Giant Rhinophyma.

A 42-year-old woman presented with a clinically obvious giant rhinophyma. The protocol of the unit handling the case mandated that all lesions amenable to biopsy should have a core biopsy before any definitive surgery, but the unnecessary biopsy was not representative and suggested an incorrect diagnosis of perifollicular fibroma. The lesion was excised. The sections showed dilated hair follicle pores on the skin surface, squamous lined hair follicles plugged with keratin, prominent sebaceous glands, perifollicular inflammation without granulomas, intradermal budding of hair follicle basal cells, and extensive hypocellular, mildly edematous fibrous tissue with slightly dilated, thin-walled vessels and a few chronic inflammatory cells. These fully representative sections confirmed the diagnosis of giant rhinophyma, suggesting that preoperative core biopsies of this condition are unnecessary and may be misleading.

Pleural malignant mesothelioma versus pleuropulmonary synovial sarcoma: a clinicopathological study of 22 cases with molecular analysis and survival data.

The aim of this study was to carry out a comparative analysis by transducin-like enhancer of split 1 (TLE1) immunohistochemistry and molecular analysis of SYT-SSX, for 16 pleural predominantly sarcomatoid mesotheliomas and six cases of pleuropulmonary synovial sarcoma (five pleural in distribution only, with one case of a predominantly subpleural upper lobe synovial sarcoma), all of which were solely or predominantly monophasic. Our comparison included survival and some clinical data. We consider that the following points emerged from this study.

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club: Peritoneal Lipofuscinosis and Deciduosis in Pregnancy.

Peritoneal lipofuscinosis is a very rarely recognized condition occurring during pregnancy characterized by brown pigmentation of the omentum and peritoneum, a decidual reaction and benign mesothelial cells. The iron negative pigment, which is likely to be confused with hemosiderin in the hematoxylin and eosin stain, is lipofuscin. The seminar case, apparently the third published, arose in a 37-year-old woman who presented in October 2015 at 24 weeks pregnancy with abdominal pain. Investigations revealed a ruptured left ovarian cyst and rising serum carcinoembryonic antige levels. At laparotomy, there was no free intraperitoneal blood but the omentum and uterine serosa were black. Histology showed lipofuscinosis and a decidual reaction. The patient delivered a normal baby in February 2016 and was clinically well after delivery. A left ovarian endometriotic cyst was removed in February 2017. The patient made a good recovery with no clinically apparent symptoms from the liposuscinosis. We postulate that the endometriotic cyst had ruptured and released blood into the peritoneal cavity in 2015. The iron from the red cells breakdown was then rapidly resorbed because of the pregnancy requirements for iron, leaving lipofuscin in peritoneal macrophages.

Selected Case From the Arkadi M. Rywlin International Pathology Slide: Massive Localized Lymphedema in Morbid Obesity Complicated by a Nonspecific Subcutaneous Abscess.

A 59-year-old morbidly obese female developed an ulcerated, slowly growing, 25 cm, subcutaneous, pendulous mass in the right groin which became infected and was excised in January 2014. The excised skin and subcutaneous fat weighed 1901 g. The skin exhibited a cobblestone appearance, the dermis was thickened and edematous, and the subcutaneous fat was traversed by fibrous septae. Histologically, there were dilated, thin-walled vessels, perivascular chronic inflammatory cells, slightly atypical macrophages, and expanded subcutaneous fibrous septae surrounding degenerating adipocytes resembling lipoblasts. Nearly all the Club members agreed with the submitted diagnosis of massive localized lymphedema in morbid obesity. Postoperatively, the wound healed well, there has been no recurrence or further infection as of July 2015, but the patient suffers from diabetes and congestive cardiac, the more common complications of morbid obesity.

Selected Case From the Arkadi M. Rywlin International Pathology Slide Club: Polypoid Endometriosis in the Pouch of Douglas in a Perimenopausal Woman.

A 50-year-old female had a hysterectomy and bilateral salpingo-oophorectomy for adenomyosis, uterine leiomyomas, ovarian and cervical endometriosis. Nine months later, organ imaging revealed a 43×74×52 mm, apparently malignant extraperitoneal mass lying between the vagina and rectum. The patient had been receiving a progestogen. Preoperative needle biopsies were interpreted as recurrent endometriosis. The mass was easily excised and sections were interpreted as polypoid endometriosis with a decidual reaction and foci of necrosis. Slides circulated to the club were from the excised specimen. The majority agreed with this diagnosis although a minority favored a Mullerian adenosarcoma. One club member commented that pseudosarcomatous change, including periglandular stromal cuffing and condensation, may occur in patients on hormones, especially tamoxifen. The patient was well with no recurrence 15 months after surgery.

