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PubMed1994

Glandular peripheral nerve sheath tumours.

Glandular differentiation in peripheral nerve sheath neoplasms is a rare but well recognised phenomenon, believed to represent the least common of the various forms of heterologous differentiation which may occur in tumours of peripheral nerve. This group of tumours--the 'glandular peripheral nerve sheath neoplasms'--are the subject of this short review. The majority of glandular peripheral nerve sheath neoplasms arise in patients with von Recklinghausen's disease and follow a malignant course, but both benign and sporadic forms have now been described. Neither the presence nor the appearance of the glandular foci are believed to influence patient outcome. Predictions of likely biologic behaviour are best based upon an assessment of the nature of the accompanying spindle cell stroma. Glandular peripheral nerve sheath neoplasms must be distinguished from various tumours of soft tissue which may share similar histological features e.g. synovial sarcoma and the epitheloid nerve sheath tumours. Immunohistochemistry and ultrastructural examination may aid in this regard, and have also contributed to our understanding of the nature and origin of the glandular structures, though questions regarding their histogenic origins remain somewhat controversial.

Cell Differentiation↗
PubMed2002

Forward planning using multileaf collimation as a replacement for patient tissue compensation.

In treatment planning, a dosimetrist may encounter a technique that would best be treated by including some type of compensation to correct for tissue or depth variations throughout the field, allowing for a more homogeneous dose distribution. Recent innovations, such as intensity-modulated radiotherapy (IMRT), have been introduced in an effort to address these issues. In many institutions, however, the treatment planning capabilities available may not accommodate consideration of such new technologies. The treatment planner is therefore left to determine how to incorporate these concepts with the current technologies available. While compensation may be an option, this may not always be possible due to the position of the beam or to actual mechanical restraints. Some institutions may also lack the ability and equipment to consider compensation at all. The answer is forward planning IMRT. This concept combines current forward planning techniques with multiple asymmetrically blocked treatment fields, varying the intensity of the beam from a given orientation to produce the desired treatment plan.

Humans↗
PubMed2006

Intrathoracic paraspinal malignant peripheral nerve sheath tumor.

Schwannoma is the most common nerve sheath tumor in the posterior mediastinum, whereas intrathoracic paraspinal malignant peripheral nerve sheath tumor (MPNST) is quite rare. Both benign and malignant nerve sheath tumors may be symptomatic, rendering clinical differentiation of limited utility. On radiographic imaging, erosion of the ribs and vertebral bodies, irregularity in contour, and inhomogeneity in attenuation are not sufficiently reliable for diagnosis of MPNST. Histologically, MPNSTs reveal hypercellularity, nuclear atypia, and mitotic activity. Surgical resection is the main modality of treatment. Postoperative radiation therapy for MPNST has led to a significant reduction in local recurrence. The prognosis is unfavorable. Herein, we present an unusual case of a posterior mediastinal mass in a 50-year-old female with delayed diagnosis of 2 years. After surgical intervention, the histologic finding was MPNST. Postoperative radiation therapy was applied because of incomplete resection. The follow-up chest computed tomography 5 months later revealed a residual soft tissue mass with significant reduction in size over the parathoracic spine area. No neurologic sequelae were identified after surgery.

Female↗
PubMed1992

Malignant parotid salivary gland peripheral nerve sheath tumour in a twelve-year-old girl.

A case of a malignant parotid salivary gland nerve sheath tumour is reported in a 12-year-old girl who developed a right parotid mass. Initial incisional biopsy showed a tumour with a mesenchymal spindle cell appearance. Immunohistochemical studies showed positive staining of tumour cells for vimentin and focally for S-100 protein. These features together with ultrastructural evidence of basal lamina material suggested that the tumour was of nerve sheath origin. After subtotal parotidectomy the tumour metastasised to cervical lymph node and lung. There was evidence of a partial response to chemotherapy. A detailed illustrated histopathological description of the tumour is given.

Child↗
PubMed2000

Malignant peripheral nerve sheath tumors with t(X;18). A pathologic and molecular genetic study.

