Pediatdc Dermatology Vol. 9 No. 3 272-274
Neurothekeoma in a 15-Year-Old Boy: Case Report Marci Pepine, M.D.,* Franklin Flowers, M.D.,t and Francisco A. Ramos-Caro, M.D.t * Department of Medicine, and '''Division of Dermatology & Cutaneous Surgery, University of Florida College of Medicine, Gainesville. Florida
Abstract: A 15-year-old boy experienced a rapidly enlarging nodule on his left nose. Histologic findings and immunoperoxidase stains were consistent with the diagnosis of neurothekeoma. The patient was treated with local excision without evidence of recurrence.
Neurothekeoma is a term first coined by Gallager and Helwig in 1980 referring to a benign, cutaneous, soft tissue tumor (1). Prior to this it was referred to as a nerve sheath myxoma, as described by Harkin and Reed (2). The literature appears to contain only 18 documented cases of neurothekeoma involving the face (1,3,4) and only 31 cases occurring in patients less than 20 years of age (1,4,5). This uncommon tumor should be included in the differential diagnosis of pediatric soft tissue tumors involving the centra] face. CASE REPORT A 15-year-old black boy was first seen for evaluation of an enlarging mass on the left side of his nose (Fig. I). He first noted the mass several months earlier, when it was the size of a small pimple. Since then it enlarged rapidly. The mass was reported to be only minimally tender but did bleed easily. The patient was an otherwise healthy boy in no apparent distress, with no pertinent medical history. Physical examination revealed a firm, skincolored nodule, 1 x 1 cm, on the left ala nasi with some telangiectasias. A 2-mm punch biopsy specimen demonstrated fibrosis with increased mucin. On a follow-up visit Address correspondence lo Franklin Flowers, M.D., Division of Dermatology, PO Box 100277, Gainesville, FL 326100277.
three months later, even though the mass had not changed in size or character, the patient requested that it be removed. A parallel excision was performed. Gross examination revealed a 1-cm, round, tancolored nodule. Microscopic examination showed aggregates of epithelioid and stellate cells in a myxoid stroma that were separated by dense, fibrous connective tissue consistent with a neurothekeoma (Fig. 2). Immunocytochemistry was negative for S-100 protein and EM A, which is consistent with that diagnosis. The patient had an unremarkable course without recurrence after nine months of observation. DISCUSSION This case was unusual compared with those described by Gallager and Helwig. In their series, the majority of lesions (68.8%) occurred on other parts of the body, namely, the arms and shoulders (1). Furthermore, our patient was male, and neurothekeomasare more common infemales (4.3:1 ratio)(l), with only nine reports of them occurring in males (4,6-12). In addition, to our knowledge no neurothekeomas reported have been reported in African-Americans. Furthermore, only one other affected male was under 20 years of age (4), Only 32
Pepine et al: Neurothekeoma in a 15-Year-Old Boy
Figure 1. One-centimeter nodule on the left side of the nose.
cases have been described in those under 20 years of age—28 by Galiager and Helwig, 2 by Barnhill (4), and 1 by Aronson (5) (Table 1). Although neurothekeomas are uncommon, they should be included in the clinical differential diagnosis of facial tumors in children and young adults, along with dermoid cyst, epidermoid cyst, hemangioma, juvenile xanthogranuloma, lipoma, nasal glioma, neurofibroma, tieurothekeoma, pilomatricoma, and solitary mastocytoma. In differentiating tumors involving the nose, a cautious approach must be taken; a biopsy should be performed prior to total excision of the lesion to rule out an eticephalocele. Radiography and neurosurgery consultations should be the initial step to differentiate a nasal glioma from an encephalocele. The latter is connected to the central nervous system by a sinus tract (13). Our patient's nasal biopsy specimen showed features consistent with neurothekeoma and the lesion was subsequently completely excised after the possibility of encephalocele was excluded.
273
Figure 2. Section of neurothekeoma, myxoid variety, illustrates cellular nests separated by fibrous septa. (Hematoxylin & eosin; magnification lOOx.)
The histopathology described by Galiager and Helwig included "numerous cellular nests and cords packed among the dermal collagen bundles; within the nests there is considerable variation in cell size and shape; in most cases cells were spindle shaped; the presence of a mucinous background matrix within the cell nests was characteristic" (1). Two subtypes of neurothekeoma recently were documented—cellular and myxoid (4,6). Whether one is a precursor of the other or whether the two are entirely different types of neoplasms is subject to debate. Tumors histologically similar to neurothekTABLE 1. Neurothekeoma in Pediatric Patients No. of Pts.
