEPHEW
+ve = PATIENT
OF THE
with
170 Headington, J. T., Batsakis, J. G., Beals, T. F., Campbell, T. E., Simmons, J. L. and Stone, W. D. (1977). Membranous basal cell adenoma of parotid gland, dermal cylindromas and trichoepitheliomas. Comparative histochemistry and ultra-structure. Cancer, 39, 2460. Reingold, I. M., Keashey, L. E. and Graham, J. H. (1977). Multicentric dermal-type cylindromas of the parotid glands in a patient with florid turban tumour. Cancer, 40, 1702.
BRITISH JOURNAL
OF PLASTIC SURGERY
The Authors M. G. Alawi, FRCSEQ, FRCSEng, Senior House Officer in Plastic Surgery. J. A. E. Hobby, BSc, FRCS, Consultant Plastic Surgeon, Wessex Centre for Plastic and Reconstructive Surgery, Odstock Hospital, Salisbury, Wiltshire. Milena Lesna, MD, LMSSA, MRC, Path, Consultant Histopathologist, Royal Victoria Hospital, Bournemouth, Dorset. Requests for reprints to: J. A. E. Hobby, BSc, FRCS, Consultant Plastic Surgeon, Wessex Centre for Plastic and Reconstructive Surgery, Odstock Hospital, Salisbury, Wiltshire.