Spindle cell sarcomas often present the surgical pathologist with a considerable diagnostic challenge. Malignant peripheral nerve sheath tumor, leiomyosarcoma, fibrosarcoma, and monophasic synovial sarcoma may all appear similar histologically. The application of ancillary diagnostic modalities, such as immunohistochemistry and electron microscopy, may be helpful in the differentiation of these tumors, but in cases in which these adjunctive techniques fail to demonstrate any more definitive evidence of differentiation, tumor categorization may remain difficult. Cytogenetic and molecular genetic characterization of tumors have provided the basis for the application of molecular assays as the newest components of the diagnostic armamentarium. Because the chromosomal translocation t(X;18) has been observed repeatedly in many synovial sarcomas, it has been heralded as a diagnostic hallmark of synovial sarcoma. To formally test the specificity of this translocation for the diagnosis of synovial sarcoma, RNA extracted from formalin-fixed, paraffin-embedded tissue from a variety of soft tissue and spindle cell tumors was evaluated for the presence of t(X;18) by reverse transcriptase-polymerase chain reaction. Although 85% of the synovial sarcomas studied demonstrated t(X;18), 75% of the malignant peripheral nerve sheath tumors in our cohort also demonstrated this translocation. We conclude that the translocation t(X;18) is not specific to synovial sarcoma and discuss the implications of the demonstration of t(X;18) in a majority of malignant peripheral nerve sheath tumors.

Adolescent↗
PubMed2004

Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report.

STUDY DESIGN: A case report of primary malignant peripheral nerve sheath tumor (MPNST) of the cauda equina in a child is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the treatment of primary intradural MPNSTs. SETTING: A department of orthopaedic surgery in Japan. METHODS: A 4-year-old boy complained of low-back pain radiating to the left calf. MRI revealed an intradural tumor at L3-L5 level. Following laminectomy of L3, L4 and L5, the tumor was removed en bloc. Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST. RESULTS: Although adjuvant chemotherapy was administered local recurrence and cerebral and spinal metastases of the tumor were found 6 months after the operation. Following additional incomplete removal of the recurrent tumor, radiation therapy was administered. Although recurrent and metastatic tumors disappeared or diminished in size by radiation, tumors increased in size thereafter, despite additional adjuvant chemotherapy. At 21 months after the first operation, he died of pneumonia. CONCLUSIONS: Reported clinical outcomes for patients with primary intradural MPNST are very poor. Although no gold standard for the treatment of tumors has been established yet, surgical removal of tumors combined with postoperative high-dose radiation may be recommended.

Brain Neoplasms↗
PubMed1999

Primary malignant peripheral nerve sheath tumour of the heart.

AIMS: Malignant peripheral nerve sheath tumour (MPNST) is an unusual sarcoma of the heart and should be differentiated from other spindle cell sarcomas. METHODS AND RESULTS: We combined morphological, immunohistochemical and cytogenetic techniques in the differential diagnosis, particularly from monophasic synovial sarcoma. The tumour consisted of dense fascicles, alternating with hypocellular, myxoid zones. There was a marked herniation of tumour cells into the vascular lumen. Immunohistochemically, the tumour cells expressed vimentin and focally S100 protein. There was no expression of cytokeratin or epithelial membrane antigen (EMA). Although we found a complex karyotype, the finding of involvement of 17q11. the loss of chromosome 22, and the absence of a t(X:18)(p11.2:q11.2), which characterizes synovial sarcoma allowed us to the final diagnosis of malignant peripheral nerve sheath tumour. CONCLUSION: MPNST of the heart can be diagnosed by combining morphological and immunohistochemical data. Cytogenetics can be useful in reaching a correct diagnosis, also by helping to rule out monophasic synovial sarcoma.

Aged↗
PubMed2003

Intraneural reticular perineurioma of the neck.

Perineuriomas are infrequent tumors that may be intraneural or extraneural in soft tissue with no apparent relation with a nerve. Some cases of soft tissue perineurioma may have a retiform pattern, but this pattern has not been described to date in intraneural perineurioma. We report a case of a unique perineurioma arising in a nerve of the neck that had a distinctive reticular pattern and was intraneural. The patient was a 21-year-old woman who had a lump on the left side of the neck abutting the vena jugularis. The tumor had features of both reticular perineurioma and intraneural perineurioma with pseudo-onion bulb pattern. The tumor cells were positive for epithelial membrane antigen. The patient is free of tumor 6 months after operation.

