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At least 19 recordsLinked to original sources

PubMed1990

Cellular neurothekeoma. A distinctive variant of neurothekeoma mimicking nevomelanocytic tumors.

We describe the clinical, histopathologic, and immunohistochemical characteristics of five examples of a distinctive subtype of neurothekeoma we term "cellular neurothekeoma" (CNT). These lesions are nondescript papules or nodules primarily involving the head and neck areas of young adults. Histopathologically, CNT are fairly well-defined proliferations involving the reticular dermis; they consist of fascicles of polygonal and spindle cells with eosinophilic or pale-staining cytoplasm and neuroid characteristics. Low-grade cytologic atypia and mitotic activity are common. All immunohistochemical markers--including S-100 protein, myelin basic protein, epithelial membrane antigen, and histiocytic antigens--have failed to show positivity in our laboratory. Separation from myxomatous variants of neurothekeoma is based on greater cellularity, less myxomatous change, and less pronounced plexiform compartmentalization by fibrous septae, which resemble perineurium. The differential diagnosis usually includes spindle and epithelioid cell (Spitz) nevus, malignant melanoma (particularly desmoplastic-neurotropic melanoma), cellular blue nevus, and fibrohistiocytic proliferations. The recognition of CNT and its differentiation from melanoma are important so that overly aggressive therapy is avoided.

Adolescent↗

PubMed1991

Studies on the cellular origin of neurothekeoma: clinical, light microscopic, immunohistochemical, and ultrastructural observations.

The clinical, histopathologic, and immunohistochemical features of 11 cases of neurothekeoma are reported. One case was examined by electron microscopy. The mean age of the patients was 27.1 years; the study comprised eight female and three male patients. Most lesions were nondescript papules and located on the upper part of the body, seven cases of neurothekeoma on the head. Eight cases were classified as cellular neurothekeoma on the basis of a striking fascicular pattern and three cases as myxomatous neurothekeoma because of prominent myxoid stromal change. All cellular neurothekeomas failed to express S-100 protein, whereas the three myxomatous types were strongly positive for this marker. Other than vimentin, there was no significant immunoreactivity with other immunohistochemical markers. Ultrastructural study of one case of cellular neurothekeoma was inconclusive for cell type although a perineurial origin could not be excluded. On the basis of these results, we conclude that cellular neurothekeoma differs from myxomatous neurothekeoma not only by clinical and histologic findings but also by immunoreactivity with S-100 protein. These findings also suggest the existence of two distinct subtypes of neurothekeoma and possible origin of the two variants of neurothekeoma from different cell types or at least variation in phenotypic expression of a common cell type. On the other hand, it cannot be excluded that these two variants are different stages in the natural history of neurothekeoma.

Adolescent↗

PubMed1999

PGP9.5: a marker for cellular neurothekeoma.

Neurothekeomas are benign, predominantly cutaneous neoplasms that are divided histologically into myxoid, intermediate, and cellular types. Although it is generally agreed that the myxoid type of neurothekeoma has a neural origin, the lack of consistent immunoreactivity to neural markers and insufficient ultrastructural evidence of neural differentiation in cellular neurothekeoma have brought the origin of cellular neurothekeoma into question. In this report the authors show that immunoreactivity to protein gene product 9.5 (PGP9.5)--a broad neural marker--is positive in 100% of cases of cellular neurothekeoma using microwave antigen retrieval, as well as in all cases of myxoid and intermediate neurothekeoma. In contrast, immunoreactivity to S-100 protein is only positive in 3 of 12 cases of cellular neurothekeoma. These results show that PGP9.5 is a useful marker for identifying cellular neurothekeoma, as well as other types of neurothekeomas using the antigen retrieval method. The results are consistent with the notion that cellular neurothekeoma has a neural differentiation.

Adolescent↗

PubMed2004

Cellular neurothekeoma with histiocytic differentiation.

