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Cutaneous meningioma underlying congenital localized hypertrichosis
Journal of the American Academy of Dermatology Volume 30, Number 2, Part 2
study of two typical cases. Am ] Dermatopathol 1989; 11:112-23. 23.
Delsol G, Saati AL, Gatter KG, et a1. Coexpression ofepithelial membrane antigen (EMA), Ki-I , and interleukin-2 receptor by anaplastic large cell lymphomas: diagnostic value in so-called malignant histiocytosis. Am ] Pathol 1988;130:59-70.
24. Oshima K, Kikuchi M, Masuda Y, et a1. Genotypic and
Pehas et al.
immunophenotypic analysis of anaplastic large cell lymphoma (Ki-l lymphoma).Pathol ResPract 1990;186:582-8 . 25. Kadin ME. Ki-t-positive anaplastic large-cell lymphoma:a clinicopathologic entity? J Clin Oneal 1991;9:533-6. 26. Nakamura S, Takagi N, Kojima M, et a1. Clinicopathologicstudy oflarge cell anaplastic lymphoma (Ki-l-positive large cell lymphoma) among the Japanese. Cancer 1991; 68:118-29.
Cutaneous meningioma underlying congenital localized hypertrichosis Pablo F. Pefias, MD,a Maria Jones-Caballero, MD,a Ana Amigo, MD,b Maximiliano Aragiies, MD,a and Amaro Garcia-Diez, MD a Madrid, Spain A 28-year-old man had a circumscribed hypertrichoticarea on the left lumbar region since birth. A biopsy specimen showed numerous normal-appearing hair follicles and arrector pili muscles. In the subcutaneouslayer a massof densecollagenous connective tissue formed the backgroundin which ill-defined fociof meningothelial cellsin nestsand pseudovascular spaces were seen. Collagenous structures (pseudopsammoma bodies). some of which showedcalcification (psammoma bodies), were also seen. A diagnosis of cutaneous meningioma was made. (J AM ACAD DERMATOL 1994;30:363-6.) Meningiomas are among the most common primary tumors of the central nervous system (eNS). They can occur rarely in an extracranial site; one of the more frequent sites is the skin or subcutaneous tissue. These tumors have been called cutaneous meningiomas and have been classified into three categories by Lopez et al. 1: type I, primary cutaneous meningioma; type II , soft tissue and skin meningiomas; and type III, eNS meningiomas with extension into the skin . The first type is a congenital, nonneoplastic lesion, whereas the others are acquired, neoplastic proliferations of meningothelial cells. 1-3 We describe a patient with a congenital, circumscribed hypertrichotic area overlying a type I cutaneous meningioma. From the Departments ofDermatology' and Pathology," Hospital de la Princesa, Universidad Autonoma. Presented at the Twenty-first Congress of the Spanish Academy of Dermatology and Venereology, La Corufia, Spain, May 30, 1992. Reprint requests: Pablo F. Pefias, MD, Secci6n de Dermatologia, Hospital de la Princesa, Diego de Leon 62, 28006 Madrid, Spain. Copyright ® 1994 by the American Academy ofDermatology, Inc, 0190-9622/94/$3.00 + 0 16/4/48042
CASE REPORT
A 28-year-old man had a 6 X 3 em circumscribed hypertrichotic area in the left lumbar region, 10 cm away from the midline (Fig. 1). A 2 X 1 em, subcutaneous, nontender, firm nodule was detected beneath that area. This lesion had been present since birth and had slowly enlarged with the growth of the rest of his body. A biopsy specimen showed a normal epidermis and numerous normal-appearing hair follicles and arrector pili muscles. In the subcutaneous tissue there was a mass of densecollagenous connectivetissuein whichill-defined foci of meningothelial cells were found (Fig. 2). These cellswerearranged invarious ways, that is, pseudovascular spaceslined with flattened cells, nests ofpolygonal cells with a syncytial appearance, cells that formed poorlydeveloped whorls, and cells that surrounded rounded collagenous structures (pseudopsammoma bodies),some of which were partially calcified (psammoma bodies) (Fig. 3). No evidence of atypia was found. Tmmunohistochemical studies showedpositivestaining of the meningothelial cells with vimentin and epithelial membrane antigen. They were negative for S-IOO and cytokeratins. Normal structures within the sections provided positive and negative controls. Additional evaluations included a computed tomo-
363
364 Pehas et
at.