Selected case from the Arkadi M. Rywlin International Pathology Slide Club: aggressive angiomyxoma, left labial region in a postmenopausal woman.

Club members unanimously agreed with the diagnosis of an unencapsulated 8×2.5×3.6 cm aggressive angiomyxoma, which was invading the voluntary muscles of the pelvic floor beneath the left labium of a female aged 65. The tumor consisted of histologically bland, round, stellate to fusiform cells set in a myxocollagenous matrix with occasional mast cells, a few extravasated red cells, and prominent blood vessels varying from thin-walled capillaries 7 µm in diameter to larger thick-walled vessels >250 µm in diameter. The tumor cells stained positively for estrogen and progesterone receptors, vimentin, and desmin. A stain for the nuclear transcription factor HMGA2, which is emerging as a useful and relatively specific marker for aggressive angiomyxoma, was not performed. The tumor had not recurred 4 years after the surgical excision. One member commented that virtually all lesions diagnosed as aggressive angiomyxomas in superficial locations turn out to be either fibroepithelial stromal polyps or superficial angiomyxomas. None of the club had seen a metastasizing aggressive angiomyxoma nor had they any experience with gonadotropin hormone-releasing and luteinizing hormone-releasing agonists therapy, which have been reported to cause tumor regression.

Selected case from the Arkadi M. Rywlin International Pathology Slide Seminar: sclerotic (fibroma-like) lipoma, dorsum of right hand.

Approximately 26 sclerotic lipomas have been reported since Zelger and colleagues' 1997 report of 5 cases involving the scalp and hands. In 2006, Laskin and colleagues published 21 sclerotic lipomas in a study restricted to tumors of the hands and feet. Patients' ages ranged from 7 to 72 years (mean and median 39 y); there were 12 males and 9 females; the masses, which were mostly asymptomatic and solitary, ranged from 0.6 to 2.2 cm (median 1.2 cm, mean 1.3 cm) and involved fingers (N=17), hands or wrists (N=3), and toes (N=1). Microscopically, lesions were well circumscribed, of low cellularity, and consisted of cytologically bland spindle and stellate-shaped cells with a minor component of randomly dispersed adipocytes embedded in a collagenous to myxocollagenous stroma. Eight tumors were fibroma-like with dense fibrosclerotic stroma and inconspicuous blood vessels. One tumor exhibited features of spindle cell/pleomorphic lipoma, whereas another displayed a vague onion skin-like arrangement of collagen reminiscent of a sclerotic (storiform) fibroma. The mitotic activity was negligible. Nonlipogenic cells were immunoreactive for CD99 (6/6) CD34 (6/8), S100 (4/7), and smooth muscle actin (2/6). Follow-up on 8 patients (range, 1 to 20 y, median 9.5 y) revealed no recurrence in 6 and possible persistent tumor in 2 after simple excision. The slide circulated to club members was from a 35-mm tumor on the dorsum of the right hand of a male aged 75. Club members generally agreed with a diagnosis of sclerotic lipoma but opinions differed as to whether it is a specific entity or a variant of spindle cell lipoma or dendritic fibromyxolipoma.

Selected case from the Arkadi M. Rywlin international pathology slide seminar: injection-site high-grade angiosarcoma, subcutis, left buttock region.

A high-grade angiosarcoma with epithelioid features located in the buttock of an 87-year-old woman arose in an area of old, palpable fat necrosis at the site of several subcutaneous injections administered 20 years previously. The nature of the injected material is unknown, but is presumed to have been an iron compound. Two weeks before surgery, the buttock lesion started to enlarge and was excised. It consisted of 3 contiguous nodules of old, calcified fat necrosis associated with plentiful hemosiderin. One of the nodules was largely replaced by an angiosarcoma, which was invading the edges of the other 2 nodules. The patient died from wound sepsis 41 days postoperatively, with no clinically apparent metastases. Vaccination injection-site sarcomas are well known to occur in cats, whereas in humans, rare sarcomas associated with prostheses and foreign materials have been reported; however, human injection-site sarcomas are vanishingly rare. The Club members agreed with the diagnosis of angiosarcoma at an injection site, with the majority calling it an epithelioid angiosarcoma. Many accepted that the injected material was probably iron, but one cautioned about regarding the injections as the cause of the angiosarcoma.