BACKGROUND: It is generally accepted that the two types of neurothekeoma (myxoid type and cellular type) represent the two poles of a spectrum. This concept, however, has recently been challenged, and cellular neurothekeomas have been suggested as a separate classification and are included in the "fibrohistiocytic" category by some authors. Cellular neurothekeomas have been reported to show negative immunohistochemical staining for histiocytic markers, and PG-M1 is now considered to be the most reliable histiocytic marker. CASE REPORT: We report a case of cellular neurothekeoma. The histopathological features in this case were typical for cellular neurothekeoma. Immunohistochemically, the neoplastic cells were diffusely positive for S-100A6 protein, PGP9.5, CD10, CD68 (KP1), PG-M1, and Vimentin, and negative for other antibodies including S-100 protein and factor XIIIa. CONCLUSIONS: Cellular neurothekeoma expressing both KP-1 and PG-M1 is considered to show histiocytic differentiation, and may be interpreted as a neoplasm with immature nerve sheath differentiation, incidentally expressing histiocytic markers, or as an undifferentiated neoplasm derived from the neural crest cells of nerve sheath/fibrohistiocyte lineage. These results, such as the concomitant expressions of PGP9.5/S-100A6 and PG-M1/CD68 (KP-1), support the theory of multiple differentiation in cellular neurothekeomas. The significance of the expression of CD10 in this cellular neurothekeoma is unclear.

Adult↗

PubMed2004

Neurothekeoma: report of a case in an infant and review of the literature.

Neurothekeomas (nerve sheath myxomas) are uncommon benign tumors of nerve sheath origin. We describe an infant with a neurothekeoma and review the literature of neurothekeomas in children and adults. Neurothekeomas have been reported in 292 patients whose ages have ranged from 15 months to 84 years. They occur twice as often in women as in men. Neurothekeomas were most commonly located on the upper extremities and the head and neck. They also occurred on the trunk, the lower extremities, and mucosa. Histologic variants of neurothekeomas include classical, cellular, and mixed tumors. Surgical excision was the most common treatment. Recurrences were attributed to an incomplete excision; treatment of these tumors is by complete excision. Neurothekeomas should be included in the differential diagnosis of dermal nodules in infants and children.

Female↗

PubMed2003

Antibody to S100a6 protein is a sensitive immunohistochemical marker for neurothekeoma.

BACKGROUND: Neurothekeoma is a benign tumor of putative peripheral nerve sheath origin. It occurs in a myxoid (classic) variant, cellular variant, and intermediate (mixed) variant. Cellular neurothekeoma (CNT) usually involves the head and neck or extremities of young patients. Histologically, CNT can be confused with melanocytic and fibrohistiocytic lesions. An immunohistochemical antibody panel is often necessary to confirm the histological impression and exclude melanocytic and/or fibrohistiocytic lesions. METHODS: Formalin-fixed, paraffin-embedded archival tissues were evaluated by immunohistochemistry using antibodies specific for S100A6 and PGP9.5 in 11 cases of neurothekeoma (seven cellular, four myxoid). A variety of other antibodies were evaluated by immunohistochemistry at the time of initial diagnosis. RESULTS: All 11 neurothekeoma cases were positive for S100A6 protein (four cases, weak/1+; seven cases, strong/2+), corresponding to 100% sensitivity. In contrast, eight of 11 neurothekeoma cases (73% sensitivity) were positive for PGP9.5. All seven CNT cases were negative for S100B, as expected. CONCLUSIONS: Anti-S100A6 is a highly sensitive antibody for neurothekeomas, including CNT, and, in our experience, is superior in sensitivity to PGP9.5. However, like other antibodies used in evaluating neurothekeomas, S100A6 lacks specificity, as has been demonstrated in previous studies. Nevertheless, S100A6 can be useful in an immunohistochemical antibody panel to evaluate lesions where the differential diagnosis includes CNT.

Adolescent↗

PubMed1994

Histologic spectrum of neurothekeoma and the value of immunoperoxidase staining for S-100 protein in distinguishing it from melanoma.

