Primary extra-cranial meningioma following total hip replacement.

A 61-year-old man presented with pain at the left hip and decreased mobility 10 years after total hip replacement. Imaging demonstrated a large destructive expansile mass adjacent to the prosthesis. Histological analysis confirmed the presence of an extra-cranial meningioma. Primary tumours after total hip replacement are rare and include soft tissue sarcomas, bone sarcomas and lymphomas. To our knowledge, no previous cases of primary extracranial meningioma have been identified. The imaging features, histology, pathogenesis and differential diagnosis are discussed.

Malignant peripheral nerve sheath tumour in the ischio-rectal fossa.

Primary sarcomas in the ischiorectal fossa are occasionally reported and represent a significant challenge due to the proximity of rectum, levator muscles and pudendal neurovascular structures. We report a case in which the diagnosis changed between biopsy (desmoid tumour) and resection (malignant peripheral nerve sheath tumour), requiring a multidisciplinary surgical approach involving different sub-specialties. It also illustrates the importance of undertaking sarcoma surgery in a recognized sarcoma centre with sarcoma expertise available across a range of disciplines.

A rare case of low-grade endometrial stromal sarcoma with myxoid differentiation and atypical bizarre cells.

Endometrial stromal sarcoma (ESS) is a rare mesenchymal tumor with characteristic histological appearances, consisting of diffuse infiltrate of small uniform endometrial stromal cells with a multinodular arrangement and distinct vascular pattern. Less common variants of ESS include "mixed endometrial stromal and smooth muscle tumors", "endometrial stromal tumors resembling ovarian sex cord tumors" and "endometrial stromal neoplasms with endometrial glands", and "aggressive endometriosis". Rarely do endometrial stromal tumors have a prominent fibrous or myxoid appearance which causes confusion and possible misdiagnosis as myxoid leiomyosarcoma. In this report we present a very unusual subtype of ESS in a 32-year-old woman. The tumor revealed atypical pleomorphic bizarre cells which were stained positive only with vimentin and CD10 in an abundant myxoid matrix. A low-proliferative rate was established with MIB-1 staining. To our knowledge such appearance has not been previously reported.

Endometriosis of the superior gluteal nerve.

Endometriosis that involves nerves is rare and usually occurs in the pelvis. We describe a case of extra-pelvic endometriosis that involved the superior gluteal nerve. The patient presented with ill-defined buttock and thigh pain and a Trendelenberg gait. MRI demonstrated atrophic changes in the gluteus minimus, gluteus medius and tensor fascia lata muscles indicating involvement of the superior gluteal nerve. MRI showed a mass in the line of the superior gluteal nerve. The mass was hyperintense on STIR and T1-weighted images and did not enhance after intravenous contrast although surrounding edema did enhance. Histological examination of material obtained at open biopsy showed endometriosis. The patient was commenced on hormonal treatment to suppress ovarian activity.

Superficial acral fibromyxoma.

Superficial acral fibromyxoma is a rare soft tissue tumour affecting the digits, particularly the nail bed region. The condition was first described in 2001. We report the case of a 71-year-old man who presented with a long standing history of a lump affecting the pulp of the his right ring finger. This was surgically excised and subsequent pathological analysis confirmed the lesion to have been a superficial acral fibromyxoma. We use this case to highlight the features of this rare clinical entity, which has never previously been described in the surgical literature.

Benign mixed tumor of the vagina: case report with expanded immunohistochemical profile.

We present a case of benign mixed tumor of the vagina with its typical clinicopathologic and expanded immunohistochemical features. The strong coexpression of the mesenchymal spindle cell component with epithelial markers such as CK7, together with strong CD10, Bcl2, and estrogen and progesterone receptors favors a mullerian derived tumor. The tumor is best labeled as benign mixed mullerian tumor as originally designated.

Prognostic indicators for gastrointestinal stromal tumours: a clinicopathological and immunohistochemical study of 108 resected cases of the stomach.

