Nerve Sheath Myxoma (Neurothekeoma) of the Gingiva,A Case Report and Review of the Literature

Nerve sheath myxoma (NSM) is a benign peripheral nerve sheath tumor that rarely occurs in the oral cavity. Among the 23 reported intraoral cases, no lesion has previously been reported on the gingiva. In this report, we describe the first gingival case of oral neurothekeoma with histopathologic and immunohistochemical characteristics. The patient, a 32 year old female presented with a slowly growing gingival mass diagnosed clinically as an epulis. The lesion was surgically excised. Histopathologically, the lesion presented as a submucosal multinodular mass composed of spindle and stellate-shaped cells with a myxoid background. Immunohistochemically, the tumor cells were sporadically positive for S-100 and NSE and negative for GFAP, EMA, SMA, CD68 and HMB45. The immunoprofile of this lesion confirmed a Schwann cell origin. The lesion was followed up for 10 months with no reports of recurrence.     

Giant retroperitoneal liposarcoma: report of a case

Liposarcoma is a rare and primary malignancy developed from mesenchymal tissue. We report the case of a 32-year-old woman who had a complete exeresis of a 15 cm retroperitoneal myxoid liposarcoma. Three months later, a 9 cm local recurrence was excised and radiotherapy was performed. In 2000, a local recurrence and an intestinal localisation of the liposarcoma were observed. The retroperitoneal tumor could not be removed and she had an intestinal resection followed by chemotherapy. With the literature data, we review the pathological, therapeutic and prognostic aspects of this tumor.     

Liposarcoma: Clinico-pathological analysis,prognostic factors and survival in a series of 307 patients treated at a single institution

Background and objectives

Liposarcoma (LPS) is a malignant mesenchymal tumor and the most common soft tissue sarcoma. Four different subtypes are described: well differentiated (WD) LPS or atypical lipomatous tumor (ALT), dedifferentiated (DD) LPS, myxoid LPS, and pleomorphic LPS (PLS). The objective of the study was to investigate prognostic factors and clinical outcome of liposarcoma.

Atypical cellular neurothekeoma--a diagnosis to be aware of.

We present a rare case of atypical cellular neurothekeoma arising in the area of a previous nose piercing on the ala of a 34-year-old female. Neurothekeoma is a benign tumour of probable nerve sheath origin. The cell of origin for neurothekeoma is still unknown but most ultrastructural and immunohistochemical studies have favoured the Schwann cell perineurium or fibroblasts. A similar but distinct, more cellular variant with a less prominent myxoid component, was termed cellular neurothekeoma and, unlike myxoid neurothekeoma, the cellular variety generally lacks immunoreactivity to most neuronal markers. Atypical cellular neurothekeoma is characterised by the following features: large size of up to 6 cm, penetration into subcutaneous fat and or muscle, diffusely infiltrating borders, vascular invasion, a high mitotic rate and marked cytological pleomorphism. In this report we provide a review of the relevant literature and describe the clinical, histological and immunohistochemical features of an atypical cellular neurothekeoma excised from the ala nasi of a 34-year-old female, a hitherto unreported lesion in the plastic surgery literature.     

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.     

Pulmonary extraskeletal myxoid chondrosarcoma: A case report and literature review

BackgroundExtraskeletal myxoid chondrosarcoma (EMC) accounts for the 3% of all soft tissue sarcomas and it's categorized as a tumour of uncertain differentiation. This entity has shown to have the recurrent balanced chromosomal translocation t(9;22) (q22;q12.2), which leads to the oncogenic fusion gene EWSR1-NR4A3. This sarcoma usually presents as a slow growing, palpable mass in the extremities. EMC arising from the lung is extremely infrequent. We report one case of pulmonary extraskeletal mixoid chondrosarcoma and a review of the world literature.Case reportA 69-year-old male patient presented with intermittent hemoptysis for the last 6 months. A PET/CT scan showed a hypermetabolic solid mass with lobulated borders of approximately 29 × 26 mm in the inferior right lobe. We performed a right thoracotomy with inferior lobectomy and lymphadenectomy of levels VII, VIII, X, and XI levels. The neoplasm was constituted by cords of small cells with small round nucleus and scarce cytoplasm immerse in an abundant myxoid matrix. The immunophenotype was positive for MUM-1, CDK4, MDM2, and showed focal expression for S-100 protein and CD56. The final pathology report revealed a pulmonary extraskeletal mixoid chondrosarcoma. No further surgical interventions or adjuvant therapies were needed.ConclusionEMC is an intermediate-grade neoplasm, characterized by a long clinical course with high potential for local recurrence and distant metastasis. Treatment for EMC is surgical and non-surgical treatment is reserved for recurrence or metastatic disease. Pulmonary extraskeletal myxoid chondrosarcoma is a rare neoplasm with only isolated case reports found in the literature.     

