M. Francisca Garijo

Extravulvar subcutaneous cellular angiofibroma

Cellular angiofibroma is a rare distinctive mesenchymal neoplasm of the vulva or perineal region. We report here one unique extravulvar case. A 43‐year‐old woman presented with an asymptomatic tumor, 7 cm in diameter, located in the subcutaneous tissue of the chest below the left submammary sulcus. Histologically, the lesion was composed of uniform spindled stroma cells, numerous thick‐walled vessels, and scarce mature adipocytes. An additional feature was the presence of prominent perivascular lymphoid aggregates. The stromal cells were positive for vimentin and negative for CD34 and muscle, epithelial, myoepithelial, or neural markers. Although nasopharyngeal angiofibroma or the group of acral angiofibromas have a concurrent heading, cellular angiofibroma should not be mistaken with them. Differential diagnosis of this distinctive tumor especially includes aggressive angiomyxoma, angiomyofibroblastoma, superficial angiomyxoma, vascular myxolipoma, and other tumors with spindle cells reminiscent of those in angiofibroma.

Pagetoid dyskeratosis of the prepuce. An incidental histologic finding resembling extramammary Paget's disease.

Background: Pale cells resembling those of pagets disease have been seen as an incidental finding within the epidermis in a variety of benign papules most commonly located in intertriginous areas. This lesion, called pagetoid dyskeratosis, is considered a reactive process in which a small part of the normal population of keratinocytes is induced to proliferate. Among the inductors friction is suspected. As far as we know, these cells have not been reported in the penis.

Pilonidal sinus of the penis. A report of two cases, one of them associated with actinomycosis

Pilonidal sinus is a long‐standing chronic inflammatory condition which occurs most commonly in the sacrococcygeal area and it is rare in the penis. Two cases of penile pilonidal sinus in patients aged 25 and 28 years are reported and the literature is reviewed. The lesion is acquired and has to be distinguished from balanoposthitis, epidermal cyst and carcinoma. For pilonidal sinus to occur at this site, it is hypothesized that the coronal sulcus acts as a cleft where hair may accumulate and be driven into the shaft and prepuce by the natural movement between these two surfaces. One of our cases was associated with actinomycosis. Actinomycosis associated with pilonidal sinus of the penis is extremely rare and there are only two previous reports. It is suggested that pilonidal sinus may have been predisposed to the infection with the actinomyces organisms. Information stating that penile pilonidal sinus and actinomycosis may occur simultaneously is necessary to accomplish an early diagnosis.

Predictors of axillary lymph node metastases in patients with invasive breast carcinoma by a combination of classical and biological prognostic factors.

The presence of axillary lymph node metastases (ALNMs) is the most important prognostic factor in breast carcinoma. If ALNMs were predictable without performing axillary lymph node dissection (ALND), this procedure would not be necessary in selected patients. Using a combination of some of the new biological markers with the classical ones, our objective was I) to identify the best set of predictors of ALNMs, and II) to define predictive models with either high or low probability of ALNMs. We studied 102 patients with invasive breast carcinoma. All patients underwent ALND, and at least 10 axillary lymph nodes per case were obtained. In the primary tumour we evaluated size, histological subtype and grade, lymphatic/vascular invasion and margin. Hormone receptor status, MIB1 index, microvessel density, c-erbB-2 and cathepsin D expression were assessed by immunohistochemistry, and DNA ploidy and S-phase by flow cytometry. Risk factors for ALNMs were estimated by nonlinear logistic regression analysis. The best predictors of ALNMs were: tumour size > 2 cm [OR 6.45, 95% confidence interval (CI) 21.74 to 1.91], presence of lymphatic/vascular invasion [OR 4.95, CI (14.50 to 1.69)], infiltrative margin [OR 9.87 CI (37.44 to 2.60)] and high MIB-1 index [OR 8.39, CI (33.47 to 2.10)]. Two subsets had a very high risk of ALNMs: I) tumour size > 2 cm, with lymphatic/vascular invasion and infiltrative margin; 26 (89.66%) of 29 patients of this subgroup had ALNMs, and (II) tumour size > 2 cm, with lymphatic/vascular and high MIB1 index.; eight of the nine (89%) patients of this subgroup had ALNMs. We could also identify a two-variable model with a very low risk of ALNMs constituted by tumour with circumscribed margin and low MIB-1 index. Of the 19 patients showing these features, only 1 (5.26%) had ALNMs. Therefore, pathological features of the primary tumour can help to assess the risk for ALNM in invasive breast carcinoma. Such risk assessment might avoid regional surgical overtreatment.