Neurothekeoma, a benign cutaneous lesion of probable nerve sheath origin, is divided histologically into two subtypes--myxoid and cellular. However, we believe that neurothekeoma encompasses a wider spectrum of lesions, with the myxoid and cellular subtypes falling at either end of the morphologic spectrum. Because the cellular variant of neurothekeoma sometimes resembles melanoma, it presents a difficult diagnostic problem. We report the histologic and immunohistochemical findings in 14 cases of neurothekeoma and review the findings in 35 additional cases from the literature. A detailed analysis of the histologic spectrum is also included. When examined by immunostains, only the myxoid variants of neurothekeoma stain positively for S-100 protein. We conclude that when the histological differential diagnosis is between cellular neurothekeoma and melanoma, an S-100-positive lesion should be regarded as melanoma.

Adult↗

PubMed2005

Neurothekeoma.

OBJECTIVES: The purpose of this study was to investigate and share the experiences at The University of Texas M. D. Anderson Cancer Center in diagnosing, treating, and following patients with neurothekeoma. We report 13 cases. STUDY DESIGN: Case series. The clinical, surgical, and pathology records of the 7 patients with neurothekeoma treated at M. D. Anderson Cancer Center were retrospectively reviewed. In addition, the pathology records of 6 patients reviewed by the Department of Pathology for diagnosis only were retrospectively reviewed. RESULTS: Nine patients were women. Their mean age was 30 years (median, 24 years). Six of the lesions were on the head or neck, 4 were on extremities, and 2 were on the trunk, and the location of 1 was not recorded. Two lesions had been previously diagnosed, histologically, as leiomyosarcomas, 1 as a malignant nodular hidradenoma, and 1 as a clear cell hidradenoma. All the lesions had the characteristic nested pattern of growth, with various degrees of myxoid background. Mitotic figures and marked cellular pleomorphism were not common. Six cases were treated with wide local excision. A lesion of the nasal ala was excised by using Mohs micrographic surgery. Most cases were limited to the dermis, but 2 lesions infiltrated subcutaneous tissue and skeletal muscle. All patients were without evidence of disease at 8 months median follow-up range (0 to 35 months). CONCLUSION: Neurothekeoma is a benign neoplasm occurring usually in women and commonly in the head and neck. Because it may be locally invasive, treatment with wide local excision using frozen section control of margins is recommended. Care must be taken in the pathologic diagnosis of cutaneous neoplasms, and neurothekeoma should be considered in the differential diagnosis for spindle cell lesions. SIGNIFICANCE: Neurothekeoma is commonly misdiagnosed, pathologically and clinically, and it can be treated successfully with surgical excision. EBM RATING: C.

Adolescent↗

PubMed2004

Microphthalmia transcription factor and NKI/C3 expression in cellular neurothekeoma.

While the usual or myxoid-type neurothekeoma has been reasonably well established as being a tumor of neural origin, the cellular neurothekeoma remains in disputed histogenesis. We studied a series of 11 cellular neurothekeomas using paraffin immunoperoxidase staining with microphthalmia transcription factor (Mitf), NKI/C3, and S-100. The majority of the tumors in our series stained with NKI/C3 (9/11) and Mitf (9/11). All failed to stain with S-100. Furthermore, we divided our series of cellular neurothekeomas according to cytomorphology; tumors demonstrating predominantly spindled morphology, predominantly epithelioid morphology, and mixed spindle and epithelioid morphology. The two tumors that failed to stain with NKI/C3 both demonstrated predominantly spindled morphology. One of the tumors that failed to stain with Mitf showed exclusive spindled morphology, while the other showed mixed morphology (spindle and epithelioid). Two of the tumors, which stained strongly with Mitf, however, showed exclusive epithelioid morphology. This current study furthers the concept that cellular neurothekeoma is a tumor of neuroectodermal origin, and further suggests that it may express some component of melanocytic differentiation.

Adolescent↗

PubMed2000

The "neurothekeoma": immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics.