AIMS: Whether immunohistochemical markers increase accuracy in predicting prognosis for gastrointestinal stromal tumours (GISTs) remains uncertain. However, past studies have used only small, heterogeneous patient groups. Our aim was to test previously studied and more novel morphological features as well as four immunohistochemical markers as prognostic indicators amongst a large cohort of surgically resected, gastric GISTs. METHODS AND RESULTS: Tissues from 127 gastric mesenchymal tumours were collected retrospectively and subjected to repeat histological assessment and immunophenotyping. Further immunohistochemistry was performed for Ki67, p53, Bcl-2 and cyclin D1. Complete follow-up data were collected for 108 patients with immunophenotyped diagnoses of GIST (i.e. c-kit+ tumours). At the census point, 52 patients were alive, 24 had died from their GISTs and the remainder of other causes. Univariate analysis showed the following predicted for shorter disease-specific survival: size > or =50 mm; necrosis, no intratumoral lymphocytes; mitotic count > or =5/50 high power fields; Ki67 labelling index > or =5%; p53 immunopositivity. Of these variables, multivariate analyses showed only mitotic count and, to a lesser extent, Ki67 labelling to be independent prognostic indicators. CONCLUSIONS: Mitotic count remains the best predictor of outcome following surgical resection of gastric GISTs. Ki67 immunohistochemistry does not provide better prognostication and p53, Bcl-2 and cyclin D1 immunohistochemistry provide no additional prognostication.

Malignant mixed mesodermal tumours: biology and clinical aspects.

Mixed mesodermal tumours (MMTs) are relatively rare gynaecological tumours that have been poorly studied in clinical and molecular terms. They are chemosensitive (at least initially), although ultimately they have a poor prognosis. The biology of the tumour is fascinating in view of its composition of both epithelial and mesenchymal entities. We review herein the literature on the clinical and biological aspects of this malignancy.

Cervical implant from villoglandular endometrial adenocarcinoma masquerading as cervical villoglandular adenocarcinoma.

This is an unusual presentation of a rare subtype of endometrial adenocarcinoma (villoglandular papillary carcinoma, VGPC) in a 71-year-old woman, which was initially diagnosed on cervical biopsy as being primary cervical VGPC. Loop excision failed to show any evidence of residual disease. Subsequent hysterectomy revealed a localized villoglandular carcinoma involving the uterine fundus and invading the inner one-third of the myometrium, the background endometrium was atrophic. The remaining cervix contained a focus of papillary forming endometrial type adenocarcinoma involving the surface epithelium and the superficial subepithelial glands. In conclusion, VGPC of cervix occurs mainly in young women and can be treated conservatively, pathologists should be cautious in making such a diagnosis in a postmenopausal woman before ruling out a primary endometrial origin.

Cotyledonoid hydropic intravenous leiomyomatosis: a new variant leiomyoma.

AIMS: We present the histopathological findings of a series of six cases of a benign uterine smooth muscle tumour with an unusual growth pattern. METHODS AND RESULTS: All cases have the appearances of the recently described dissecting (cotyledonoid) leiomyoma. In addition, three of these lesions demonstrate the features of intravenous leiomyomatosis with varying degrees of hydropic degeneration. CONCLUSIONS: This combination of phenotypes has not previously been described within the literature; therefore we propose that these are classified as examples of 'cotyledonoid hydropic intravenous leiomyomatosis', a new variant of unconventional leiomyoma.

Sarcomatoid/metaplastic carcinoma of the breast: a clinicopathological study of 12 cases.

AIMS: To analyse the clinical and pathological features with long-term follow-up of a series of 12 cases of sarcomatoid carcinoma of the breast. methods and results: The cases were selected from the surgical files of the Department of Pathology, University of Edinburgh, between 1977 and 1988. The following clinical parameters were recorded: the age of the patients, size of tumour, presence or absence of lymph node or distant metastases, and patient survival. Pathological assessment included: the type of epithelial and mesenchymal components, the proportion of monophasic to biphasic tumours and the presence of adjacent in-situ carcinoma/atypical epithelial proliferation. The mean age of the patients was 61 years with a median of 64 and range 46-82 years. The mean size of the tumour was 52 mm (range 22-100 mm). None of the patients had distant metastasis at presentation and only one case had local lymph node metastasis which had a carcinomatous appearance. Five women were still alive after a minimum 12-year follow-up period. Four patients died of their disease (three with lung metastasis only and one with lung and bone metastases), one died of carcinoma of the cervix and two patients were lost to follow-up. Pathologically, four cases (33.3%) had no or almost undetectable epithelial structures by light microscopy, i.e. "monophasic sarcomatoid carcinoma". The remaining cases revealed varying proportions of both epithelial and mesenchymal elements, i.e. "biphasic sarcomatoid carcinoma". Of the epithelial component, six (50%) tumours had predominantly carcinoma of no special type, one lobular and one tubular carcinoma. The mesenchymal component was fibromatosis/nodular fasciitis-like, malignant fibrous histiocytoma-like (MFH), osteosarcoma-like and fibrosarcoma-like in five (42%), four (33%), two (17%) and one (8%) tumours, respectively. In 3/4 monophasic tumours, the mesenchymal component was of a low-grade fibromatosis/nodular fasciitis type. In 6/12 (50%) of the cases there was associated in-situ atypical epithelial proliferation (five ductal carcinoma in situ (DCIS) and one atypical ductal hyperplasia). CONCLUSIONS: From this small series it appears that sarcomatoid carcinoma is an uncommon tumour, which is large in size and tends to lack local or distant metastasis at presentation. Pathologists should be alert to the presence of the bland monophasic sarcomatoid carcinoma which has a pure mesenchymal appearance on light microscopy, but epithelial components demonstrated by cytokeratin immunohistochemistry. These showed metastases on long-term follow-up, similar to other histological patterns of sarcomatoid carcinoma.