Adult↗
PubMed1996

[Schwannoma of the obturatorius nerve as differential diagnosis in cystic adnexa tumors].

We report on a case of benign retroperitoneal schwannoma involving the obturator nerve. Discussing the difficulties of diagnosis and treatment the following became clear: 1.) Clinical examination, ultrasound and computed tomography are not helpful to differentiate a retroperitoneal schwannoma from a cystic ovarian tumour; this problem can perhaps be overcome by MR imaging. 2.) The diagnosis should be borne in mind in cases of retroperitoneal tumours of unclear origin. Appropriate surgery is necessary to avoid nerve damage and paralysis.

Aged↗
PubMed1998

Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of the literature.

Primary intraosseous malignant peripheral nerve sheath tumors (MPNST) are exceptionally rare; the case reported here represents the first documented example occurring in the spinal column. The tumor arose in the second cervical vertebra of a forty year old woman of Asian origin. She had no stigmata of von Recklinghausen's disease (VRD). Plain radiographs and magnetic resonance imaging showed an osteolytic lesion within the expanded body of the vertebra. Histology revealed spindle-shaped tumor cells with nuclei showing pleomorphism, pallisading and mitotic activity. The cells stained positively for S-100 protein. The patient underwent stabilisation of the cervical spine and tumor excision as a two-stage procedure. She died of pulmonary metastases one year later. We have reviewed the literature on the pathology of these tumors.

Adult↗
PubMed2003

Metastatic adenocarcinoma masquerading as a solitary nerve sheath tumour.

We present a case of a solitary metastasis of an adenocarcinoma to a dorsal root ganglion (DRG) following a disease free interval of 12 years after resection of a Duke's C carcinoma. The presentation of this unusually placed metastasis was associated with a 3-year complex pain syndrome and radiological appearances consistent with benign disease. The case highlights the importance of not dismissing unusual lesions as innocent in the presence of a history of malignant disease.

Adenocarcinoma↗
PubMed1994

Plexiform malignant peripheral nerve sheath tumor of infancy and childhood.

We present nine cases of an atypical cellular peripheral nerve sheath tumor, designated plexiform malignant peripheral nerve sheath tumor (MPNST) of infancy and childhood, occurring in five boys and four girls aged 50 days to 13 years (median, 1.5 years). The tumors were located in the lower extremities (five cases), upper extremities (three cases), and the orbital region (one case), and they ranged from 1.5 to 8 cm in size (median, 3 cm). Two lesions were congenital, and another was associated with a history of von Recklinghausen's disease. Follow-up, available in six cases, ranged from 6 months to 15 years (median, 51 months); four patients had local recurrences within 8 to 31 months after excision of the initial lesion, and one with an orbital tumor died of locally invasive disease within 6 months. Histologically, the initial lesions were characterized by a predominantly superficial location within the dermis and subcutis, with occasional extension into deeper soft tissues, had infiltrative or sharply demarcated margins, and resembled entangled or intertwined hypercellular nerve trunks, resulting in a plexiform appearance at low magnification. The nuclei were oval to serpentine with a vesicular chromatin pattern and small basophilic nucleoli; mitoses were seen in all cases and averaged from 1 to 18/10 high-power fields (hpf) (median, 4/10 hpf). Neither necrosis nor vascular invasion was seen. Features diagnostic of plexiform or cellular schwannoma, such as nuclear pleomorphism, Antoni B areas, thick-walled hyalinized blood vessels, and secondary degenerative features were lacking. Other than a prominent plexiform architecture, the lesions were indistinguishable from MPNST occurring in other sites in infants and children. Although none metastasized, the histologic similarity of plexiform MPNST to other childhood MPNST, its relatively high propensity for local recurrences, and its potential to behave in a locally aggressive manner indicate that it is best regarded as low-grade malignant. Overall, the behavior of plexiform MPNST is better than other MPNST in children, probably because of its relatively small size, peripheral and superficial location in most instances, absence of necrosis or vascular invasion, occurrence in young patients, and infrequent association with von Recklinghausen's disease rather than a function of its prominent plexiform architecture. Distinction of plexiform MPNST from cellular and plexiform schwannoma, plexiform neurofibroma, and hamartomatous lesions of childhood is important. Complete excision should be ensured to prevent local recurrences and potential metastases.