Journal of the American Academy of Dermatology February 1994
Table I. Differential diagnosis of congenital localized hypertrichosis
Fig. 1. Circumscribed hypertrichotic area on left lumbar region, to em from the midline.
Becker's nevus Congenital nevocellular nevus Familial hypertrichosis of the palms and sales Linear epidermal nevus Congenital smooth muscle hamartoma Congenital spinal cord hypertrichosis Meningocele Diastematomyelia Spina bifida Kyphosis Scoliosis Chest deformities Simple nevoid hypertrichosis Type I cutaneous meningioma-cutaneous heterotopic meningeal nodule
Fig. 2. Pseudovascular spaces lined by flattened cells and nest of meningothelial cells with indistinct cytoplasmic borders. Note the presence of collagen bodies. (Hematoxylin-eosin stain; XlOO.)
graphic scan of the skull and lumbar spine that showed no abnormalities. The patient refused further excision of the lesion.
DISCUSSION Type I or primary cutaneous meningiomas occur in the scalp, i-s on the midline and paravertebral regions of the back, 1, 2, 5, 7 and on the forehead, bridge of the nose, and periorbital region. j Clinically they appear as an area of alopecia or as a fibroma, skin tag, nevus, nodule, or cyst,I-3, 5 The diagnosis is usually made only after histopathologic examination of
the specimen. i-s, 5 The lesions usually date from birth and have a good prognosis.lv 5-7 On light microscopic examination they show variable architecture (cellular masses arranged in nests or whorls as in meningiomas, 1 pseudovascular lesions.v" or rudimentary cysts!- 2,5) surrounded by dense/: 5 or myxoid3, 5 stroma. However, similar cytologic features are found: regular polygonal to fusiform cells with syncytial appearance, homogeneous eosinophilic cytoplasm, and regular nuclei; pseudopsammoma and psammoma bodies can both be present. 1-5 The microscopic characteristics, the anatomic distribution,
Journal of the American Academy of Dermatology Volume 30, Number 2, Part 2
Penas et al. 365
Fig. 3. Psammoma body surrounded by meningothe1ial cells. (Hematoxylin-eosin stain; X200.)
Table II. Differential diagnosis of cutaneous meningiomas
Feature
Origin Age Localization
TypeI:
TypeII:
primary cutaneous meningioma
ectopic soft-tissue meningioma
Congenital Children and young adults Scalp, face, neck, and paravertebral areas
Acquired Adults
Acquired Adults
Periorbital, perinasal, or periauricular sites or along cranial and spinal
Usually head (direct extension or metastasis from a eNS meningioma) Yes Neoplastic Meningioma
nerves eNS meningioma Behavior Histologic features
No Benign Meningothelial cells resembling a meningioma
and the nonneoplastic behavior led Sibley and Cooper 2 and Marrogi et al.3 to describe them as "rudimentary meningoceles." Suster and Rosai," in five cases on the scalp, found proliferations of meningothelial elements admixed with adipose tissue, blood vessels, and other connective tissue elements and interpreted them as "hamartomas with ectopic meningothe1ial elements." In 1990, Theaker et al. 5 reported nests of meningothelial cells around small nerves and suggested that these lesionsrepresent the continued growth of meningeal cells within an environment (perineural) that normally supports the growth and development of a closely related cell type, the Schwann cell. They proposed the term cutaneous heterotopic meningeal nodules.
Type ill: CNS meningioma with extension into the skin
No Neoplastic, slow growing Meningioma
With regard to the origin of these meningothelial elements, Lopez et al.' suggested that they probably represent a type of developmental abnormality similar to a meningocele. Meningothelial cells from the arachnoid villous cap get sequestrated or trapped in the skin at the time of closure of the neural crest. Several authors- 3, 6,7 agreed with them, but others S' 8,9 have proposed that cutaneous meningiomas may arise from the sheath cells of cutaneous nerves. Our case was outside the midline, making the meningocele mechanism unlikely. It could best reflect growth of meningeal cells displaced with a cutaneous nerve in the fashion described by Theaker et al. 5 Our case had the histologic and immunohistochemical features of a type I cutaneous meningi-
366
Pehas et al.