Clinicopathological study of the pattern and significance of cervical involvement in cases of endometrial adenocarcinoma.

The pattern of cervical involvement in 107 endometrial adenocarcinomas was assessed. The cervix was involved in 29%, higher than noted in previous studies. In 40.6%, the lesion was confined to surface endocervical epithelium only; the remainder had cervical stromal involvement. In the majority only small areas within the circumference of the cervix were affected, indicating a need for adequate tissue sampling. In some cases, malignant epithelium was found as a "migrant" within the endocervical canal, entrapped within cervical mucus or applied to surface epithelium, supporting the concept that endometrial cancer spreads by surface contiguity or implantation rather than by deep tissue planes or via lymphatic channels. Our findings reinforce the view that high-grade lesions and histological subtypes such as uterine serous papillary carcinoma are associated with a later presentation, higher stage and poorer prognosis. We have identified atypical changes in endocervical epithelium that may be misinterpreted as cervical involvement, particularly in the form of atypical reserve cell hyperplasia with a micropapillary pattern that may reflect a reaction to the presence of tumor. It is our assertion that the presence of tumor "migrants", and not endocervical surface atypia, is an indicator of increased probability of cervical involvement by endometrial adenocarcinoma (P = 0.015).

Uterine serous papillary carcinoma: histopathologic changes within the female genital tract.

The histopathologic features of 25 patients with uterine serous papillary carcinoma (USPC) were presented, with particular emphasis on the changes seen in the remaining müllerian epithelium. The mean age at presentation was 68.9 years; 52% of patients were stage III at the time of presentation and 40% died of their disease within 24 months of diagnosis. Histologic assessment revealed: 1) pure serous carcinoma in 56% of patients and mixed differentiation of serous and endometrioid in the remainder; 2) malignant epithelium reminiscent of that of USPC and akin to carcinoma in situ, frequently seen in the remaining endometrium, cervix, and, less commonly, the fallopian tube; 3) residual endometrium that, when identified (11/25 cases), was atrophic in all cases; 4) various types of cervical involvement in 17 cases (68%); 5) tumor within the fallopian tube in three cases (12%); and 6) carcinoma with in situ-like features in five cases (20%). In conclusion, it appears that USPC is frequently associated with malignant epithelial changes (as with carcinoma in situ) in the remaining müllerian epithelium. This finding suggests either a field change or, more likely, a transepithelial tumor spread. The latter theory is preferable, because this type of spread is frequently seen on serosal surfaces in cases of serous ovarian carcinoma. Uterine serous papillary carcinoma is, therefore, biologically more akin to its ovarian counterpart.

Intra-abdominal spindle cell lesions: a review and practical aids to diagnosis.

Intra-abdominal spindle cell lesions are uncommon and often present a diagnostic challenge. An important group of such lesions are the gastrointestinal stromal tumours. Other intra-abdominal spindle cell lesions include fibromatosis, various sarcomas-in particular, leiomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumour-and, in women, endometrial stromal sarcoma. Less common lesions are inflammatory myofibroblastic tumours, the mesenteric spindle cell reactive lesions, retroperitoneal fibrosis, and solitary fibrous tumour. A variety of intra-abdominal tumours of nonmesenchymal origin may have a spindle cell/sarcomatoid morphology; these include sarcomatoid carcinoma, malignant melanoma and, in women, sarcomatoid granulosa cell tumour. Finally, metastatic sarcomas from pelvic or extra-abdominal organs need also be considered. A set of practical aids to the diagnosis of intra-abdominal spindle cell lesions is presented to assist pathologists dealing with such lesions, particularly with regards to the consideration of differential diagnoses.

