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PubMed1999

Pleomorphic rhabdomyosarcoma of the uterus in a postmenopausal woman with elevated serum CA125.

BACKGROUND: Pure rhabdomyosarcoma of the uterus is an exceedingly rare tumor. While there are no commonly associated tumor markers, a previously reported case confirmed CA125 production by a pure uterine rhabdomyosarcoma. CASE: We describe a postmenopausal woman who presented with vaginal bleeding and was diagnosed with pleomorphic rhabdomyosarcoma of the uterus. Of particular interest was an elevated serum CA125 level at presentation. Immunohistochemical staining with CA125 antibody, however, showed no activity within the tumor. Despite aggressive therapy, the patient experienced distant nodal metastases and succumbed to the disease within 25 months of diagnosis. CONCLUSION: Our patient illustrates the typical presentation and aggressive behavior of this rare neoplasm. While serum CA125 may be a useful tumor marker in selected patients, elevated levels do not directly reflect tumor burden when associated with negative immunohistochemical staining.

CA-125 Antigen↗

PubMed1999

Embryonal rhabdomyosarcoma of the uterine corpus in a 76-year-old patient.

BACKGROUND: Primary embryonal rhabdomyosarcoma of the uterine corpus is an extremely rare tumor. A case of rhabdomyosarcoma originating in the uterine myoma is presented with a review of the literature. CASE: A 76-year-old female presented with a half-year history of abdominal swelling and fever of unknown origin. A 15 x 15 x 17 cm myoma-like tumor was noted on the anterior wall of the uterine myometrium. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. The histology and immunohistochemistry aided in the final diagnosis of a pure embryonal rhabdomyosarcoma of the uterine corpus. This patient received three courses of CYVADIC chemotherapy consisting of cyclophosphamide, vincristine, doxorubicin, and dacarbazine after the surgery and is now alive with no evidence of disease 10 months from her surgery. CONCLUSION: This case is extremely rare with respect to the uterine corpus origin, especially the myoma nodule and the age of the patient.

Aged↗

PubMed1989

New aspects in the treatment of childhood rhabdomyosarcoma: results of the German Cooperative Soft-Tissue Sarcoma Study (CWS-81).

The results of the German Co-operative Soft-Tissue Sarcoma Study (CWS-81) of the treatment of rhabdomyosarcoma are presented. Prior to the introduction of chemotherapy only 10%-20% of the children were successfully treated. Combined multi-agent cytostatic treatment improved the results dramatically. In patients with primary stage III rhabdomyosarcoma, local tumour control by surgery or radiotherapy should be undertaken earlier than week 16, if complete remission has not been achieved by 7-9 weeks. Patients with complete remission or partial tumour regression should be treated with the same combination of chemotherapy, while only partial responders need radiotherapy in addition. Patients with tumours which are primarily resectable without mutilation have a 90% chance of cure; this also applies to patients with primarily unresectable tumours who achieve complete remission after 7-9 weeks of chemotherapy. Total disease-free survival rate for stage III rhabdomyosarcoma patients was 53%. The role of surgery includes primary removal of the tumour or assessment of remission by means of histological spot checks.

Child↗

PubMed1994

A new highly polymorphic DNA restriction site marker in the 5' region of the human tyrosine hydroxylase gene (TH) detecting loss of heterozygosity in human embryonal rhabdomyosarcoma.

We have isolated a new marker (cos11-5TH) that detects an MspI restriction fragment length polymorphism in the 5' region of the human tyrosine hydroxylase gene (TH) on chromosome band 11p15.5. This region of human chromosome 11 contains several important loci for disease phenotypes including Beckwith-Wiedemann syndrome (BWS), Wilms' tumor, and embryonal rhabdomyosarcoma. Thus, identification of new polymorphic markers in this region are important for future gene mapping and linkage analyses. To better define the region of 11p15.5 deleted in embryonal rhabdomyosarcoma, this new marker was used to investigate allelic losses in embryonal rhabdomyosarcoma tumors.

Base Sequence↗

PubMed1985

Alveolar rhabdomyosarcoma in a young female patient metastasizing to the brain.