Subcutaneous ossifying lipoma: case report and review of the literature

Background:  Lipomas can undergo a variety of changes, one of which is ossification. Ossifying lipoma (OL), independent of bone tissue, is rare. No case has been reported with this heading in the dermatopathological literature.

Primary Acute Torsion of the Vermiform Appendix

A case of primary acute appendiceal torsion in a 6-year-old boy with symptoms suggestive of acute appendicitis is presented. The appendix was abnormally long, measuring 13.5 cm in length. Although appendicitis is the most common intra-abdominal surgical emergency, there are few descriptions of primary acute appendiceal torsion, a rare cause of an acute abdomen. A review of the English language literature disclosed 19 reports, including the present, with 11 pediatric cases. The site of torsion occurs most frequently 1 cm or more from the appendiceal base. Rotation varies from 270 degrees to 1080 degrees with a mean of 580 degrees. The direction of the rotation is more frequently anticlockwise. Appendix is most commonly described as lying free or pelvic. In children the mean age is 9.1 years, the range 3-16 years, and the male-to-female ratio 4.5:1.

Pagetoid dyskeratosis of the nipple epidermis: an incidental finding mimicking Paget's disease of the nipple†

Pagetoid dyskeratosis (PD) is considered a selective keratinocytic response in which a small part of the normal population of pale keratinocytes is induced to proliferate. PD has been found incidentally in the squamous epithelium of the skin and mucosas in various locations, but not in the nipple. In cases in which PD cells are conspicuous, there is the danger of overdiagnosis. In a retrospective study, we describe the location and incidence of PD and other pale cells in the nipple epidermis, in 288 mastectomy specimens from women operated on for breast carcinoma, in situ or infiltrating, selected consecutively from our histopathologic files. In addition to the conventional histologic methods an immunohistochemical study was performed in selected cases. PD was found in 184 (63.9%) cases and was a prominent finding in 37 (12.8%) cases. Toker cells (TCs) were identified by standard light microscopy in 24 (8.3%) nipples. Paget carcinoma cells (PCCs) were found in 12 (4.2%) cases, and in 9 (3.1%) they were an incidental finding. The immunohistochemical profile of each type of pale cells was as follows: PD cells, EMA‐,LMWCK‐,CK7‐,HMWCK+, CEA−, HER2/neu protein−, HMB45−, HPV−; TCs, EMA+, LMWCK+, CK7+, HMWCK−, CEA−, HER2/neu protein−, HMB45−, HPV−; PCCs, EMA+, LMWCK+, CK7+, HMWCK−, CEA+, HER2/neu protein+, HMB45−, HPV−. In conclusion, friction may be the stimulus for the appearance of PD cells. PD cells must be distinguished from TCs, PCCs, clear cells of Bowens disease, pagetoid melanoma cells, cells of clear cell papulosis, koilocytes, artifactual clear cells, and glycogen‐rich squamous cells. A combination of immunohistochemical markers is useful for this distinction; however, routine histologic study is usually adequate for recognizing PD. Pathologists should be familiar with the histologic features of PD in the nipple epidermis to avoid misdiagnosis.

Soft-tissue angiomatosis in adulthood: a case in the forearm showing a prominent myxoid adipose tissue component mimicking liposarcoma.

