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Myogenic markers in the evaluation of embryonal botryoid rhabdomyosarcoma of the female genital tract.

Rhabdomyosarcoma presents special diagnostic problems when it involves the uterine cervix in young children because tumor cells may lack marked atypia and may blend with the normal, immature, condensed, cellular stroma, rendering diagnosis difficult. Myogenic makers are a valuable ancillary technique for establishing a diagnosis of rhabdomyosarcoma. However, desmin positivity has been reported in cervical stromal cells, which can confound diagnosis. To determine whether immunohistochemical markers of skeletal muscle differentiation are helpful in the diagnosis of uterine botryoid rhabdomyosarcoma, we compared the immunohistochemical staining pattern of cervical rhabdomyosarcoma from 3 patients with that of normal uteri from age-matched autopsy controls by using antibodies for desmin, smooth muscle actin, muscle-specific actin, myoD1, myogenin, and WT-1. All tumors demonstrated at least focal immunopositivity for desmin, muscle-specific actin, smooth muscle actin, myoD1, and WT-1, and 1 tumor was also positive for myogenin. Autopsy controls showed only scattered subepithelial stromal immunoreactivity for desmin, muscle-specific actin, smooth muscle actin, and WT-1 and showed cytoplasmic, but not nuclear, immunopositivity for myoD1 and myogenin. Myometrium was diffusely positive for desmin and muscle-specific actin. We conclude that desmin, muscle-specific actin, smooth muscle actin, and WT1 are not specific for discriminating embryonal rhabdomyosarcoma from normal subepithelial cells in the female genital tract of children, although the number of immunopositive cells is consistently larger in rhabdomyosarcoma. Nuclear staining for myoD1 and myogenin appears not to occur in normal tissue, but it may be absent or sparse in embryonal rhabdomyosarcoma. Our findings indicate that, in this anatomic site, the diagnosis of rhabdomyosarcoma and in particular determination of tumor margins remain very reliant on histomorphology.

Biomarkers, Tumor↗

Myogenic markers in the evaluation of embryonal botryoid rhabdomyosarcoma of the female genital tract.

Rhabdomyosarcoma presents special diagnostic problems when it involves the uterine cervix in young children because tumor cells may lack marked atypia and may blend with the normal, immature, condensed, cellular stroma, rendering diagnosis difficult. Myogenic makers are a valuable ancillary technique for establishing a diagnosis of rhabdomyosarcoma. However, desmin positivity has been reported in cervical stromal cells, which can confound diagnosis. To determine whether immunohistochemical markers of skeletal muscle differentiation are helpful in the diagnosis of uterine botryoid rhabdomyosarcoma, we compared the immunohistochemical staining pattern of cervical rhabdomyosarcoma from 3 patients with that of normal uteri from age-matched autopsy controls by using antibodies for desmin, smooth muscle actin, muscle-specific actin, myoD1, myogenin, and WT-1. All tumors demonstrated at least focal immunopositivity for desmin, muscle-specific actin, smooth muscle actin, myoD1, and WT-1, and 1 tumor was also positive for myogenin. Autopsy controls showed only scattered subepithelial stromal immunoreactivity for desmin, muscle-specific actin, smooth muscle actin, and WT-1 and showed cytoplasmic, but not nuclear, immunopositivity for myoD1 and myogenin. Myometrium was diffusely positive for desmin and muscle-specific actin. We conclude that desmin, muscle-specific actin, smooth muscle actin, and WT1 are not specific for discriminating embryonal rhabdomyosarcoma from normal subepithelial cells in the female genital tract of children, although the number of immunopositive cells is consistently larger in rhabdomyosarcoma. Nuclear staining for myoD1 and myogenin appears not to occur in normal tissue, but it may be absent or sparse in embryonal rhabdomyosarcoma. Our findings indicate that, in this anatomic site, the diagnosis of rhabdomyosarcoma and in particular determination of tumor margins remain very reliant on histomorphology.

Biomarkers, Tumor↗

Uterine artery replacement by the round ligament artery: an anatomic variant discovered during uterine artery embolization for leiomyomata.

The authors report a case of an unusual anatomic variation encountered during uterine embolization, that of absence of a uterine artery, with replacement by an enlarged round ligament artery. This case highlights the importance of understanding the arterial anatomy of the uterus and its potential variations and their potential impact on the outcome of the procedure.

Arteries↗

[Intravenous leiomyomatosis].

A 49-year-old woman presented with a uterine tumour with solid and cystic parts. Extension of the tumour into the broad ligament was noted at the operation with wormlike plugs of tumour within the veins. The histological diagnosis of intravenous leiomyomatosis was evident. The disease turned out to be far advanced: a long continuous intraluminal mass extending from the iliac veins to the right ventricle made a second operation with thoracotomy and laparotomy mandatory. Postoperative follow-up showed so far, 1.5 years after operation, no evidence of recurrence. Intravenous leiomyomatosis is a very uncommon finding. Though it is histologically a benign smooth-muscle tumour, the biological behaviour with intravenous growth which may involve the right side of the heart, is quite aggressive. About 100 cases were reported in the literature; in no case could the diagnosis be made before surgery.

Female↗

[Stromal endometriosis. A rare semimalignant tumor with a tendency to recur. Clinical follow-up over 17 years].

The authors report on a female patient with stromal endometriosis. Clinical observation at the authors' clinic extended over an unusually long period, i.e., 17 years. After describing the histology, clinical symptoms, and therapeutic possibilities, the disease course, the repeated operations necessary, and the histological findings are presented in detail. In spite of the rarity of stromatosis it should be included in the differential diagnosis when diagnosing uterine sarcoma. After histological confirmation and the completion of surgical treatment of a stromatosis, recurrence and malignancy must be considered as possibilities even many years later.

Adult↗

The sentinel node in gynaecological malignancies.

As lymph node metastasis is one of the earliest features of tumour cell spread in most human cancers, assessment of the regional lymph nodes is required for tumour staging, determining prognosis and planning adjuvant therapeutic strategies. However, complete lymph node dissections are frequently associated with significant complications. Conjugating the diagnostic advantages with decreased morbidity, the sentinel node concept represents one of the most recent advances in surgical oncology. In this review we briefly highlight the historical background of the development of the sentinel node concept, the anatomical evidence for applying the sentinel node concept in pelvic gynaecological cancers and the technical aspects of sentinel node detection. We discuss recent studies in vulval, cervical and endometrial cancer.

Cervix Uteri↗

A case of concurrent uterine cervical adenocarcinoma and renal-cell carcinoma, and subsequent vaginal metastasis from the renal-cell carcinoma.

We report a case of concurrent uterine cervical adenocarcinoma and renal-cell carcinoma and a subsequent vaginal metastasis from the renal-cell carcinoma. The renal-cell carcinoma, which was located in the upper pole of the right kidney, was detected incidentally by preoperative CT scanning. We simultaneously performed in a timely manner radical hysterectomy, pelvic and paraaortic lymphadenectomies, and a right nephrectomy. Subsequently, however, the patient suffered from vaginal metastasis arising from the renal-cell carcinoma. As a result of this case, we emphasize the importance of making a thorough preoperative assessment prior to performance any definitive surgery for a gynecologic malignancy.

Adenocarcinoma↗