A case of 14-year-old girl is reported in whom an alveolar rhabdomyosarcoma occurred in the soft tissues of the left forearm 4 years prior to death. Despite extensive surgery as well as chemotherapy and radiotherapy the tumor recurred locally and produced extensive metastases including a metastasis to the brain. Cerebral metastases have not yet been reported in the literature despite extensive reports on the pathology of alveolar rhabdomyosarcoma. The morphological diagnosis of rhabdomyosarcoma was supported by the immunohistochemical demonstration of desmin, myosin, and myoglobin in the tumor cells.

Adolescent↗

PubMed1989

Evaluation of N-(5-indanylsulfonyl)-N'-(4-chlorophenyl)-urea against xenografts of pediatric rhabdomyosarcoma.

N-(5-indanylsulfonyl)-N'-(4-chlorophenyl)-urea (LY186641), a novel anticancer compound, was evaluated against six lines of rhabdomyosarcoma xenografts, each of which was established from tissue biopsies from untreated patients, and additional sublines selected as xenografts for primary resistance to vincristine, melphalan, and ifosfamide. LY186641 was given by oral gavage twice daily for 10 consecutive days or as 5-day courses repeated at 7-day intervals. At the optimal schedule, complete regressions of advanced tumors were obtained in each of the six rhabdomyosarcoma lines. There was no apparent cross-resistance in RMS lines selected for vincristine resistance or against multiple-drug-resistance KB cells in vitro. There was slight cross-resistance in xenografts selected for melphalan resistance, but not in an ifosfamide-resistant line. These results indicate that LY186641 may have significant clinical activity in the treatment of childhood rhabdomyosarcoma.

Animals↗

PubMed1975

Rhabdomyosarcoma of the duodenum with sinus tract formation into the gastric wall, visualized by gastroduodenoscopy.

Fiberoptic instruments have improved the accuracy of diagnosis of lesions of the upper digestive tract, particularly since the capability to perform biopsy under direct vision has been developed. Primary rhabdomyosarcoma of the upper gastrointestinal tract is an extremely rare entity. A literature search has revealed only 10 instances of rhabdomyosarcoma involving the esophagus, stomach, or duodenum (1-5). In this report, we describe the use of fiberoptic diagnosis and gastroduodenoscopic biopsy in a patient with rhabdomyosarcoma of the stomach and duodenum.

Aged↗

PubMed1990

A case of alveolar rhabdomyosarcoma with a chromosomal translocation, t(2;13)(q37;q14).

We report on an alveolar rhabdomyosarcoma occurring in a 17-year-old girl. Histologically, the tumour exhibited a proliferation of small, round cells and formed alveolar structures. Immunohistochemical studies of the tumour cells showed desmin and creatine kinase MM positivity. In electron-microscopic studies, the tumour cells showed an abundance of glycogen granules, and myofilaments were recognized in the cytoplasm. Chromosome analysis revealed a translocation, t(2;13)(q37;q14), which is though to be common in this subtype of rhabdomyosarcoma. Conventional ultrastructural and immunohistochemical investigations and chromosome analysis thus appear to be a highly promising combination of methods for improved pathological diagnosis of alveolar rhabdomyosarcoma.

Adolescent↗

PubMed1993

Primary rhabdomyosarcoma of the iliac bone in an adult: a case mimicking fibrosarcoma.

Primary rhabdomyosarcoma of bone is exceedingly rare. We present a case of rhabdomyosarcoma of the iliac bone in a 32-year-old male. Histologically, the tumour consisted mainly of a uniform proliferation of elongated spindle cells arranged in a herring bone pattern, simulating fibrosarcoma. Focally there was a conventional embryonal pattern with scattered rhabdomyoblasts possessing an eosinophilic cytoplasm. Immunohistochemical studies disclosed expression of muscle markers such as desmin and muscle-specific actin, in both the embryonal and spindle-cell areas and myoglobin only in the embryonal areas. Such histological features are unusual for classical embryonal rhabdomyosarcoma. The anatomical site and age of the patient are also atypical.

Adult↗

PubMed2001

The "botryoid sign": a characteristic feature of rhabdomyosarcomas in the head and neck.

