A Rare Case of Primary Cutaneous Gamma-Delta T-cell Lymphoma with Aberrant B-cell Marker Expression.

ABSTRACT: Primary cutaneous gamma-delta T-cell lymphoma (PCGDTL) is a rare and diagnostically challenging primary skin lymphoma. We present a case of a 78-year-old otherwise healthy man who developed nonhealing nodules on his right posterior calf. Initial biopsy showed a dense, atypical, lymphoid infiltrate with gamma-delta and cytotoxic T-cell immunophenotypes. The diagnosis of PCGDTL was rendered; however, concurrent flow cytometry revealed expression of aberrant B-cell markers, including CD19 and cytoplasmic CD79a. Subsequent immunohistochemical studies corroborated this result. We report the extremely rare phenomenon of aberrant B-cell marker expression in PCGDTL, the first formally reported case to our knowledge.

Employing immunohistochemical staining to labial minor salivary gland biopsies from patients with Sjogren's syndrome increases diagnostic certainty.

BACKGROUND: Sjogren's syndrome (SjS) is an autoimmune disease characterized clinically by dry eyes and dry mouth, and histopathologically by lymphocytic infiltrates in the salivary glands. Labial minor salivary gland biopsy (MSGB) is a major diagnostic test for SjS, deemed positive by a focus score of ≥1, meaning that ≥50 lymphocytes were found in 4 mm2 tissue on hematoxylin and eosin (H&E)-stained slides. The diagnosis can be challenging, and the above diagnostic criteria has low and variable sensitivity. METHODS: We performed a retrospective study on MSGBs done for possible SjS. We compared the percent of MSGBs which met the histologic criteria by H&E stain alone and that with the addition of CD45, CD3, and CD20 immunohistochemical (IHC) staining for these patients. A total of 45 cases with complete data were analyzed. RESULTS: Thirty-five of the 45 patients had the diagnosis of Sjogren's syndrome (SjS+) based on ACR criteria. However, based on H&E staining alone, only 22/35 cases (63%) met the histologic criteria. After adding IHC staining with CD45, CD3, and CD20 to MSGBs of SjS + patients, 29/35 (83%) cases met the histological criteria for SjS. All MSGBs from patients without SjS had no significant lymphocyte infiltrate on either H&E or IHC stains. CONCLUSIONS: Immunohistochemical better identifies lymphocytic infiltrates in MSGB and increases diagnostic certainty. Due to high cost, their use should be restricted to cases where there is high clinical suspicion of SjS and negative H&E evaluation alone, or if the diagnosis is uncertain.

Proliferating pilomatricoma in a 9-year-old girl.

Proliferating pilomatricoma is a rare, benign tumor of hair matrix origin that rarely occurs in children. We report the case of a 9-year-old girl with a rapidly growing, proliferating pilomatricoma located on the glabella. The lesion was embolized and surgically excised, with histopathological examination of the tissue confirming the diagnosis of proliferating pilomatricoma.

Angiolymphoid hyperplasia with eosinophilia treated with Mohs micrographic surgery.

A 60-year-old healthy man presented with several enlarging, tender, spontaneously bleeding, and episodically pruritic nodules on his ear. Five agminated pink-red papulonodules of the superior postauricular sulcus were noted on examination. Pathological examination revealed a lobular dermal vascular proliferation with plump endothelial cells protruding into the lumen in a hobnail pattern, along with a dense perivascular inflammatory infiltrate composed of plasma cells, lymphocytes, and numerous eosinophils. The diagnosis of angiolymphoid hyperplasia with eosinophilia was confirmed. After discussing treatment modalities, the patient opted for Mohs micrographic surgery (MMS). Three stages of MMS were able to remove all large vessel involvement and clear the peripheral margins, but the tumor had a complex branching pattern of growth in the deep bed of the wound with numerous tiny foci remaining. Owing to risk of disfigurement, no further excision was undertaken. The area was reconstructed with a temporalis fascia flap and a full-thickness skin graft. Despite remaining microscopic disease, the patient remained without recurrence or symptoms at one year of follow up.

Addressing the average lifespan of skin diseases is critical to good patient care.

Dermatology patients routinely ask how long their skin condition may last, yet this critical aspect of their care has not been emphasized in the literature. When a given diagnosis may be self-limited, it is essential that clinicians meet patient expectations by properly discussing the possible time course for resolution. Furthermore, being aware of, and prioritizing the knowledge of the duration of a skin disease can help limit continued exposure to side effects of prescribed treatments once the condition may have self-resolved or remitted.

Radiation-induced circumscribed superficial morphea after brachytherapy for endometrial adenocarcinoma.

Radiation-induced morphea (RIM) is a rare and underrecognized complication of radiation therapy that most commonly occurs in women after treatment for breast cancer. Although not fully understood, RIM is hypothesized to arise from an increase in cytokines that stimulate collagen production and extracellular matrix formation. Most documented cases of RIM occur 1 year after radiation therapy and are localized to areas that were treated for breast cancer. We report on a case of a female patient with stage IB endometrial adenocarcinoma who was treated with 24 Gray of adjuvant brachytherapy. The patient developed a diffuse morpheaform, pruritic eruption only at distant sites from the brachytherapy treatment field. Although treatment for RIM is generally unsatisfactory, our patient experienced improvement in the pruritus and a regression of the lesions while applying topical 0.1% tacrolimus ointment and 0.1% triamcinolone creme. An early diagnosis of RIM can prevent extensive workup, guide treatment, and improve quality of life for patients.