A Case of Digital Superficial Angiomyxoma

Superficial angiomyxoma (SA) is a rare superficial benign tumor of the skin that is characterized by slow growth. It is painless, non-invasive, and prone to relapse. It commonly occurs on the head, neck, trunk, and limbs and in the genital region. Herein, we describe a case of a 33-year-old man who presented with a soft tender tumor measuring 1.5 × 2.0 cm on the lateral aspect of the left second finger. The histopathology of the tumor indicated that it was a lobulated tumor rich in myxoid stroma and parenchymal blood vessels in the dermis, with spindle-to-stellate tumor cells in higher-power view and no mitotic or atypical figures. Immunohistochemical studies revealed that the tumor cells were positive for CD34 and negative for S100. Based on these clinical and histological findings, the patient was diagnosed with SA.     

Myxoid Malignant Fibrous Histiocytoma of the Breast: Report of a Case

Malignant fibrous histiocytoma (MFH) is the most common type of soft tissue sarcoma, but it rarely develops as a primary tumor in the breast. Furthermore, no case of the myxoid variant of MFH in the breast has ever been documented. We report the case of a 52-year-old woman with a breast tumor that was immunohistochemically confirmed to be myxoid MFH. She underwent a radical mastectomy and is currently well with no evidence of local recurrence or metastatic spread after 3 years of follow-up. Received: August 6, 2001 / Accepted: March 5, 2002     

Neurothekeoma of the hand

Neurothekeomas are rare, benign connective tissue tumours probably of Schwann cell origin. We report an unusual case of a neurothekeoma involving the hand. Histological examination revealed characteristic myxoid nodules with spindle shaped cells. The immunocytochemical reaction for S-100 protein and neuron-specific enolase was positive. Complete excision proved curative as the tumour was well encapsulated.     

Recurrence of juxtacortical chondrosarcoma arising on the rib

Juxtacortical chondrosarcoma developing on the surface of a bone is quite rare. We report a case of juxtacortical chondrosarcoma arising on the fourth rib of a 76-year-old man. Intraregional tumor resection was performed, but local recurrence was detected after 6 months. The patient underwent wide resection including the ribs, and reconstruction of the thoracic wall. He was released with a good prognosis after a year. This case emphasizes the importance of biopsy analysis before surgery to carefully evaluate tumor spread in the cartilage and performing wide resection even if the tumor is easily separated from the bone.     

Primary synovial sarcoma of the left heart with large amount of necrotic tissue

We present a case of primary synovial sarcoma arising from the left heart, an extremely rare occurrence, with a large amount of necrotic tissue, which suggested a poor prognosis. After incomplete tumor resection, chemotherapy and radiation therapy were performed; however, PET/CT findings at 26 months after the operation revealed local recurrence. Although we performed two additional operations following chemotherapy, the patient died from local recurrence at 36 months after the initial operation. In this case of synovial sarcoma arising from the left heart, even though aggressive multimodality therapy was performed, the prognosis was still poor.     

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.     

Treatment modalities and outcomes of pleomorphic hyalinizing angiectatic tumor: a systematic review of the literature

A systematic review of the cases documented in the literature regarding Pleomorphic hyalinizing angiectatic tumor of soft parts (PHAT) was performed in order to identify (1) location on presentation (2) surgical treatment modality (3) recurrence rate (4) any associations between location, age, histology, surgery type on recurrence. A systematic review of medical literature listed on PubMed was conducted identifying any prior case report and/or case series of diagnosed PHAT, with no exclusion based on language or time. Twenty-nine articles were identified removing any articles with duplicated cases yielding a total of 93 cases. Cases were broken down by gender, presenting location (UE/LE/axial), surgery type [wide local resection, non-wide local, wide local with radiation therapy (RT), non-wide local with RT], recurrence, and time to recurrence. The mean age at presentation was 54.5 ± 17.1 (range 10–89) with the 76% of cases appearing in the lower extremity (15% UE, 9% Axial). Of the 93 patients, 74 had a known surgical procedure, 31% WL, 40% NWL, 8% WL + RT, 1% NWL + RT. Of those treated surgically, 63 pts had documented follow-up and 18 (29%) had recurrence. A strong association was observed between surgery type and recurrence. Local recurrence was more common within the group undergoing NWLE in 52% (16/41) of cases (p = 0.002). Kaplan–Meier analysis showed an estimate mean time for recurrence of 43.87 months [95% confidence interval (CI) 24.52–63.22; and standard error (SE) 7.59] for the entire population. A trend was also seen toward males having a shorter disease-free survival than females (29.4 mos. vs. 69.5 mos.). No significant association seen between size, location, histology type and recurrence. PHAT has a characteristic presentation in the LE with a relatively high rate of local recurrence and slow-growing potential. Wide local excision appears to be superior in decreasing recurrence rates and a long-term follow-up period is needed.     