Giant cell tumour of tendon sheath (localised nodular tenosynovitis): clinicopathological features of 71 cases.

AIMS/BACKGROUND: Giant cell tumour of the tendon sheath (GCTTS) is regarded as the most common neoplasm of the hand that can recur after excision. The objective of this study was to review a series of cases in our department and to determine any clinical or pathological features that might predict the likelihood of recurrence. METHODS: Clinical data, obtained from pathology request forms and in patient notes, along with the gross and microscopic appearances of 71 cases of GCTTS were evaluated. RESULTS: Clinical features and pathological features identified were similar to those of previous studies. In comparison with previous studies a higher mitotic count (range, 1-21 mitoses/10 high power fields (HPF); mean, 5/10 HPF) was noted in all cases, irrespective of recurrence and numerous apoptotic bodies (up to 30/10 HPF), mainly formed from osteoclast-like giant cells, were present. CONCLUSIONS: GCTTS is a relatively rare soft tissue tumour of uncertain histiogenesis. Mitotic and apoptotic figures are a common feature and do not indicate clinical behaviour. Complete local excision is the treatment of choice.

The borderline cervical smear: colposcopic and biopsy outcome.

AIMS: To review the outcome of women referred with smears showing borderline nuclear change (BNC), and to determine any differences in outcome if BNC was persistent, preceded by dyskaryosis, or followed treatment for cervical intraepithelial neoplasia (CIN). In addition, to determine criteria that might permit delineation of a BNC subtype, predictive of CIN. METHODS: The records of 178 women referred for colposcopy in 1993, with last smear showing BNC, were obtained from our laboratory database. The cytology, colposcopy, and biopsy follow up for a five year period were also obtained. The patients were divided into three categories according to their smear status before the last referral borderline smear: category 1, persistent BNC (n = 39); category 2, BNC preceded by dyskaryotic smears (n = 100); and category 3, BNC after treatment for CIN (n = 39). The referral borderline smears were reviewed on cases with negative outcome and those with a biopsy diagnosis of CIN2 and CIN3. RESULTS: In 50 women (28%) no biopsy was deemed necessary after colposcopic assessment. The biopsy results in the remaining 128 (72%) women were as follows: normal in 18 (10%), koilocytosis in 12 (7%), CIN1 in 45 (25%), CIN2 in 32 (18%), and CIN3 in 21 (12%) women. High grade lesions (CIN2, CIN3) were seen on biopsy in 14 of 39, 33 of 100, and six of 39 cases in category 1, category 2, and category 3, respectively. Blind review of the referral borderline smears from 53 women with a biopsy diagnosis of high grade lesions (32 CIN2, 21 CIN3) confirmed they were borderline in 23, upgraded them to mild dyskaryosis in 15, and found that 14 cases of isolated moderate or severe dyskaryotic cells had been missed originally. The borderline change was in mature squamous cells in five of 23 and in immature metaplastic epithelium in 18 of 23 cases. After smear review in 68 women with negative outcome, 36 smears were reclassified as negative in keeping with inflammation and atrophy, three were considered unsatisfactory, one was upgraded to CIN1, and 28 were confirmed as BNC. Of the latter, 25 of 28 were in mature squamous cells. The five year follow up on women with negative colposcopy (n = 50), negative loop excision of transformation zone (LETZ) (n = 18), and LETZ with koilocytosis (n = 12) showed subsequent high grade CIN on LETZ in 16, 0, and two patients, respectively. CONCLUSIONS: On referral of women for colposcopy with last smear showing BNC, the outcome was high grade CIN in over 30% of cases, irrespective of whether the borderline smear was preceded by another borderline smear or by a dyskaryotic smear. In contrast, in those referred because of BNC after treatment of CIN, high grade CIN was seen less frequently (15% of cases). Furthermore, in cases that necessitated loop excisions, high grade CIN was seen in 41%. This study also showed that BNC associated with inflammation or atrophy, or BNC in mature squamous cells, appears to have lower predictive value for CIN than those cases where BNC is associated with immature metaplastic epithelium. The use of terms such as "BNC favour reactive" for the former and "BNC favour dyskaryosis" for the latter is recommended, together with follow up by cytology and colposcopy, respectively.