oma but had a clinically atypical feature: a congenital localized hypertrichosis outside the midline. Congenital localized hypertrichosis (Table I) occurs in association with other cutaneous abnormalities, 10 but it may occur in apparently normal skin overlying the spinal cord. This finding is usually associated with spinal dysraphisml- 12 such as meningocele, spina bifida, or diastematomyelia or with other vertebral deformities.l'' When the congenital localized hypertrichosis is located away from the spine in apparently normal skin, it has been named simple nevoid hypertrichosis. 13 We believe that some cases of simple nevoid hypertrichosis and congenital smooth muscle hamartoma might conceal deep proliferations of meningothelial cells. The mechanism by which these proliferations stimulate the growth of hair follicles and arrector pili muscles is unknown. Although it has been suggested that abnormal mesodermal cells could explain the hypertrichosis overlying bony (mesodermal) defects, 10 specific neurotrophic growth factors could be implicated, as has been proposed in hypertrophic hair surrounding cranial neuroectodermal abnormalities.!" These lesions should be differentiated from type II and type III cutaneous meningioma (Table II). This can easily be done on the basis of clinical and histologic findings. I, 2 Microscopically, they may be confused with vascular" and other tumors.v 15 but familiarity with the features makes this unlikely. Immunohistochemical studies can be of aid in difficult cases on the basis of positive staining with epithelial membrane antigen and vimentin and negative staining with cytokeratins and Sol 00. 2-5, 15 Our case represents an exception to the usual description of type I cutaneous meningioma or cutaneous heterotopic meningeal nodule because the le-
Journal of the American Academy of Dermatology February 1994
sion clinically appeared as a circumscribed congenital hypertrichosis and was located outside the midline of the body. REFERENCES 1. Lopez DA, Silvers DN, Helwig EE. Cutaneous meningiomas: a clinicopathologic study. Cancer 1974;34:728-44. 2. Sibley DA, Cooper PH. Rudimentary meningocele: a variant of "primary cutaneous meningioma." J Cutan Pathol 1989;16:72-80. 3. Marrogi AI, Swanson PE, Kyriakos M, et al. Rudimentary meningocele of the skin. J Cutan PathoI1991;18:178-88. 4. Suster S, Rosai J. Hamartoma of the scalp with ectopic meningothelia1 elements: a distinctive benign soft tissue lesion that may simulate angiosarcoma. Am J Surg Pathol 1990;14:1-11. 5. Theaker JM, Fletcher CD, Tudway AJ. Cutaneous heterotopicmeningealnodules. Histopathology 1990;16:475-9. 6. Nochomovitz LE, Jannotta F, Orenstein JM. Meningioma of the scalp: light and electron microscopic observations. Arch Pathol Lab Med 1985;109:92-5. 7. Zaaroor M, Borovich B, Bassan L, et al. Primary cutaneous extravertebral meningioma: case report. J Neurosurg 1984;60:1097-8. 8. Bain GO, Shnitka TK. Cutaneous meningioma (psamorna): report of a case. Arch DermatoI1956;74:590-4. 9. Daugaard S. Ectopic meningioma of a finger: case report. J Neurosurg 1983;58:778-80. 10. Reed OM, Mellete JR Jr., Fitzpatrick JE. Familial cervical hypertrichosis with underlying kyphoscoliosis. J AM ACAD DERMATOL 1989;20:1069-72. 11. Harris HW, Miller OF. Midline cutaneous and spinal defects. Arch Dermatol1976;1l2;1724-8. 12. Tavafoghi V, Ghandchi A, Hambrick GW, et al. Cutaneous signs of spinal dysraphism. Arch Dermato1 1978; 114:573-7. 13. Bertolino AP, Freedberg 1M. Hair. In: Fitzpatrick TB, Eisen AZ, W olff K, et al., eds. Dermatology in general medicine. New York: McGraw-Hill, 1987:627-66. 14. Commens C, Rogers M, Kan A. Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair. Arch DermatoI1989;125:l253-6. 15. Theaker JM, Fleming KA. Meningioma of the scalp: a case report with immunohistological features. J Cutan Pathol 1987;14:49-53.
study of two typical cases. Am ] Dermatopathol 1989; 11:112-23. 23.
