Aortic intimal separation resulting from manual cardiopulmonary resuscitation-completing the spectrum of blunt thoracic aortic injury complicating CPR.

Blunt thoracic aortic injury (BTAI) resulting from cardiopulmonary resuscitation (CPR) is rarely reported and most reports are of aortic rupture. Clinical reports have also documented aortic dissection and intramural hematomas with sequential imaging showing the development of these aortic injuries after the administration of CPR, suggesting that non-transmural aortic injury may also result from CPR. We report partial separation of an aortic intimal atheromatous plaque as a component injury in a case with multiple complications of manual CPR. A 74-year-old male presented to the emergency room (ER) with a 2-day history of chest pain. While in the ER, he suffered witnessed cardiac arrest and resuscitative attempts were pursued for 60 min prior to declaring death. At autopsy, there were numerous injuries attributable to CPR, including bilateral rib fractures, sternal fracture, retrosternal and mediastinal hemorrhage, epicardial ecchymoses, and ruptured pericardium. There was a perforated inferior wall myocardial infarct with a large left hemothorax. There was partial separation/laceration of an intimal atheromatous plaque on the anterior wall of the ascending aorta proximal to the origin of the brachiocephalic artery, forming a triangular flap, without associated intramedial dissection or hematoma. There was no thrombus formation, effectively excluding existence of the laceration prior to circulatory arrest. This aortic injury provides pathologic confirmation of non-transmural BTAI definitively sustained during manual CPR. Pathologists and clinicians alike should be cognizant of the possibility of BTAI resulting from CPR, which may manifest the full range of severity from intimal tear through aortic rupture.

Luteinized Thecomas ("Thecomatosis") with Sclerosing Peritonitis (LTSP): Report of 2 Cases and Review of an Enigmatic Syndrome Associated with a Peritoneal Proliferation of Specialized (vimentin+/keratin+/CD34+) Submesothelial Fibroblasts.

OBJECTIVE: To present the clinicopathologic features of two cases of luteinized thecomas with sclerosing peritonitis (LTSP), characterize the cellular proliferation in the sclerosing peritonitis (SP), and review the literature. METHODS: The clinical, laboratory, and imaging data, operative findings, and pathology materials were reviewed and summarized. Samples of the SP were stained with keratin AE1/AE3, vimentin, CD34, calretinin, smooth muscle actin, ER/PR, CD10 and desmin. A literature search was performed to identify cases of LTSP for comparison. RESULTS: A total of 43 cases of LTSP syndrome were identified. Frequent clinical features included ascites (74%), abdominal pain (35%), bowel obstruction (42%), and bilateral masses (84%). We isolated a distinct form of ovarian luteinized thecoma (thecomatosis) and peculiar sclerosing peritonitis (SP). IHC analysis shows a proliferation of specialized (vimentin+/keratin+/CD34+) submesothelial fibroblasts (SMF) with patchy expression of calretinin and hormone receptors. CONCLUSION: LTSP syndrome is a rare entity presenting with abdominal pain, bowel obstruction, ascites, ovarian masses, and SP containing specialized (vimentin+/keratin+/CD34+) SMF. LTSP must be distinguished from abdominal cocoon, isolated SP, Meigs' syndrome, and peritoneal carcinomatosis. The importance of recognizing the diagnosis is stressed, as failure to manage this disease conservatively leads to significant morbidity and mortality. The SP and bowel obstruction may persist for months, even after resection of the tumours, resulting in extended medical therapy. Based on the immunophenotype of the peritoneal lesions, strategies to elucidate 'targeted' pharmacologic agents that could inhibit the proliferation of specialized (vimentin+/keratin+/CD34+) SMF may be of benefit.

Impact of donor benign intimal thickening on cardiac allograft vasculopathy.

BACKGROUND: Epicardial cardiac allograft vasculopathy (CAV) is commonly described as a homogeneous smooth muscle cell (SMC)-rich inward intimal lesion with the SMC oriented circumferentially around the vessel. Recent findings have called this description into question. In this study we aimed to clarify the clinical presentation of epicardial CAV. METHODS: Autopsied samples of the 3 major coronaries were analyzed from patients fitting cardiac donor criteria (n = 10) and patients who had undergone cardiac transplantation (n = 34). Histology and immunohistochemistry were performed to identify cellular components of CAV, and image analysis was used to measure the various vascular compartments. RESULTS: Of the 34 cases examined, 28 of the epicardial intimal lesions contained 2 clearly definable layers overlying the media. The layer most adjacent to the media was SMC-rich, with the SMC oriented longitudinally along the vessel length and containing few macrophages, both characteristics of donor-derived benign intimal thickening (BIT). Transplants harvested at 1, 4 or 10 days post-transplant confirmed retention of BIT after transplantation. Image analysis of later transplants supported a hypothesis of carry-over BIT in CAV. The more lumenal CAV layer more closely resembled naturally occurring atherosclerosis. CONCLUSIONS: We propose that retention of the SMC-rich BIT layer after transplantation accounts, to a large extent, for the donor-derived, SMC-rich nature of human CAV, and that perturbation of the BIT provides the inflammatory foundation for the development of an accelerated atherosclerosis in the epicardial coronaries of transplant patients. This expanding accelerated atherosclerosis along with the underlying BIT demonstrates the characteristics ascribed to CAV.