Therapeutic experience with primary liposarcoma from the sigmoid mesocolon accompanied with well-differentiated liposarcomas in the pelvis

Multifocal liposarcoma with different histological types is rare, especially that originating from the mesentery. We herein report a case of primary myxoid liposarcoma from the sigmoid mesocolon that was accompanied with well-differentiated liposarcomas in the pelvis. The location, the fat signal and the relationship with adjacent organs of the liposarcomas were well shown in the axial, coronal and sagittal dimensions of MRI, giving a comprehensive and specific image before surgery. To alleviate the patient’s symptoms and mental stress, a laparotomy was performed. The tumors were all completely excised with macroscopic free margins. The final histopathological report showed that the tumor in the sigmoid mesocolon was a pure myxoid liposarcoma, while the pelvic tumors were spindle cell liposarcomas, a special type of well-differentiated liposarcoma. According to the AJCC staging system, they were all stage IIB. No further adjuvant therapy was performed. Close follow-up after the surgery has been performed, and the patient has remained healthy without any evidence of recurrence or metastasis for 17 months after the surgery.     

Chondroblastoma of the Foot: 40 Cases From a Single Institution

Chondroblastoma (CB) of the foot is a rare lesion. The purpose of this report was to report a large series from a single institution to analyze clinical, radiographic, and histologic characteristics; treatment; and local recurrence.We present 40 patients (30 males, 10 females; mean age 25 years) diagnosed and treated for CB of the foot from 1975 to 2012. The mean follow-up visit was 55 months (range 7 months to 11 years). Clinical presentation, histology, imaging, surgical treatment, and local recurrence were evaluated. Males were more affected than females. The main symptom was pain (100%) accompanied by swelling (35%), with median duration of 12 months. The talus (50%) and calcaneus (37.5%) were the most affected bones. All patients underwent surgery: curettage (10 cases), curettage and bone graft (15 cases), curettage and cement (13 cases), wide resection (1 case), and Chopart amputation (1 case). Ten patients (25%) had secondary aneurysmal bone cyst. One patient had local recurrence after surgery.In conclusion, patients with CB of the foot are usually older than 20 years, and males are most affected. The hindfoot is the most affected area. Surgical treatment is required, and intralesional curettage and packing with cement or graft is curative in most cases. Local recurrence in foot is lower than in other locations.     

Intrathoracic rupture of an extraskeletal myxoid chondrosarcoma

Extraskeletal myxoid chondrosarcoma (EMC) is typically a slow-growing tumor with a prolonged clinical course. We describe a case of EMC that was complicated by intrathoracic rupture of the tumor and took an aggressive clinical course. A 64-year-old man with a precordial tumor suddenly began suffering from acute chest pain. Radiographic examination revealed a massive pleural effusion. Emergency surgery was performed but resulted in rupture of the pleural side of the tumor. The tumor was resected with the chest wall. The patient died 16 months after surgery owing to abdominal wall recurrence.     

Myxoid liposarcoma of the ankle: a case report

Myxoid liposarcoma occurs predominantly in the deep soft tissues of the extremities, and tends to metastasize to a wide range of soft tissue or bone locations. We report a case of myxoid liposarcoma arising in the ankle of a 19-year-old man. A diagnosis of myxoid liposarcoma was made by open biopsy, and a wide resection requiring plastic surgical reconstruction was performed. Cytogenetic analysis of the resected tumor exhibited a reciprocal translocation t(12;16)(q13;p11) as the sole anomaly, which is found in more than 90% of myxoid liposarcoma cases. The patient had no evidence of local recurrence or metastasis within 7 years of follow-up. Although myxoid liposarcoma is rare in the ankle, it should be considered in the differential diagnosis of a painless soft tissue mass in this region.     

Limb salvage operation using intraoperative extracorporeal autogenous irradiated bone and tendon graft for myxoid liposarcoma on dorsum of foot

Foot and ankle sarcomas are conventionally treated with amputation. In this article, we report a case of myxoid liposarcoma on the dorsum of the foot treated by limb salvage operation with intraoperative extracorporeal autogenous irradiated bone and tendon grafts. The patient was a 76-year-old woman with a soft tissue tumor beneath the extensor tendons with attachment to the tarsal and metatarsal bones. The histological diagnosis was myxoid liposarcoma. Wide margin was achieved by splitting the tarsal and metatarsal bones into dorsal and plantar parts. The dorsal part of the bones and tendons was isolated from the resected material, irradiated ex-vivo and re-implanted into the host's bones and tendons. No local recurrence was detected around the irradiated bones and tendons during the follow-up at 36 months. The functional rating was 74% according to the ISOLS rating system. This method may be a better procedure for limb salvage operation of sarcomas on the dorsum of the foot.