In contrast with the myxoid variant of neurothekeoma (nerve sheath myxoma), evidence of neurosustentacular (NS) differentiation in the so-called cellular and mixed (intermediate) variants of neurothekeoma remains controversial. In this study, we selected 22 tumors coded as neurothekeoma or nerve sheath myxoma from the Soft Tissue Registry of the AFIP. Each tumor was histologically subtyped as either a myxoid/hypocellular neurothekeoma (MN) (N = 11) or as a "cellular" or "mixed" (intermediate) neurothekeoma variant (C&MV) (n = 11) and analyzed immunohistochemically. The MNs were composed of small, cytologically bland cells arranged in a loose cellular network or in files within highly myxomatous nodules delineated by dense collagen. The tumors showed clear-cut evidence of NS differentiation by exhibiting consistent immunoreactivity for S-100 protein (11 of 11 cases) and low-affinity nerve growth factor receptor, p75(NGFR), (NGFR) (10 of 10), and variable reactivity for glial fibrillary acidic protein (GFAP) (10 of 11) and CD57 (Leu-7) (5 of 9). They also showed pericellular collagen type IV (CIV) expression (9 of 9), scattered intralesional CD34-positive spindled cells (10 of 10), epithelial membrane antigen (EMA)-positive spindled cells located within the adjacent dense collagen (8 of 11), and immunoreactivity for alpha-smooth muscle actin (SMA) (3 of 10) and calponin (4 of 9). In 4 cases, scattered intralesional neuraxons were detected by the Bodian histochemical method or immunohistochemically with anti-neurofilament protein. The tumors had a male-to-female ratio of 6:5, a peak incidence in the 4th decade of life, and an anatomic distribution that included the upper and lower limbs and back. The C&MVs included 9 "mixed" and 2 "cellular" variants. C&MVs differed histologically from MNs by their higher cellularity and presence of larger spindled or epithelioid cells with vesicular nuclei. Immunohistochemically, the tumor cells expressed CIV (9 of 10), calponin (7 of 9), SMA (5 of 10), Leu-7 (1 of 7), S-100 protein (1 of 11), but not NGFR, GFAP, or CD34. EMA-positive spindled cells surrounded tumor fascicles in 1 case. Intralesional neuraxons were not identified. Clinically, these tumors differed from the MNs by exhibiting a male-to-female ratio of 4:7, a peak incidence in the 2nd decade, and an upper body distribution. Our results indicate that the MN shows NS differentiation and is the bona fide nerve sheath tumor, whereas the C&MVs fail to show convincing evidence of NS differentiation and probably warrant a separate classification.

Adolescent↗

PubMed2000

[Cellular neurothekeoma of the bulbar conjunctiva. A case report].

CASE REPORT: Cellular neurothekeoma is a distinctive subtype of neurothekeoma with an undetermined histopathogenesis. We report a very rare case of bulbar conjunctiva cellular neurothekeoma in a 23-year-old woman. DISCUSSION: Neurothekeoma or nerve sheath myxoma is a rare benign tumor, which arises during childhood, and early adult life. It mainly locates in the upper portion of the body (head, neck and upper limbs). As far as we know, this is the first case report of cellular neurothekeoma in this location. Excisional biopsy is necessary to make the differential diagnosis from other cystic and malignant pathologies of the conjunctiva.

Adult↗

PubMed2002

Cytologic findings of myxoid neurothekeoma: case report based on fine-needle aspiration cytology, immunohistochemistry, and correlating histopathology.

Myxoid neurothekeomas (nerve sheath myxomas) are rare benign cutaneous neoplasms that may morphologically mimic other myxoid neoplasms of skin and soft tissue. The cytologic and histopathologic features of this lesion may resemble various myxoid sarcomas, chordoma, myxoid neurofibroma, dermal cutaneous mucinosis, and cutaneous myxoma as well as other myxoid or chondroid neoplasms. In this study, a myxoid neurothekeoma was analyzed using multiple techniques. We found that myxoid neurothekeomas reveal a nonspecific pattern by fine-needle aspiration, including stellate cells embedded within an abundant metachromatic myxoid stromal matrix. These are cytologic features shared by various other subcutaneous neoplasms and thus may not be helpful in forming a definitive diagnosis. Histopathologically, the tumor is composed of nodules of myxoid stroma containing interspersed bland spindled and stellate cells. Immunohistochemical studies show tumor cell positivity for S-100 protein and vimentin, a profile shared by other neoplasms with similar cytologic features and therefore of little diagnostic value. The histologic and cytologic differential of subcutaneous and soft tissue myxomatous lesions is broad and, therefore, is of unique value to the cytopathologist to consider myxoid neurothekeomas among the differential of other myxomatous neoplasms.

