Elastic fiber pattern in scleroderma/morphea

Background: Scleroderma/morphea is characterized by expansion of the dermis with thickened collagen bundles and loss of CD34⁺ dermal dendrocytes. Variable elastic fiber changes have been described, but to our knowledge, no systematic study of the elastic fiber pattern correlated with CD34 expression has been reported.
Methods: To better define the typical elastic fiber morphology, we examined seven cases of normal skin and 28 cases of scleroderma/morphea ranging from inflammatory to sclerosing stages. All but four biopsies were submitted with a clinical impression of either scleroderma or morphea. CD34 immunohistochemistry was performed on 26 biopsies with available tissue.
Results: Elastic van Gieson stain showed preservation of elastic fibers in all cases. In addition, straightening with parallel orientation and compression between thickened collagen bundles was frequently present and was graded as limited in 46% and diffuse in 54% of cases. The extent of elastic fiber alteration correlated with the degree of sclerosis. A variable loss of CD34⁺ dermal dendritic cells was seen in all cases.
Conclusion: This study confirms the preservation and frequent presence of parallel, straightened and compressed elastic fibers in scleroderma/morphea and suggests that the elastic fiber pattern, in addition to CD34 immunohistochemistry, may serve as a useful diagnostic adjunct.     

Unstable recent intracapsular femoral neck fractures in young adults: Osteosynthesis and primary valgus osteotomy using broad dynamic compression plate

Background:

Displaced intracapsular femoral neck fractures continue to be a difficult problem to treat. Various treatment modalities and their modifications have been proposed to improve the outcome. Osteosynthesis and primary valgus angulation osteotomy is one of them. Technique and outcome in a consecutive series of recent intracapsular femoral neck fractures in young adults, from a single center, is presented.

Materials and Methods:

Fifty-five patients of recent (<3 weeks old) displaced intracapsular fracture neck femur (Garden III and IV, Pauwels III, with or without comminution) in the age group 20-50 years (mean 35.4±10.4 years) were subjected to osteosynthesis and primary valgus intertrochanteric osteotomy using contoured broad dynamic compression plate (DCP). The patients were followed up from two to six years (mean 4.6 years).

Results:

Fifty-one fractures united by six months of the index procedure (92.7% union range). Avascular necrosis (AVN) developed in six patients (11%). The other complications were shortening (six), coxa vara (two), infection (two) and delayed union at osteotomy site (one). Excellent results were achieved in 48, good/fair in four and poor in three patients.

Conclusion:

Osteosynthesis with cancellous screw and primary valgus intertrochanteric osteotomy stabilized by a contoured broad DCP is a simple, easy to perform, biological treatment. Failure in a particular case can be treated with any appropriate second procedure.

Level of Evidence:

IV     

Adult T-cell leukemia/lymphoma with follicular mucinosis: an unusual histopathological finding and a commentary

Follicular mucinosis is currently recognized as a histopathological finding characterized by the accumulation of mucin within follicular epithelium and is commonly associated with follicular mycosis fungoides (MF). We report the finding of follicular mucinosis in a cutaneous nodule of human T-lymphotropic virus type 1 (HTLV-1) associated adult T-cell leukemia/lymphoma (ATLL). The patient was a 69-year-old female of Caribbean descent with a history of ATLL who presented with erythematous nodules on the chest and abdomen. Histopathologic examination showed a pan-dermal infiltrate of medium-to-large sized atypical lymphocytes extending into follicular epithelium where they associated with large mucin deposits. Immunohistochemical stains showed that the atypical lymphocytes were positive for CD3, CD4 and CD25 and negative for CD30. Cutaneous lesions of ATLL, which often present histopathologically as an epidermotropic lymphoma with Pautrier-type collections, are often difficult to distinguish from MF. Until recently, lymphoma-associated follicular mucinosis seemed specific to MF and Sézary syndrome (SS), being reported only once in a lesion of ATLL. We report a second case of ATLL-associated follicular mucinosis to increase awareness of this possible association, and briefly review the literature of follicular mucinosis-associated hematologic malignancies, ultimately cautioning against the interpretation of all cutaneous lymphoma-related follicular mucinosis as MF/SS.     