Delsol G, Saati AL, Gatter KG, et a1. Coexpression ofepithelial membrane antigen (EMA), Ki-I , and interleukin-2 receptor by anaplastic large cell lymphomas: diagnostic value in so-called malignant histiocytosis. Am ] Pathol 1988;130:59-70.
24. Oshima K, Kikuchi M, Masuda Y, et a1. Genotypic and
Pehas et al.
immunophenotypic analysis of anaplastic large cell lymphoma (Ki-l lymphoma).Pathol ResPract 1990;186:582-8 . 25. Kadin ME. Ki-t-positive anaplastic large-cell lymphoma:a clinicopathologic entity? J Clin Oneal 1991;9:533-6. 26. Nakamura S, Takagi N, Kojima M, et a1. Clinicopathologicstudy oflarge cell anaplastic lymphoma (Ki-l-positive large cell lymphoma) among the Japanese. Cancer 1991; 68:118-29.
Cutaneous meningioma underlying congenital localized hypertrichosis Pablo F. Pefias, MD,a Maria Jones-Caballero, MD,a Ana Amigo, MD,b Maximiliano Aragiies, MD,a and Amaro Garcia-Diez, MD a Madrid, Spain A 28-year-old man had a circumscribed hypertrichoticarea on the left lumbar region since birth. A biopsy specimen showed numerous normal-appearing hair follicles and arrector pili muscles. In the subcutaneouslayer a massof densecollagenous connective tissue formed the backgroundin which ill-defined fociof meningothelial cellsin nestsand pseudovascular spaces were seen. Collagenous structures (pseudopsammoma bodies). some of which showedcalcification (psammoma bodies), were also seen. A diagnosis of cutaneous meningioma was made. (J AM ACAD DERMATOL 1994;30:363-6.) Meningiomas are among the most common primary tumors of the central nervous system (eNS). They can occur rarely in an extracranial site; one of the more frequent sites is the skin or subcutaneous tissue. These tumors have been called cutaneous meningiomas and have been classified into three categories by Lopez et al. 1: type I, primary cutaneous meningioma; type II , soft tissue and skin meningiomas; and type III, eNS meningiomas with extension into the skin . The first type is a congenital, nonneoplastic lesion, whereas the others are acquired, neoplastic proliferations of meningothelial cells. 1-3 We describe a patient with a congenital, circumscribed hypertrichotic area overlying a type I cutaneous meningioma. From the Departments ofDermatology' and Pathology," Hospital de la Princesa, Universidad Autonoma. Presented at the Twenty-first Congress of the Spanish Academy of Dermatology and Venereology, La Corufia, Spain, May 30, 1992. Reprint requests: Pablo F. Pefias, MD, Secci6n de Dermatologia, Hospital de la Princesa, Diego de Leon 62, 28006 Madrid, Spain. Copyright ® 1994 by the American Academy ofDermatology, Inc, 0190-9622/94/$3.00 + 0 16/4/48042
CASE REPORT
A 28-year-old man had a 6 X 3 em circumscribed hypertrichotic area in the left lumbar region, 10 cm away from the midline (Fig. 1). A 2 X 1 em, subcutaneous, nontender, firm nodule was detected beneath that area. This lesion had been present since birth and had slowly enlarged with the growth of the rest of his body. A biopsy specimen showed a normal epidermis and numerous normal-appearing hair follicles and arrector pili muscles. In the subcutaneous tissue there was a mass of densecollagenous connectivetissuein whichill-defined foci of meningothelial cells were found (Fig. 2). These cellswerearranged invarious ways, that is, pseudovascular spaceslined with flattened cells, nests ofpolygonal cells with a syncytial appearance, cells that formed poorlydeveloped whorls, and cells that surrounded rounded collagenous structures (pseudopsammoma bodies),some of which were partially calcified (psammoma bodies) (Fig. 3). No evidence of atypia was found. Tmmunohistochemical studies showedpositivestaining of the meningothelial cells with vimentin and epithelial membrane antigen. They were negative for S-IOO and cytokeratins. Normal structures within the sections provided positive and negative controls. Additional evaluations included a computed tomo-
363
364 Pehas et
at.