Race/Sex
Age (yrs)
Location (reference)
28 1 2 1
NR/NR W/F NR/F.M B/M
Neurothekeoma in a 15-Year-Old Boy: Case Report Marci Pepine, M.D.,* Franklin Flowers, M.D.,t and Francisco A. Ramos-Caro, M.D.t * Department of Medicine, and '''Division of Dermatology & Cutaneous Surgery, University of Florida College of Medicine, Gainesville. Florida
Abstract: A 15-year-old boy experienced a rapidly enlarging nodule on his left nose. Histologic findings and immunoperoxidase stains were consistent with the diagnosis of neurothekeoma. The patient was treated with local excision without evidence of recurrence.
Neurothekeoma is a term first coined by Gallager and Helwig in 1980 referring to a benign, cutaneous, soft tissue tumor (1). Prior to this it was referred to as a nerve sheath myxoma, as described by Harkin and Reed (2). The literature appears to contain only 18 documented cases of neurothekeoma involving the face (1,3,4) and only 31 cases occurring in patients less than 20 years of age (1,4,5). This uncommon tumor should be included in the differential diagnosis of pediatric soft tissue tumors involving the centra] face. CASE REPORT A 15-year-old black boy was first seen for evaluation of an enlarging mass on the left side of his nose (Fig. I). He first noted the mass several months earlier, when it was the size of a small pimple. Since then it enlarged rapidly. The mass was reported to be only minimally tender but did bleed easily. The patient was an otherwise healthy boy in no apparent distress, with no pertinent medical history. Physical examination revealed a firm, skincolored nodule, 1 x 1 cm, on the left ala nasi with some telangiectasias. A 2-mm punch biopsy specimen demonstrated fibrosis with increased mucin. On a follow-up visit Address correspondence lo Franklin Flowers, M.D., Division of Dermatology, PO Box 100277, Gainesville, FL 326100277.
three months later, even though the mass had not changed in size or character, the patient requested that it be removed. A parallel excision was performed. Gross examination revealed a 1-cm, round, tancolored nodule. Microscopic examination showed aggregates of epithelioid and stellate cells in a myxoid stroma that were separated by dense, fibrous connective tissue consistent with a neurothekeoma (Fig. 2). Immunocytochemistry was negative for S-100 protein and EM A, which is consistent with that diagnosis. The patient had an unremarkable course without recurrence after nine months of observation. DISCUSSION This case was unusual compared with those described by Gallager and Helwig. In their series, the majority of lesions (68.8%) occurred on other parts of the body, namely, the arms and shoulders (1). Furthermore, our patient was male, and neurothekeomasare more common infemales (4.3:1 ratio)(l), with only nine reports of them occurring in males (4,6-12). In addition, to our knowledge no neurothekeomas reported have been reported in African-Americans. Furthermore, only one other affected male was under 20 years of age (4), Only 32
Pepine et al: Neurothekeoma in a 15-Year-Old Boy
Figure 1. One-centimeter nodule on the left side of the nose.
cases have been described in those under 20 years of age—28 by Galiager and Helwig, 2 by Barnhill (4), and 1 by Aronson (5) (Table 1). Although neurothekeomas are uncommon, they should be included in the clinical differential diagnosis of facial tumors in children and young adults, along with dermoid cyst, epidermoid cyst, hemangioma, juvenile xanthogranuloma, lipoma, nasal glioma, neurofibroma, tieurothekeoma, pilomatricoma, and solitary mastocytoma. In differentiating tumors involving the nose, a cautious approach must be taken; a biopsy should be performed prior to total excision of the lesion to rule out an eticephalocele. Radiography and neurosurgery consultations should be the initial step to differentiate a nasal glioma from an encephalocele. The latter is connected to the central nervous system by a sinus tract (13). Our patient's nasal biopsy specimen showed features consistent with neurothekeoma and the lesion was subsequently completely excised after the possibility of encephalocele was excluded.
273
Figure 2. Section of neurothekeoma, myxoid variety, illustrates cellular nests separated by fibrous septa. (Hematoxylin & eosin; magnification lOOx.)
The histopathology described by Galiager and Helwig included "numerous cellular nests and cords packed among the dermal collagen bundles; within the nests there is considerable variation in cell size and shape; in most cases cells were spindle shaped; the presence of a mucinous background matrix within the cell nests was characteristic" (1). Two subtypes of neurothekeoma recently were documented—cellular and myxoid (4,6). Whether one is a precursor of the other or whether the two are entirely different types of neoplasms is subject to debate. Tumors histologically similar to neurothekTABLE 1. Neurothekeoma in Pediatric Patients No. of Pts.
Race/Sex
Age (yrs)
Location (reference)
28 1 2 1
NR/NR W/F NR/F.M B/M