Combined intraepidermal neuroendocrine (Merkel cell) and squamous cell carcinoma in situ with CM2B4 negativity and p53 overexpression(*)

Primary cutaneous neuroendocrine carcinoma, also known as Merkel cell carcinoma (MCC), usually presents as a dermal and/or subcutaneous tumor. Rarely, it is confined to the epidermis or adnexal epithelium [MCC in situ (MCCIS)]. Little is known about the spectrum of features and biology of MCCIS. Herein, we report a case of MCCIS arising on the cheek of a 77-year-old Caucasian male, which was associated with squamous cell carcinoma in situ. The tumor cells of both the neuroendocrine and squamous components prominently involved adnexal structures but did not invade the dermis. The tumor cells with neuroendocrine features were immunoreactive for cytokeratin-20, chromogranin and synaptophysin. They also expressed p53 but were non-reactive with the monoclonal antibody CM2B4. Lack of labeling for CM2B4 is in keeping with prior observations of combined squamous and MCC. Our findings support the concept of a distinct subtype of virus-independent cutaneous neuroendocrine carcinoma that differs from conventional MCC. The observed overexpression of p53 suggests that the development of this tumor type may be related to chronic ultraviolet damage.     

Local control after craniospinal irradiation,intensity‐modulated radiotherapy boost,and chemotherapy in childhood medulloblastoma

BACKGROUND:

The current study was conducted to determine whether the use of cochlear‐sparing intensity‐modulated radiotherapy (IMRT) boost results in excess local failures in children with medulloblastoma.

METHODS:

Fifty children with a median age of 7.8 years underwent resection, craniospinal irradiation (CSI), IMRT posterior fossa (PF) and/or tumor bed (TB) boost, and cisplatin‐based chemotherapy for medulloblastoma. For standard‐risk patients, the CSI dose was 18 to 23.4 grays (Gy) and was followed either by an IMRT PF boost to 36 Gy and a TB boost of 54 to 55.8 Gy (n = 29) or by an IMRT TB boost to 55.8 Gy (n = 4). For high‐risk patients, the CSI dose was 36 to 39.6 Gy followed by an IMRT PF boost to 54 to 55.8 Gy (n = 8), an IMRT PF boost to 45 Gy and a TB boost to 55.8 Gy (n = 2), or an IMRT TB boost to 55.8 Gy (n = 7). For the TB boost, a 2‐cm margin around the surgical bed was treated in most patients.

RESULTS:

The 5‐year overall and progression‐free survival rates (±standard deviation) were 72% ± 6.6% and 68.3% ± 6.8%, respectively, for all patients; 77.8% ± 7.4% and 75.1% ± 7.6%, respectively, for standard‐risk patients; and 60.8% ± 12.8% and 55.4% ± 12.8%, respectively, for high‐risk patients. The 5‐year PF control rate was 90.5% ± 4.6%. TB failures occurred in 3 patients (including 2 patients who had distant failure), whereas an isolated non‐TB PF failure occurred in 1 patient.

CONCLUSIONS:

The use of IMRT was associated with excellent local control and did not result in excess PF failures outside of the TB. Cancer 2011. © 2010 American Cancer Society.     

Antimycobacterial Evaluation of Fifteen Medicinal Plants in South Africa

Fifteen plant species were collected from the Nelspruit Botanical Garden based on a list of plants provided by Phytomedicine Programme at the University of Pretoria and their ethnopharmacological information. Hexane, dichloromethane (DCM), acetone and methanolic extracts were screened for antimycobacterial activity against Mycobacterium smegmatis. The acetone extract of Milletia stulhimannii was the most active, showing activity against Mycobacterium smegmatis with minimum inhibitory concentration (MIC) value of 0.13 mg/ml. Acetone extracts for all plants had lower MIC values ranging between 0.11–1.25 mg/ml against M. smegmatis. Milletia stulhimannii, Albizia gummifera, Xanthocercis zambesiaca and Barringtonia racemosa have shown great potential as anti-tuberculosis agents. They were active against M. smegmatis with average MIC values of acetone extracts of 0.13 mg/ml.