Journal of the American Academy of Dermatology February 1994
Table I. Differential diagnosis of congenital localized hypertrichosis
Fig. 1. Circumscribed hypertrichotic area on left lumbar region, to em from the midline.
Becker's nevus Congenital nevocellular nevus Familial hypertrichosis of the palms and sales Linear epidermal nevus Congenital smooth muscle hamartoma Congenital spinal cord hypertrichosis Meningocele Diastematomyelia Spina bifida Kyphosis Scoliosis Chest deformities Simple nevoid hypertrichosis Type I cutaneous meningioma-cutaneous heterotopic meningeal nodule
Fig. 2. Pseudovascular spaces lined by flattened cells and nest of meningothelial cells with indistinct cytoplasmic borders. Note the presence of collagen bodies. (Hematoxylin-eosin stain; XlOO.)
graphic scan of the skull and lumbar spine that showed no abnormalities. The patient refused further excision of the lesion.
DISCUSSION Type I or primary cutaneous meningiomas occur in the scalp, i-s on the midline and paravertebral regions of the back, 1, 2, 5, 7 and on the forehead, bridge of the nose, and periorbital region. j Clinically they appear as an area of alopecia or as a fibroma, skin tag, nevus, nodule, or cyst,I-3, 5 The diagnosis is usually made only after histopathologic examination of
the specimen. i-s, 5 The lesions usually date from birth and have a good prognosis.lv 5-7 On light microscopic examination they show variable architecture (cellular masses arranged in nests or whorls as in meningiomas, 1 pseudovascular lesions.v" or rudimentary cysts!- 2,5) surrounded by dense/: 5 or myxoid3, 5 stroma. However, similar cytologic features are found: regular polygonal to fusiform cells with syncytial appearance, homogeneous eosinophilic cytoplasm, and regular nuclei; pseudopsammoma and psammoma bodies can both be present. 1-5 The microscopic characteristics, the anatomic distribution,
Journal of the American Academy of Dermatology Volume 30, Number 2, Part 2
Penas et al. 365
Fig. 3. Psammoma body surrounded by meningothe1ial cells. (Hematoxylin-eosin stain; X200.)
Table II. Differential diagnosis of cutaneous meningiomas
Feature
Origin Age Localization
TypeI:
TypeII:
primary cutaneous meningioma
ectopic soft-tissue meningioma
Congenital Children and young adults Scalp, face, neck, and paravertebral areas
Acquired Adults
Acquired Adults
Periorbital, perinasal, or periauricular sites or along cranial and spinal
Usually head (direct extension or metastasis from a eNS meningioma) Yes Neoplastic Meningioma
nerves eNS meningioma Behavior Histologic features
No Benign Meningothelial cells resembling a meningioma
and the nonneoplastic behavior led Sibley and Cooper 2 and Marrogi et al.3 to describe them as "rudimentary meningoceles." Suster and Rosai," in five cases on the scalp, found proliferations of meningothelial elements admixed with adipose tissue, blood vessels, and other connective tissue elements and interpreted them as "hamartomas with ectopic meningothe1ial elements." In 1990, Theaker et al. 5 reported nests of meningothelial cells around small nerves and suggested that these lesionsrepresent the continued growth of meningeal cells within an environment (perineural) that normally supports the growth and development of a closely related cell type, the Schwann cell. They proposed the term cutaneous heterotopic meningeal nodules.
Type ill: CNS meningioma with extension into the skin
No Neoplastic, slow growing Meningioma
With regard to the origin of these meningothelial elements, Lopez et al.' suggested that they probably represent a type of developmental abnormality similar to a meningocele. Meningothelial cells from the arachnoid villous cap get sequestrated or trapped in the skin at the time of closure of the neural crest. Several authors- 3, 6,7 agreed with them, but others S' 8,9 have proposed that cutaneous meningiomas may arise from the sheath cells of cutaneous nerves. Our case was outside the midline, making the meningocele mechanism unlikely. It could best reflect growth of meningeal cells displaced with a cutaneous nerve in the fashion described by Theaker et al. 5 Our case had the histologic and immunohistochemical features of a type I cutaneous meningi-
366
Pehas et al.