Biopsy, Needle↗

PubMed1998

Cellular 'neurothekeoma': an epithelioid variant of dermatofibroma?

AIMS: Cellular neurothekeoma is a rare benign cutaneous neoplasm with conflicting opinions regarding its histogenetic origin (nerve sheath, smooth muscle, myofibroblasts) as well as its relation to myxoid neurothekeoma (nerve sheath myxoma). The present series describes 15 cases whose clinicopathological features indicate a relationship to dermatofibroma. METHODS AND RESULTS: In this retrospective clinicopathological study, the lesions preferentially occurred in adolescents to young adults on the upper half of the body, often clinically diagnosed as some kind of fibrohistiocytic tissue response. Besides characteristic whorled nests to fascicles of palely eosinophilic epithelioid cells, all lesions showed variable clues pointing to dermatofibroma: acanthosis, ill-defined storiform periphery, peripherally accentuated prominent sclerosis and lymphocytic demarcation/infiltration. Immunohistochemically, all cases were positive with NK1C3 (CD57), KiM1p and proliferating cell nuclear antigen, seven were positive for neurone specific enolase, five for factor XIIIa, six for smooth muscle specific actin and three for E9, an antimetallothionein marker. These findings are similar to those of conventional dermatofibromas, the variability of the profile being best explained by time cycle and function dependent changes. Ultrastructurally, two cases showed microfilaments, attachment plaques, prominent pinocytosis and focal remnants of basal lamina. A careful study of the data and photomicrographs from the literature reveals that in many cases similar conclusions could be reached. Obvious discrepancies are most likely due to the confusion with myxoid neurothekeoma, a well circumscribed, more spindly and myxoid, S100 positive lesion of Schwannian origin. CONCLUSION: The appearance of dermatofibromas is markedly influenced by architectural, e.g. in deep penetrating dermatofibroma, and/or cellular/stromal criteria, e.g. in epithelioid cell histiocytoma or sclerosing dermatofibroma. Cellular neurothekeoma seems to be a variant of dermatofibromas with both architectural and cellular/stromal peculiarities, i.e. plexiform pattern, epithelioid cytology and stromal sclerosis.

Actins↗

PubMed2003

Atypical cellular neurothekeoma.

BACKGROUND: Neurothekeoma is a distinctive neoplasm that in the past was often lumped into the broad category of benign peripheral nerve sheath tumors. Atypical cellular neurothekeoma is a rare variant of this neoplasm. This atypical variant is characterized by features that can include the following: large sizes (up to 6 cm), deep penetration extending into skeletal muscle and/or subcutaneous fat, diffusely infiltrative borders, vascular invasion, high mitotic rate, and marked cytologic pleomorphism. METHODS: In this report, we describe the clinical, histopathologic, and immunohistochemical features of a case of atypical cellular neurothekeoma in a 34-year-old woman. RESULTS: This neoplasm demonstrated both the cellular subtype and intermediate subtype. In the cellular area, cells demonstrated rounded or ovoid nucleus with abundant cytoplasm. Mitotic activity ranged from 8 to 12 per 10 high-powered fields, and few individual cell necroses were present. In the intermediate area, cells were arranged as smaller nests with admixed abundant mucin. Mitotic activity ranged from 1 to 2 per 10 high-powered fields. These cells were negative for S100, epithelial membrane antigen, and smooth muscle actin. CONCLUSION: Dermatologists should be aware of the features of atypical cellular neurothekeoma. Although the atypical features raise concern about the malignant potential of this lesion, previous cases show that complete surgical excision of these lesions is curative.

Adult↗

PubMed2006

Multiple cellular neurothekeomas--a case report and review on the role of immunohistochemistry as a histologic adjunct.