Development of 3-dimensional transperineal ultrasound for image guided radiation therapy of the prostate: Early evaluations of feasibility and use for inter- and intrafractional prostate localization.

PURPOSE: Transperineal ultrasound (TPUS) allows for continuous imaging of the prostate gland, but the accuracy of TPUS has not been rigorously studied. We determined the feasibility of prostate imaging with TPUS and subsequently compared prostate localization with TPUS and computed tomography (CT). METHODS AND MATERIALS: We completed 2 sequential evaluations of TPUS. The feasibility study included 15 men with localized prostate cancer and tested if TPUS adequately imaged the prostate. Image qualities of the prostate and adjacent normal structures were measured. The subsequent study included 17 men who at the time of initial radiation treatment planning and in 3 subsequent sessions had CT and TPUS imaging performed and compared. RESULTS: Feasibility of TPUS was confirmed in the first trial. After expected hardware and software modifications were completed, TPUS provided near complete edge definition of the prostate in the final 5 patients in the feasibility trial. The second study allowed for the comparison of 30 image sets. The differences between TPUS and CT in each direction (mean + standard deviation) were found to be 0.06 ± 2.86 mm (anteroposterior), 0.49 ± 3.49 mm (superoinferior), and 0.63 ± 3.27 mm (left-right), with no significant difference between the 2 modalities (all P > .32). The Euclidean distance variance using the 2 techniques was 5.25 ± 1.79 mm, which was significantly different. CONCLUSIONS: TPUS provides good imaging of the prostate gland. We noted excellent correlation in gland localization when TPUS is compared with CT scans when comparing routine 3-dimensional positional data. Euclidean distance variation suggests the potential that summation of small errors may in fact lead to significant differences in actual gland positional certainty. The reported difference is within the range of standard planning target volume expansion however requires additional evaluation.

Adrenal incidentalomas: are they being worked up appropriately?

INTRODUCTION: Adrenal incidentalomas are defined as masses picked up on imaging studies that were done for apparently different reasons. With frequent use of imaging modalities, incidental adrenal masses are commonly encountered in clinical practice. Guidelines are currently available for the diagnosis and management of adrenal incidentalomas, but the appropriateness of initial work-up and subsequent follow-up of incidental adrenal masses in the community hospital setting is unknown. OBJECTIVE: We studied the appropriateness of initial work-up and follow-up of incidental adrenal masses discovered on abdominal computerized tomography (CT). METHODS: In our retrospective study, we reviewed sequential CT scans of the abdomen performed in the month of January 2010 at a community hospital. Once patients with one or more adrenal masses were identified, outpatient charts for initial biochemical testing and follow-up imaging were obtained either through directly accessing the electronic medical records or through contacting primary care physician's offices. Patient charts were reviewed to assess the data for the next 2 years following the discovery of an adrenal abnormality. RESULTS: Twenty adrenal masses were incidentally discovered on 723 abdominal CTs performed within the month of January 2010 resulting in an overall incidence of 2.76%. Of the patients with incidentally discovered adrenal masses, appropriate biochemical and follow-up imaging were only performed in patients referred to an endocrinologist (2 of 20 patients). Thirty percent of patients with incidental masses received a repeat CT scan for non-adrenal reasons, and no change in the mass size was noted. CONCLUSION: Despite published guidelines, the initial work-up and follow-up of patients with an incidentally discovered adrenal mass is unsatisfactory. There is a desperate need for education of providers regarding appropriate work-up of incidental adrenal masses.

Hypoglycemia, with or without insulin therapy, is associated with increased mortality among hospitalized patients.

OBJECTIVE: Hypoglycemia is associated with increased mortality in hospitalized patients. We investigated the relationship between spontaneous hypoglycemia versus insulin-associated hypoglycemia and mortality in hospitalized patients. RESEARCH DESIGN AND METHODS: Data for this retrospective cohort study were obtained from electronic databases of patients admitted between 1 April 2008 and 30 November 2010. Patients with one or more blood glucose values ≤50 mg/dL on point-of-care glucose testing were considered hypoglycemic. Patients treated with insulin were assumed to have insulin-associated hypoglycemia. Age-, sex-, and race-matched patients with all blood glucose values >70 mg/dL were selected as controls. The Charlson comorbidity index (CCI) was used to control for severity of illness. RESULTS: There were four groups: 1) noninsulin-treated hypoglycemia (NTH) (n = 135), 2) insulin-treated hypoglycemia (ITH) (n = 961), 3) noninsulin-treated control (NTC) (n = 1,058), and 4) insulin-treated control (ITC) (n = 736). Mortality was higher in the ITH group compared with the ITC group (20.3 vs. 4.5%, P < 0.0001), with a relatively higher CCI (1.8 vs. 1.5%, P < 0.0001), but much higher in the NTH group compared with the NTC group (34.5 vs. 1.1%, P < 0.0001), with much higher CCI (2.4 vs. 1.1%, P < 0.0001). Mortality was higher in the NTH group compared with the ITH group (P < 0.0001) but lower in the NTC group compared with the ITC group (P < 0.0001). After controlling for age, sex, CCI, and admission to the intensive care unit, insulin treatment was associated with a lower mortality among the hypoglycemic patients; hazard ratio of death in the ITH group relative to the NTH group was 0.34 (95% CI 0.25-0.47, P < 0.0001). CONCLUSIONS: Insulin-associated and spontaneous hypoglycemia are associated with increased mortality among hospitalized patients.