oma but had a clinically atypical feature: a congenital localized hypertrichosis outside the midline. Congenital localized hypertrichosis (Table I) occurs in association with other cutaneous abnormalities, 10 but it may occur in apparently normal skin overlying the spinal cord. This finding is usually associated with spinal dysraphisml- 12 such as meningocele, spina bifida, or diastematomyelia or with other vertebral deformities.l'' When the congenital localized hypertrichosis is located away from the spine in apparently normal skin, it has been named simple nevoid hypertrichosis. 13 We believe that some cases of simple nevoid hypertrichosis and congenital smooth muscle hamartoma might conceal deep proliferations of meningothelial cells. The mechanism by which these proliferations stimulate the growth of hair follicles and arrector pili muscles is unknown. Although it has been suggested that abnormal mesodermal cells could explain the hypertrichosis overlying bony (mesodermal) defects, 10 specific neurotrophic growth factors could be implicated, as has been proposed in hypertrophic hair surrounding cranial neuroectodermal abnormalities.!" These lesions should be differentiated from type II and type III cutaneous meningioma (Table II). This can easily be done on the basis of clinical and histologic findings. I, 2 Microscopically, they may be confused with vascular" and other tumors.v 15 but familiarity with the features makes this unlikely. Immunohistochemical studies can be of aid in difficult cases on the basis of positive staining with epithelial membrane antigen and vimentin and negative staining with cytokeratins and Sol 00. 2-5, 15 Our case represents an exception to the usual description of type I cutaneous meningioma or cutaneous heterotopic meningeal nodule because the le-
Journal of the American Academy of Dermatology February 1994
sion clinically appeared as a circumscribed congenital hypertrichosis and was located outside the midline of the body. REFERENCES 1. Lopez DA, Silvers DN, Helwig EE. Cutaneous meningiomas: a clinicopathologic study. Cancer 1974;34:728-44. 2. Sibley DA, Cooper PH. Rudimentary meningocele: a variant of "primary cutaneous meningioma." J Cutan Pathol 1989;16:72-80. 3. Marrogi AI, Swanson PE, Kyriakos M, et al. Rudimentary meningocele of the skin. J Cutan PathoI1991;18:178-88. 4. Suster S, Rosai J. Hamartoma of the scalp with ectopic meningothelia1 elements: a distinctive benign soft tissue lesion that may simulate angiosarcoma. Am J Surg Pathol 1990;14:1-11. 5. Theaker JM, Fletcher CD, Tudway AJ. Cutaneous heterotopicmeningealnodules. Histopathology 1990;16:475-9. 6. Nochomovitz LE, Jannotta F, Orenstein JM. Meningioma of the scalp: light and electron microscopic observations. Arch Pathol Lab Med 1985;109:92-5. 7. Zaaroor M, Borovich B, Bassan L, et al. Primary cutaneous extravertebral meningioma: case report. J Neurosurg 1984;60:1097-8. 8. Bain GO, Shnitka TK. Cutaneous meningioma (psamorna): report of a case. Arch DermatoI1956;74:590-4. 9. Daugaard S. Ectopic meningioma of a finger: case report. J Neurosurg 1983;58:778-80. 10. Reed OM, Mellete JR Jr., Fitzpatrick JE. Familial cervical hypertrichosis with underlying kyphoscoliosis. J AM ACAD DERMATOL 1989;20:1069-72. 11. Harris HW, Miller OF. Midline cutaneous and spinal defects. Arch Dermatol1976;1l2;1724-8. 12. Tavafoghi V, Ghandchi A, Hambrick GW, et al. Cutaneous signs of spinal dysraphism. Arch Dermato1 1978; 114:573-7. 13. Bertolino AP, Freedberg 1M. Hair. In: Fitzpatrick TB, Eisen AZ, W olff K, et al., eds. Dermatology in general medicine. New York: McGraw-Hill, 1987:627-66. 14. Commens C, Rogers M, Kan A. Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair. Arch DermatoI1989;125:l253-6. 15. Theaker JM, Fleming KA. Meningioma of the scalp: a case report with immunohistological features. J Cutan Pathol 1987;14:49-53.