Mitral valve and short-term ventricular assist devices; potential mechanical complications.

Mechanical complications of ventricular assist devices (VADs) are rare but serious. The authors describe two cases of different mechanical complications of VADs that can affect the mitral valve. Attention should be paid to the position of the inflow/outflow cannula after off-loading of the ventricle, especially in acute heart failure and normal atrial dimensions. Complete off-loading of the left ventricle in the presence of a bioprosthetic mitral valve might cause fusion of the valve leaflets leading to mitral stenosis, which will call for another intervention.

Myeloid sarcoma of the vagina: a report of 2 cases.

OBJECTIVE: To describe 2 cases of myeloid sarcoma of the vagina, in a patient without a history of acute myeloid leukemia (AML) and in another whose condition was previously diagnosed with AML. MATERIALS AND METHODS: The clinical histories of 2 patients whose conditions were diagnosed with myeloid sarcoma of the vagina were obtained from their medical records. RESULTS: Case 1: A 77-year-old woman with no systemic illnesses presented with a vaginal lump. Clinically, there was a 6-cm periurethral mass that was examined by biopsy. The histopathologic specimen was evaluated on routine and immunohistochemical stains, and myeloid sarcoma was diagnosed after extensive immunohistochemical analysis. The patient was treated with pelvic radiation. She developed extensive myeloid sarcoma of the skin and AML 4.5 months later; she died 2 weeks later, 5 months after the initial presentation. Case 2: A 36-year-old woman with a known history of AML who has had multiple leukemic and extramedullary recurrences presented with a pelvic mass. Physical findings revealed large masses in the vagina and rectovaginal septum, which were confirmed as myeloid sarcoma after biopsy and histologic examination. The patient was treated with pelvic/vaginal radiation. Five months later, she had another leukemic relapse and died within 1 day of palliative chemotherapy. CONCLUSIONS: Myeloid sarcoma of the vagina is extremely rare. Most patients have a poor prognosis and either have a history of or will subsequently develop AML.

Endometrioid carcinomas of the uterine corpus with sex cord-like formations, hyalinization, and other unusual morphologic features: a report of 31 cases of a neoplasm that may be confused with carcinosarcoma and other uterine neoplasms.

We describe a series of unusual endometrioid carcinomas (ECs) of the uterine corpus characterized in significant part by cords of epithelioid cells, spindle cells, and a hyalinized stroma that sometimes formed osteoid. These features, particularly when prominent, produced an appearance strikingly different from that of conventional EC, sometimes resulting in problems in differential diagnosis, especially with a malignant mullerian mixed tumor (carcinosarcoma). The 31 patients ranged in age from 25 to 83 years (mean, 52 years). The proportion within each stage were as follows: stage Ia, 9.7%, stage Ib, 45.2%, stage Ic, 9.7%, stage IIb, 16.1%, stage IIIc 3.2%, and stage IV, 3.2%. In 4 patients (12.9%), staging information was not available. On microscopic examination, typical EC, which accounted for 10% to 90% of the tumor, was admixed in 90% of cases with cords of epithelioid or spindle cells within a hyalinized stroma. In 3 cases, the tumor contained cords of cells without a hyalinized stroma. Areas with a diffuse growth of fusiform cells suggesting endometrial stromal cells were also occasionally seen in minor amounts. Seventy percent of the tumors exhibited squamous differentiation, and in 50% of the tumors there was a background of endometrial hyperplasia. Two thirds of the tumors were grade 2 and the remainder were grade 1. Vascular space invasion was identified in seven tumors. On immunohistochemical analysis, the typical EC component was strongly positive for keratin, whereas the keratin staining was more focal and variable in the epithelial cells in the cords. Muscle markers (desmin, actin), CD10, and inhibin were negative in the latter. Overexpression of p53 was found in only 1 case. Eighty-three percent of the patients were alive with no evidence of disease on follow-up (range, 2-115 months; mean, 34.4 months). The clinical features, including a typically low stage and generally good prognosis, and histologic findings are different from those of malignant mullerian mixed tumors that are characterized by both high-grade carcinomatous and sarcomatous components and an aggressive clinical course. Confusion with other neoplasms, particularly those with sex cord-like growth, such as uterine tumors resembling ovarian sex cord tumors and epithelioid smooth muscle tumors, may also arise. We refer to tumors with the features described herein as "corded and hyalinized endometrioid carcinomas," a designation that reflects their two most striking and consistent features. Corded and hyalinized endometrioid carcinomas are yet another example of the protean phenotype of endometrioid adenocarcinomas of the female genital tract that has been appreciated only in the last two decades.