We investigated nine patients with rhabdomyosarcoma in the head and neck (6-53 years of age), using CT and MRI. The tumours originated in the paranasal sinuses (3), cheek (2), soft palate (1), orbit (1), sternocostoclavicular muscle (1) and parapharyngeal space (1). The histological subtype was embryonal in five, alveolar in three and pleomorphic in one case. The tumours enhanced markedly and heterogeneous on CT and MRI. The masses were isointense or gave slightly higher signal than surrounding muscles on T1- and heterogeneously high signal on T2-weighted images. In four tumours, multiple ring enhancement resembling bunches of grapes. This appears to be characteristic of rhabdomyosarcoma and probably reflects a component of botryoid-type rhabdomyosarcoma in which mucoid-rich stroma is covered with a thin layer of tumour cells. We have named this imaging feature the "botryoid sign".

Adolescent↗

PubMed2005

Clinical characteristics and surgical treatment of perianal and perineal rhabdomyosarcoma: analysis of Japanese patients and comparison with IRSG reports.

Rhabdomyosarcomas of the perianal and perineal regions are uncommon. This study was performed to clarify the clinical characteristics and guidelines of surgical treatment of patients with perianal and perineal rhabdomyosarcomas younger than 20 years of age. Twenty-nine patients, 26 patients identified in the Japanese literature and three of our own, were analyzed and the results were compared with the data reported from the Intergroup Rhabdomyosarcoma Study Group (IRSG). Female predominance and a twin-peak age distribution in infancy and adolescence were characteristic findings of the Japanese patients that were not observed in the IRSG studies. The demographic differences between the two groups were attributed to the differences in demographics of patients younger than 10 years of age. Of the 29 patients, 17 were categorized into clinical groups III/IV and 21 patients into stages 3/4. Alveolar histology was diagnosed in 18 patients. In patients more than 10 years of age, the female predominance was more prominent and the incidences of advanced clinical groups/stages and alveolar histology were significantly higher than those in patients younger than 10 years of age. Inguinal lymph nodes were always involved in patients with lymph node metastases and three patients developed metastases to the breast. Information regarding the survival time was available for 18 patients and the 5-year overall survival was 20%. Two patients with a group I/stage 2 tumor and one with a group II/stage 3 tumor survived for more than 2 years with no evidence of the disease. In these patients, the tumors were excised by primary surgery or primary reexcision and they were not accompanied by lymph node metastasis. Based on these data, complete tumor resection prior to chemotherapy should be pursued and the inguinal lymph nodes should be at least sampled because nodal involvement is closely associated with the patient's prognosis.

Adolescent↗

PubMed1998

Primary conjunctival rhabdomyosarcoma: 2.5 years' follow-up after combined chemotherapy and brachytherapy.

BACKGROUND: The 2.5-year outcome of a 3-year-old girl with a primary intraconjunctival rhabdomyosarcoma treated by biopsy-controlled combined chemo- and brachytherapy is reported. METHODS: The patient presented with a conjunctival dermoid-like lesion which showed modest growth on 1-month follow-up. The first biopsy appointment was missed because of a viral illness. When she showed up 3 months later a further increase in size was obvious and several incisional biopsies were taken immediately. RESULTS: Light microscopy revealed an undifferentiated subepithelial small cell tumour. On immunohistochemistry the tumour reacted with vimentin and desmin antibodies. Thus, the diagnosis of an embryonal rhabdomyosarcoma was made. The patient underwent 9 cycles of polychemotherapy and the tumour mass disappeared macroscopically. However, immunohistochemistry of incisional biopsies showed residual tumour cells. The latter were eradicated by an individually shaped strontium-90 applicator. CONCLUSION: At 2.5 years after biopsy-controlled combined chemotherapy and brachytherapy the patient is free of tumour at a manageable level of complications. This new approach appears to be a good alternative in the treatment of primary conjunctival rhabdomyosarcomas since it takes advantage of the unique location of this rare tumour and avoids hemifacial retardation, known to be induced by external beam radiation.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2000

Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

Rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.

Adult↗

PubMed2000

Rhabdomyosarcoma presenting as an anterior cervical mass in an adult: report of a case.