BACKGROUND: Cellular neurothekeoma is a relatively rare, benign cutaneous neoplasm, which usually presents as a solitary papule or nodule involving the head and neck area of young adults. Multiple neurothekeomas have not, to date, been known to occur. METHODS: We report a 30-year-old, otherwise healthy, male who presented with multiple neurothekeomas (15) in the head and neck area over a period of 12 years. RESULTS: While the unifying feature of all biopsied (10 of 15) lesions was the presence of epithelioid cells--the lesions differed in their cellularity and the degree of sclerosis of the stromal component. Antigenic profiling of the lesional cells revealed expression of vimentin, NKI/C3, PGP 9.5, factor XIIIa and CD68 but not S100, HMB45, MelanA, EMA, MSA, desmin, CD57 or NGF-R. CONCLUSIONS: This case report is the first to document the occurrence of multiple cellular neurothekeomas. An unusual histologic feature of some of the biopsied lesions was the presence of a markedly sclerotic stroma.

Adult↗

PubMed2005

Mixed-type neurothekeoma presenting with an unusual clinical appearance of multiple satellite lesions on the back.

BACKGROUND: Neurothekeoma is a rare cutaneous neoplasm, often occurring as a nondescript cutaneous nodule on the central face, shoulders, and upper extremities. OBJECTIVE: To present a patient with mixed-type neurothekeoma with an unusual clinical presentation. METHODS: We report a 29-year-old female who developed an asymptomatic, red, dermal nodule with satellite papules on the back over a period of 6 months. RESULTS: The coalescing papules on the back were excised, and the histopathology and immunohistochemical study revealed a mixed-type neurothekeoma. CONCLUSION: Neurothekeoma usually presents with a small, solitary, and slow-growing nodule or papule on the upper body. The case is interesting because of the unusual clinical manifestation of one dermal tumor with several satellite lesions.

Adult↗

PubMed2006

Atypical cellular neurothekeoma treated with Mohs micrographic surgery.

BACKGROUND: Atypical cellular neurothekeoma is a rare neoplasm generally regarded as a benign tumor with locally aggressive behavior. Recurrence is common with inadequate excision, but metastatic disease has yet to be reported. OBJECTIVE: The objective was to report a case of atypical cellular neurothekeoma of the nasal ala in a young woman treated by Mohs micrographic surgery. The biologic behavior and histopathology will be reviewed. METHODS: Case report and review of the literature. RESULTS: The neoplasm was extirpated in a three-stage, five section Mohs surgery procedure. The resulting defect was repaired by a two-stage, pedicled nasolabial transposition flap with a cartilage graft taken from the ipsilateral ear. No clinical recurrence was noted after 6-months of follow-up. CONCLUSION: Mohs micrographic surgery is unsurpassed in its efficacy in treating a wide variety of nonmelanoma skin cancers. Although most commonly used to address basal and squamous cell carcinoma, it has also been reported as a successful treatment for melanoma and a wide variety of cutaneous malignancies. We discuss a case of atypical cellular neurothekeoma removed with the Mohs technique. Debate in the literature is ongoing regarding the true histogenesis of this rare tumor. Because of this tumor's local destructive behavior and propensity to recur with inadequate resection, we recommend Moths micrographic surgery for the treatment of cellular neurothekeomas.

Adult↗

PubMed2000

[Cellular neurothekeoma, a rare cutaneous tumor. Anatomo-clinical and immunohistochemical study of 2 cases].

Cellular neurothekeoma is an unusual cutaneous tumor described in 1986. We report two new cases in 14-year-old girls. Both tumors measured less than one centimeter and were located on the forearm and the shoulder. They had a plexiform architecture and were developed in the dermis and the dermo-hypodermic junction. Spindle and epithelioid tumor cells were immunoreactive for vimentin, NKI-C3, NSE, CD68 and smooth muscle actin. There was no recurrence 2 and 7 months after the operation. Cellular neurothekeoma is a benign tumor. Its histogenesis is still unknown. Histological and immunohistochemical features, with positivity of the tumor cells for NKI-C3, a non specific melanocytic marker, and negativity for S-100 protein and HMB-45, allow to differentiate cellular neurothekeoma from myxoid neurothekeoma and melanocytic tumors.

Actins↗