Adolescent↗
PubMed1997

Soft-tissue perineurioma. Evidence for an abnormality of chromosome 22, criteria for diagnosis, and review of the literature.

Reported herein are two examples of soft-tissue perineurioma (STP), one arising in the maxillary sinus and the other in subcutaneous tissue of the thigh. Electron microscopy and immunohistochemistry were performed in both cases. Based on our findings and a critical review of the literature, STPs are generally small, well-circumscribed but not encapsulated tumors. Histologically, most STPs resemble fibroblastic tumors, being composed of elongated, wavy cells. The immunohistochemical reactivity for epithelial membrane antigen, the lack of reactivity for S-100 protein, and the presence of ultrastructural features of perineurial cells are typical of this tumor. To explore the possibility that STP, like the intraneural variety of perineurioma, exhibits an abnormality of chromosome 22, we performed fluorescence in situ hybridization with a probe specific for the M-bcr locus, which maps to the chromosome band 22q11. In both our tumors, a high percentage of nuclei having only one M-bcr signal (44 and 96%) was observed. Our findings indicated deletion of part or all of chromosome 22 and support the view that both soft-tissue and intraneural perineurioma are part of a spectrum of perineurial neoplasia.

Chromosome Aberrations↗
PubMed1997

Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults.

This report describes 19 cases of a distinctive sclerosing perineurial tumor of the hands. Fourteen patients were male and five were female (age range 9-55 years; median age 24.5 years). The process typically presented as a painless mass and was present from 6 months to 40 years before resection. Sites of involvement were the thumb (n = 6); index (n = 3), middle (n = 4), and ring (n = 4) fingers; and the palm (n = 2). The lesions were generally well marginated but nonencapsulated. They had a firm, fibrous consistency and ranged in size from 0.7 to 3.3 cm in maximum dimension. Microscopic examination showed abundant dense collagen and variable numbers of small, epithelioid, and spindled cells exhibiting corded, trabecular, and whorled (onion bulblike) growth patterns. Immunoreactivity was present for epithelial membrane antigen (15 of 15); a cytokeratin cocktail containing AE1, AE3, and CK1 (four of 14); CAM 5.2 (one of 12); vimentin (12 of 12); muscle-specific actin (nine of 14); alpha-smooth muscle actin (six of 14); collagen IV (six of six); laminin (five of six); and CD99 (three of five). Ultrastructural features consistent with perineurial cells were noted. All of the lesions were locally excised. Follow-up was obtained for seven patients, with mean and median follow-up intervals of 12 years 7 months and 10 years 6 months, respectively. None of the lesions have recurred. This study advances the morphologic spectrum of perineurioma, a rare tumor of nerve sheath derivation. Familiarity with this distinctive subtype should help to avoid confusion with other processes, including a fibroma of tendon sheath, the sclerotic fibroma associated with Cowden's disease, an epithelioid neurofibroma, a late stage of tenosynovial giant cell tumor, and sclerosing adnexal tumors.

Adolescent↗
PubMed1997

Malignant peripheral nerve sheath tumor of the pleura with epithelial and rhabdomyoblastic differentiation: report of a case clinically simulating mesothelioma.

A primary malignant peripheral nerve sheath tumor (MPNST) of the pleura that clinically mimicked a malignant mesothelioma in a 57-year-old man is described. Histologically, the tumor had features similar to those described in cases of the so-called epithelioid MPNST. A unique finding in this case was the demonstration of keratin expression in the epithelioid component of the tumor, as well as the presence of rhabdomyoblasts. This is the first example of an MPNST with heterologous elements arising in the pleura. Immunohistochemical and ultrastructural studies were important in differentiating this tumor from other malignancies with sarcomatoid and epithelioid features involving the pleura.

Actin Cytoskeleton↗