We report herein the case of a 61-year-old man who was referred to our hospital for treatment of a rapidly growing tumor in the right anterior cervical region. A 13 x 14 x 15 cm mass was palpated on physical examination. Computed tomography (CT) findings indicated a thyroid tumor, but cytology results suggested rhabdomyosarcoma. A tumor, measuring 9.3 x 7.2 x 5.2 cm and weighing 220 g, was resected and histopathological examination confirmed a diagnosis of rhabdomyosarcoma. Cervical rhabdomyosarcoma is rarely found in adults.

Diagnosis, Differential↗

PubMedDate not supplied

Intergroup Rhabdomyosarcoma Study: update for pathologists.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, and 75% of such cases in the United States are reviewed at the Pathology Center for the Intergroup Rhabdomyosarcoma Study Group (IRSG). The first four generations of IRSG therapeutic trials (IRS I-IV) and supportive pathologic studies have generated a new International Classification of Rhabdomyosarcoma (ICR) that offers new morphologic concepts to the practicing pathologist. The objective of this report is to clearly define emerging histopathologic categories of RMS as defined by the ICR, and to emphasize correlative immunohistochemical or molecular studies. Emerging ICR variants of RMS place the patient in widely divergent prognostic categories (superior, botryoid or spindle cell variants; poor, solid alveolar or diffusely anaplastic variants). The cardinal histopathologic features of the ICR combined with results of studies of fusion genes seen with t(1;13) and t(2;13) will help delineate therapeutic subgroups of RMS for the fifth generation (IRS V) of IRSG studies. Consequently, it is imperative for the practicing pathologist to be familiar with the practical workup and diagnosis of RMS in childhood.

Biomarkers, Tumor↗

PubMed1994

Neonatal rhabdomyosarcoma: the IRS experience.

Neonatal rhabdomyosarcoma is rare, and the characteristics and optimal therapy for patients with this condition have not been well described. Of 3,217 eligible patients entered in the Intergroup Rhabdomyosarcoma Study (IRS), 14 were less than 30 days old at the time of diagnosis. Among these patients, male gender, Caucasian race, caudal tumors, and embryonal/botryoid and undifferentiated histology predominate. Although half the neonates survived, neither histology, tumor size, nor type of surgery were predictive of outcome. The presence of necrosis and small round cell configuration coincides with a poor prognosis, regardless of histological diagnosis. Neonatal rhabdomyosarcoma of caudal origin has a favorable prognosis.

Age Factors↗

PubMed1986

Embryonal rhabdomyosarcoma of the uterine corpus and cervix.

Embryonal rhabdomyosarcomas of the uterus and cervix are rare tumors with a very poor prognosis historically. Adjuvant multidrug chemotherapy combined with local radiation therapy and/or surgery has resulted in markedly improved survival rates. Six patients with embryonal rhabdomyosarcoma of the uterus/cervix are reported, with a compilation of 28 additional patients from the literature. Nineteen of 24 patients (79%) treated with combination therapy were alive and well 4-147+ months (median 61 months) after diagnosis. The six cases from USC Medical Center and affiliated hospitals are all well 17-144 months (mean 95 months) after diagnosis. Analysis of the collected series indicates that extent of disease is an important prognostic factor. Adjuvant chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) improves survival. Initial therapy for embryonal rhabdomyosarcoma confined to the uterus/cervix should include radical hysterectomy, pelvic lymphadenectomy, and adjuvant chemotherapy with VAC. Pelvic radiotherapy should be employed for involved surgical margins or positive nodes. Advanced disease managed initially with chemotherapy and radiotherapy may allow subsequent curative extirpative surgery.

Adolescent↗

PubMed1990

Rhabdomyosarcoma of the uterus in a postmenopausal patient.

Pure rhabdomyosarcomas of the female genital tract most commonly occur in infancy or childhood as sarcoma botryoides (embryonal rhabdomyosarcoma) and involve the vagina and cervix. Such tumors rarely occur in adults. A pure rhabdomyosarcoma of the uterus that arose in a postmenopausal patient is described. The pertinent literature is discussed.

Aged↗