Soft‐tissue angiomatosis is a rare condition characterized by diffuse proliferation of benign vascular structures, accompanied by mature adipose tissue, which affect a large segment of the body in a contiguous fashion. Most patients present in childhood or adolescence. We report a case of large soft‐tissue angiomatosis, located in the left forearm of a 65‐year‐old man, which showed extensive intramuscular and intermuscular infiltration, and embracement of the radius and the main vascular and neural bundles of the forearm. Due to the deep location, large size, ill‐defined limits and non‐homogeneous composition with areas suggestive of an adipose component in the radiological examination, the lesion raised the possibility clinically of a liposarcoma. The myxoid adipose tissue component of the lesion, a feature previously undescribed, and the predominance of small, capillary‐sized vessels were considered concordant with the clinical diagnosis. This presentation is rare. The features of the lesion made complete surgical excision impossible and amputation of the extremity was considered the best treatment. The main differential diagnoses include infiltrating lipoma, myxolipoma, angiomyxolipoma, angiolipoma, intramuscular angioma, liposarcoma and low‐grade myxofibrosarcoma. In conclusion, angiomatosis might simulate liposarcoma, particularly if the mass is large, heterogeneous, myxoid and appears later in life.

Pagetoid dyskeratosis of the lips

Pagetoid dyskeratosis is an incidental finding in a variety of lesions of the skin and squamous mucosa. The lesion is considered a selective keratinocytic response in which a small part of the normal population of keratinocytes is induced to proliferate in response to friction. As far as we know, pagetoid dyskeratosis has not been reported in the lips. In this article, we describe the location of the lesion in the lips and its incidence in a group of 90 unselected patients who underwent biopsy or were surgically treated for diverse labial lesions. Histochemical staining and immunohistochemical studies were performed in selected cases. Pagetoid dyskeratosis was found in 38 cases (42.2%) but only in 6 cases (6.7%) the lesion was conspicuous. There was no significant difference between the upper and the lower lip in terms of incidence of the lesion. Labial pagetoid dyskeratosis was more frequent in younger patients (46.7 ± 25.0 versus 58.5 ± 20.5;p < 0.05) and in women (&khgr; 2 = 3.89;p < 0.05). Pagetoid cells were more common in suprabasal location and in the labial mucosa. These cells showed positivity for high-molecular weight cytokeratin and negative reaction for low-molecular weight cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, and human papilloma virus. The immunohistochemical profile is different from the surrounding keratinocytes, indicating premature keratinization. The main differential diagnoses include white sponge nevus, leukoedema, oral koilocytoses, hairy leukoplakia, pagetoid squamous cell carcinoma in situ, and extramammary Pagets disease of the oral mucosa. The morphologic features of dyskeratotic pagetoid cells are distinctive and easily recognized as an incidental finding, thus preventing confusion with other important entities including an intraepidermal tumor.

Anticipated diagnosis of left atrial myxoma following histological investigation of limb embolectomy specimens: a report of two cases.

Nonfamilial myxoma occurs as a random event. The tumor is rare and can mimic other diseases. Cardiac myxomas should always be considered as a source of embolization, which need meticulous investigation and prompt indication of surgical resection. Tumors with a villous surface are prone to embolize. We report two cases of cardiac myxoma presenting as acute ischemia of one or two limbs due to embolic phenomena. The patients were females aged 55 and 37 years. Histological study of emboli taken from obstructed limb arteries in the two patients showed a picture indicating systemic embolization of a cardiac myxoma. The embolic tissue fragments showed the gross characteristics (i.e. villous surface) of the cardiac tumor. Further echocardiography and surgical removal confirmed the cardiac myxoma. Immunohistochemical study of embolectomy material disclosed strong reactivity of the tumor cells for calretinin. The histological examination of the embolectomy material can anticipate the cardiac lesion and its gross features. Calretinin is a useful marker in the differential diagnosis of cardiac myxoma with a myxoid thrombus. The necessity of histological examination of the embolectomy material is stressed.