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.

Characterization of peripheral nerve sheath tumors with 3T proton MR spectroscopy.

BACKGROUND AND PURPOSE: The characterization of peripheral nerve sheath tumors is challenging. The purpose here was to investigate the diagnostic value of quantitative proton MR spectroscopy at 3T for the characterization of peripheral nerve sheath tumors as benign or malignant, compared with PET. MATERIALS AND METHODS: Twenty participants with 24 peripheral nerve sheath tumors underwent MR spectroscopy by use of a point-resolved sequence (TE, 135 ms). Six voxels were placed in 4 histologically proven malignant peripheral nerve sheath tumors and 22 voxels in 20 benign peripheral nerve sheath tumors (9 histologically proven, 11 with documented stability). The presence or absence of a trimethylamine signal was evaluated, the trimethylamine concentration estimated by use of phantom replacement methodology, and the trimethylamine fraction relative to Cr measured. MR spectroscopy results for benign and malignant peripheral nerve sheath tumors were compared by use of a Mann-Whitney test, and concordance or discordance with PET findings was recorded. RESULTS: In all malignant tumors and in 9 of 18 benign peripheral nerve sheath tumors, a trimethylamine peak was detected, offering the presence of trimethylamine as a sensitive (100%), but not specific (50%), marker of malignant disease. Trimethylamine concentrations (2.2 ± 2.8 vs 6.6 ± 5.8 institutional units; P < .049) and the trimethylamine fraction (27 ± 42 vs 88 ± 22%; P < .012) were lower in benign than malignant peripheral nerve sheath tumors. A trimethylamine fraction threshold of 50% resulted in 100% sensitivity (95% CI, 58.0%-100%) and 72.2% (95% CI, 59.5%-75%) specificity for distinguishing benign from malignant disease. MR spectroscopy and PET results were concordant in 12 of 16 cases, (2 false-positive results for MR spectroscopy and PET each). CONCLUSIONS: Quantitative measurement of trimethylamine concentration by use of MR spectroscopy is feasible in peripheral nerve sheath tumors and shows promise as a method for the differentiation of benign and malignant lesions. Trimethylamine presence within a peripheral nerve sheath tumor is a sensitive marker of malignant disease, but quantitative measurement of trimethylamine content is required to improve specificity.

Hairy cell leukemia presenting as a peripancreatic mass: cytomorphology and radiographic correlates.

Hairy cell leukemia (HCL) usually presents with peripheral cytopenias, diffuse marrow infiltration, and splenomegaly. This chronic lymphoproliferative disorder is not typically associated with lymphadenopathy or mass lesions. We report a case of HCL first treated by splenectomy, followed by several years of interferon therapy. Twenty-five years later, the patient presented with weight loss, fatigue, and a large PET-avid mass surrounding the head of the pancreas. Fine-needle aspiration was pursued to investigate the unusual and infiltrative appearance of the lesion, which was suggestive of another primary malignancy. Cytology smears showed discohesive lymphoid cells with round nuclei and delicate cytoplasmic projections. Flow cytometry confirmed the presence of a clonal B-cell population with bright expression of CD20 as well as CD25 and CD103, diagnostic of HCL. This is the first report of HCL presenting as a peripancreatic mass. The importance of correlation with radiology and clinical history is emphasized when evaluating such lesions.

Large cell transformation of mycosis fungoides on fine needle aspiration: an unusual case mimicking classical Hodgkin lymphoma.

Mycosis fungoides (MF), the most common type of primary cutaneous T-cell lymphoma, undergoes large cell transformation in only a minority of cases that present in patch stage. We describe a 52-year-old patient with a history of unilesional patch stage MF treated with localized radiotherapy. He had disease recurrence 5 years later and began psoralen plus ultraviolet A treatment with good response. After inguinal lymphadenopathy was detected on physical examination, a follow-up PET scan showed a fluorodeoxyglucose-avid inguinal node, which was evaluated by fine needle aspiration. Cytomorphology showed numerous Reed-Sternberg-like cells, highly suspicious for Hodgkin lymphoma. Histologic sections revealed isolated sinusoidal involvement by pleomorphic CD30-positive tumor cells, diagnostic of large cell transformation of MF in light of clinical history. The cells were also positive for CD15 and CD4. This case emphasizes morphologic features of an entity with limited description in the cytology literature and adds to the growing number of reported CD15-positive T-cell lymphomas.

Peripheral nerve surgery: the role of high-resolution MR neurography.

High-resolution MRN is becoming increasingly available due to recent technical advancements, including higher magnetic field strengths (eg, 3T), 3D image acquisition, evolution of novel fat-suppression methods, and improved coil design. This review describes the MRN techniques for obtaining high-quality images of the peripheral nerves and their small branches and imaging findings in normal as well as injured nerves with relevant intraoperative correlations. Various microsurgical techniques in peripheral nerves, such as neurolysis, nerve repairs by using nerve grafts, and conduits are discussed, and MRN findings of surgically treated nerves are demonstrated.