Inflammatory monomorphic undifferentiated sarcoma with distinct clinical and pathological features: a 'new' entity?

AIM: To describe two patients with a highly aggressive, apparently 'new' and rare soft tissue and bone malignancy of childhood and early adult life that we have named inflammatory monomorphic undifferentiated sarcoma. METHODS AND RESULTS: Two histologically identical tumours located in the proximal humerus and the anterior chest wall of males aged 6 and 31 presented as solitary, painful, tender, necrotising, masses, associated with fever, leukocytosis and negative microbiological cultures. The extensively necrotic resected tumours consisted of large, monomorphic epithelioid cells with vesicular nuclei, prominent eosinophilic nucleoli, and abundant eosinophilic cytoplasm surrounded by numerous neutrophils and eosinophils which formed sterile microabscesses. Immunohistochemical and ultrastructural studies revealed no specific differentiation. Both tumours were very aggressive, with early local recurrence, metastasis to regional and distant lymph nodes and viscera, and no response to several different chemotherapeutic regimens. CONCLUSION: A careful review of the literature led us to believe that inflammatory monomorphic undifferentiated sarcoma may represent a rare and distinct clinicopathological entity that does not appear to have been previously described.

Selected case from the Arkadi M. Rywlin International Pathology Slide Seminar: benign lymphoepithelial cyst, head of pancreas.

Pancreatic lymphoepithelial cysts are more common in men, can occur anywhere in the pancreas, are sharply demarcated from surrounding tissues, and range in size from 1.2 to 17 cm. Patients are usually middle aged, presenting symptoms include abdominal pain, nausea, vomiting, and diarrhea, although many tumors are asymptomatic and are discovered incidentally on organ imaging or at autopsy. An elevated serum carbohydrate-associated antigen 19-9 may wrongly suggest a mucinous neoplasm. The diagnosis can be made preoperatively with a combination of organ imaging, fine needle aspiration biopsy, or ultrasound-guided Trucut biopsies. Cysts can be unilocular, bilocular, or multilocular, have walls up to 0.6 cm thick which are lined by squamous epithelium, occasional columnar mucinous cells, and small foci of sebaceous cells. The epithelium is surrounded by a dense rim of lymphoid tissue with scattered lymphoid follicles. Invaginations of the epithelium into the lymphoid tissue, reminiscent of a Warthin tumor, are occasionally observed. The pathogenesis is unknown. Pancreatic lymphoepithelial cysts are cured by conservative resection but if they are asymptomatic and are diagnosed before surgery, no treatment is necessary.

Medicolegal liability in pathology: an international perspective.

An inevitable outcome of modern Medicine in any country is that some patients will experience adverse events, some of which would have been preventable. Different nations have developed various approaches to such cases; their legal efficacies are probably dissimilar and dependent on a number of disparate variables. An international "snapshot" of the results of the interacting forces can be obtained by asking physicians in several countries how they view selected subjective facets of their tort systems. In the U.S., many physicians view the structure of malpractice torts as unfair, and that belief is shared by at least some pathologists. The American Medical Association has declared that a multiregional malpractice "crisis" exists which raises medical costs and threatens access to care. Furthermore, malpractice tort decisions are often flawed scientifically because lay jurors and judges cannot properly evaluate the quality of "expert" testimony given by adversarial witnesses. Despite these factors, there has been little effort to investigate the views of pathologists on malpractice actions outside the U.S. In this paper, the authors have collected the responses of an international group of pathologists to a questionnaire on that topic. The respondents practice in academic centers in 15 countries outside the U.S. As expected, a range of views was represented, with some pathologists reporting that malpractice litigation was uncommon and others noting a worrisome trend toward its growth. Interestingly, so-called "defensive medicine" was found to be relatively common in pathology in many countries.

Glomangiomyoma (glomus tumour) of the pancreas: a case report.

Glomus tumours occur in many sites. We report the first case of a glomangiomyoma in the pancreas of a 17-year-old girl. The tumour was 5cm in diameter and consisted of rounded glomus cells, blood vessels and spindled smooth muscle cells. The glomus and smooth muscle tumour cells showed moderate diffuse cytoplasmic staining with vimentin, muscle-specific actin and smooth muscle actin. There was weak focal positivity for desmin in the spindle cell component only. The patient is alive and well with no evidence of recurrence 24 months after surgery.