Spread of prostate carcinoma to the perirectal lymph node basin: analysis of 112 rectal resections over a 10-year span for primary rectal adenocarcinoma.

We recently identified metastatic prostate carcinoma (PCA) within perirectal lymph nodes (PLNs) from 2 patients undergoing abdominoperineal resection (APR) for rectal adenocarcinoma (RA). As this phenomenon has not been addressed by any studies in the literature and because these positive PLNs had the potential to be mistakenly diagnosed as metastatic RA, we were prompted to undertake a retrospective study of rectal resections for RA to determine the frequency of PCA metastasizing to the PLNs in this patient population. The laboratory information system of the Department of Pathology, Capital Health, Halifax, Nova Scotia was searched for lymph node (LN)-positive RAs resected by low anterior resection or APR in male patients between January 1, 1992 and December 31, 2002. The hematoxylin and eosin slides were retrieved and reviewed, comparing the histology of the primary rectal tumor with that of the LN metastases in each case. Metastases having a different histologic appearance than the primary rectal tumor or having a pattern suggestive of metastatic PCA were analyzed by immunohistochemistry to detect prostate specific antigen (PSA), prostatic acid phosphatase (PAP), cytokeratin 7 (CK7), cytokeratin 20 (CK20), and carcinoembryonic antigen in LN metastases and in each RA. The presence or absence of mucin in the tumors was assessed by staining with Alcian blue, periodic acid-Schiff (PAS) +/- diastase, and modified PANFOPAS (2-hydroxy-3-naphthoic acid hydrazide/fast black B/saponification/periodic acid-Schiff). The study identified 112 cases of RA with positive LNs. Of those, 5 of 112 (4.5%) were identified as having metastatic PCA within the PLNs. All five were positive for PSA and PAP and only one case had rare CK20-positive tumor cells. The primary RAs were all diffusely positive for CK20 and carcinoembryonic antigen. Two cases of metastatic PCA expressed colonic type/acetylsialomucin, which was also seen in well-differentiated primary RAs. These 5 patients had a mean age of 76.8 years (range, 68-82 years). Four (80%) underwent APR while one (20%) underwent a low anterior resection. The mean number of LNs identified per case was 14 (range, 5-26). The mean number of LNs per case with metastatic PCA was 7.6 (range, 1-18). The majority of the LNs were under 1.0 cm in diameter. Two cases (40%) were associated with significant extranodal extension of PCA. The PLNs were mistakenly diagnosed as being involved by RA 40% of the time. On follow-up, 2 patients (40%) had died with progressive pelvic tumor, while 3 patients (60%) were alive, including 2 who, as a result of the study, were referred to the urology service for management. Both of these patients were subsequently started on medical (anti-androgen) therapy and 1 additionally had bilateral orchiectomy. In 4 of the patients, the serum PSA after the rectal resection ranged from 2.5 to 13.5 ng/mL. In 1 patient, the serum PSA was markedly elevated (5961 ng/mL), and this patient was subsequently identified on bone scan as having extensive skeletal metastases. This study identified a subset (4.5%) of patients with RA and PLNs positive for PCA. PCA may extend to the PLN basin and therefore influence the management of patients with rectal tumors and the staging, LN dissection, and management of patients with PCA. Moreover, the LNs were incorrectly diagnosed as metastatic RA 40% of the time, emphasizing the need to consider the differential diagnosis of metastatic PCA when evaluating PLNs. The diagnosis of metastatic PCA can be confirmed using an immunohistochemical panel consisting of PSA, PAP, CK20, and carcinoembryonic antigen along with mucin stains in some cases. In patients undergoing APR for RA, there should be preoperative screening for PCA as it will not be possible to do a digital rectal examination or transrectal ultrasound post-APR, and a prostate biopsy, if necessary, would have to be done via the more difficult transperineal approach.

Unusual epithelial and stromal changes in myoinvasive endometrioid adenocarcinoma: a study of their frequency, associated diagnostic problems, and prognostic significance.

The authors have noted that when the myoinvasive glands of endometrioid carcinomas evoke a prominent fibromyxoid stromal reaction, they sometimes undergo distinctive changes. These are characterized by outpouchings from typical neoplastic glands that become detached and often lined by flattened epithelium, sometimes appearing as microcysts. The glands may less often become elongated or undergo fragmentation into small solid clusters or single cells. For this constellation of changes, which in aggregate are distinctive, the authors have coined the acronym MELF (microcystic, elongated, fragmented). The authors evaluated the prognostic significance of these stromal and glandular features and their association with each other and with other histopathologic and clinical prognostic factors by studying 115 unselected myoinvasive endometrial endometrioid carcinomas. The histologic slides and clinical records were reviewed to collect data on age, recurrences or metastases, survival, stromal reaction pattern (fibromyxoid, lymphocytic, or absent), presence of MELF, FIGO grade, depth of myometrial invasion, vascular invasion, squamous differentiation, and presence or absence of necrosis. Factors associated with an unfavorable outcome (recurrence or death) included a fibromyxoid stromal reaction, age older than 70 years, advanced stage, vascular invasion, FIGO grade, depth of myoinvasion, and the presence of tumor necrosis. The presence of a host lymphocytic reaction was associated with a favorable outcome. A multivariate logistic regression model identified stage and age older than 70 years as independent prognostic factors. The MELF changes were associated with the presence of a host stromal reaction (most strongly with a fibromyxoid reaction) and vascular invasion. Within the group associated with a fibromyxoid reaction, patients exhibiting MELF had a better survival. In conclusion, a fibromyxoid reaction in cases of endometrioid carcinoma is associated with a higher frequency of death or recurrence and it is frequently accompanied by distinctive morphologic changes (MELF) in myoinvasive glands as well as lymphatic or blood vessel invasion. MELF is associated with a fibromyxoid reaction but is not independently associated with an adverse effect on prognosis. A lymphocytic stromal reaction is associated with a favorable effect on prognosis and is less often accompanied by the distinctive morphologic changes (MELF) highlighted herein.

Cutaneous ciliated cyst of the abdominal wall: a case report with a review of the literature and discussion of pathogenesis.

A cutaneous ciliated cyst is a rare lesion typically found on the lower extremity of young girls shortly after puberty. Here, we report a case involving a previously unreported site (i.e., abdominal wall) in a 14-year-old girl. We also describe immunohistochemical and ultrastructural findings, review and analyze the world literature, and offer insights as to the pathogenesis of this lesion.

Uterine Endometrioid Carcinoma with Small Nonvillous Papillae: An Analysis of 26 Cases of a Favorable-Prognosis Tumor To Be Distinguished from Serous Carcinoma.

We have encountered a number of endometrioid carcinomas with small papillary buds lacking fibrovascular cores that could be confused with the small cellular papillae of serous papillary carcinoma (SPC). We have designated these tumors "endometrioid carcinoma with small nonvillous papillae" (ECSP). Because they have not been investigated previously we analyzed 26 examples and compared their features with those of 21 SPCs of the uterus. Three hundred and ninety consecutive cases of endometrial carcinoma diagnosed between January, 1989, and January, 1994, were retrieved from our hospital files; 26 (6.7%) of them, (8% of the endometrioid carcinomas) were identified as ECSP, and 21 (5.4%) as SPC. Tumors were classified as ECSP when the small papillae were present within the glands of otherwise typical endometrioid carcinoma or on the villous projections of villoglandular endometrioid carcinoma. Most of the papillae were in the form of buds of cells with ample eosinophilic cytoplasm and a low nuclear-to-cytoplasmic ratio, but some papillae had a more complex pattern. The papillae arose on a background of International Federation of Gynecology and Obstetrics (FIGO) grade 1 or 2 endometrioid carcinomas, which showed squamous differentiation in half the cases. SPCs were identified according to generally accepted criteria. The mean age of the patients with ECSP was 67 years, intermediate between that of the patients with endometrioid carcinoma lacking small nonvillous papillae (62 years) and that of the patients with SPC (71 years). Patients with ECSP more frequently presented at an earlier stage (73% stage I/II) than those with SPC (29% stage I/II). The overall 5-year survival of patients with ECSP was 84% (95%CI: 0.68-1), more than double that of patients with SPC, 33% (95%CI: 0.10-0.56). ECSP may be confused with SPC on microscopic examination but has clinical and pathological features similar to those of endometrioid carcinoma lacking small nonvillous papillae, and unlike SPC, should be treated in the same manner as the former. Int J Surg Pathol 8(4):279-289, 2000