Bulky mesonephric adenocarcinoma of the uterine cervix treated with neoadjuvant chemotherapy and radical surgery: report of the first case.

AIMS AND BACKGROUND: Malignant mesonephric adenocarcinoma of the uterine cervix is a rare occurrence with few cases described in the literature. Although surgery seems to be effective in the treatment of early-stage tumor, no cases describing outcomes of locally advanced stage are available. METHODS: We report the first case of a patient with International Federation of Obstetrics and Gynecologists stage IIB mesonephric adenocarcinoma undergoing neoadjuvant chemotherapy and radical surgery. CONCLUSIONS: Despite the inherent limitation of a single description of a case, our experience supports the utilization of neoadjuvant chemotherapy in patients with malignant mesonephric adenocarcinoma of the uterine cervix. Further prospective multi-institutional studies are needed.

Malignant mesonephric tumors of the female genital tract: a clinicopathologic study of 9 cases.

Nine malignant mesonephric tumors were obtained from the consultation files of one of the authors (J.P.) over a 13-year period (1988-2001). There were 4 adenocarcinomas (ACs) and 5 malignant mixed mesonephric tumors (MMMTs). The ACs were found in the cervix (3) and vagina (1). The MMMTs involved the uterus (1), cervix (3), and vagina (1). Most patients presented with abnormal vaginal bleeding. The 4 patients with mesonephric AC ranged in age from 24 to 54 years (mean, 41 years). The tumors measured 2 to 6 cm (mean, 3.7 cm). Two ACs were stage I and two were stage II. Two of the three patients with follow-up information were alive without clinical evidence of disease at 3 and 11.5 years, and the other was alive with recurrent tumor 8.5 years postoperatively. The 5 patients with MMMTs ranged in age from 37 to 62 years (mean, 49 years). The mean size of four tumors was 5.2 cm (range, 3.5-8 cm). The uterine MMMT infiltrated the entire myometrial wall extending to the endometrial cavity where it resembled an endometrial polyp. Although the most common histologic pattern in the current series was the glandular (ductal) pattern, retiform, tubular, and solid growth patterns were also encountered. Among the MMMT subgroup, the sarcomatous component was homologous in 3 cases (endometrial stromal or spindle cell) and heterologous in the other 2 cases (skeletal muscle and cartilage). Of the 4 patients with follow-up information available, 1 (stage II) died of disease 7 months after surgery, another (stage IV) was alive with bone metastases at 3.3 years, and the other 2 patients (stages IB and IC) had no clinical evidence of disease at 1 and 3.7 years, respectively. Evidence of mesonephric hyperplasia was found in 5 (42%) cases. The MMMT that arose in the corpus presented as an endometrial polyp. In this case, histologic differential diagnosis includes serous carcinoma, endometrial stromal sarcoma, and uterine tumor resembling ovarian sex cord-stromal tumor. Immunostainings are not helpful. Mesonephric ACs often present in early stage and have better prognosis than their müllerian counterparts. Surgery alone appears to be the treatment of choice. In contrast, MMMTs may present in advanced stage and are aggressive tumors, similar to malignant mixed müllerian tumors.

Mesonephric adenocarcinoma of the uterine corpus: CD10 expression as evidence of mesonephric differentiation.

Mesonephric (wolffian) neoplasms of the female genital tract are infrequent and found in sites where embryonic remnants of wolffian origin are usually detected, such as the uterine cervix, broad ligament, mesosalpinx, and ovary. Their diagnosis is difficult because of the absence of specific immunohistochemical markers for mesonephric derivatives. We present the first report of adenocarcinoma of mesonephric type arising as a purely myometrial mass without endometrial or cervical involvement in the uterine corpus of a 33-year-old woman. The tumor showed a combination of patterns, with retiform areas, ductal foci, and small tubules with eosinophilic secretion, which merged with solid sheets of cells with a sarcomatoid appearance. Immunohistochemically, neoplastic cells were diffusely positive for cytokeratin 7, epithelial membrane antigen, and CD15 and focally positive for BerEP4 and vimentin. A hitherto unreported feature was the positivity for CD10 in neoplastic cells, which was also present in a large number of control tissues obtained from male mesonephric derivatives and female mesonephric remnants and tumors. Furthermore, CD10 was negative in controls from müllerian epithelia of the female genital tract and in their corresponding tumors. Therefore, the expression of CD10 by mesonephric remnants may be useful in establishing the diagnosis of tumors with mesonephric differentiation.

Endodermal sinus tumour of the vulva: an interesting clinico-pathological problem.

Endodermal sinus tumours (EST) of the lower female genital tract are uncommon malignancies. The majority of these involve the vagina and cervix, though there are a few case reports of tumours involving the vulva. These are usually either locally advanced or have metastatic disease present at initial diagnosis, and generally do badly on treatment. This case report discusses primary vulval involvement by EST. It shows that the absence of tumour markers can be misleading, and discusses the role of radiation and chemotherapy in the treatment of this rare disease.

Fine needle aspiration biopsy findings in endodermal sinus tumors. A report of four cases with cytologic, immunocytochemical and ultrastructural findings.

We present four cases of endodermal sinus tumor affecting children (median age, 20 months). Three tumors were located in the sacrococcygeal region and one in the right testicle. All cases were diagnosed by fine needle aspiration biopsy. The most characteristic features were cells arranged in a papillary-like configuration, vacuolated cytoplasm and intracellular and extracellular deposits of pink, homogeneous material. Immunocytochemical study showed alpha-fetoprotein-positive cells. Ultrastructural study showed intracytoplasmic inclusions of electron-dense material and basement membrane-like material in the intercellular spaces.

Management of yolk sac tumors in children.

The current trend toward minimizing treatment morbidity in children with yolk sac carcinomas is laudable. As most children will present with stage I disease and be cured by radical orchiectomy alone, careful surveillance is adequate with the knowledge that effective chemotherapy is available should tumor recur. All new yolk sac carcinomas in children should be reported to the Prepubertal Testicular Tumor Registry and should ideally be treated at institutions that have experience with surveillance protocols and pediatric oncology. In this way, prospective studies on treatment options can be initiated, and management controversies may ultimately be ironed out. In particular, more information is needed on patients more than 2 years old, as the literature to date is contradictory regarding the prognosis for these patients and the need for adjuvant therapy when the older child presents with stage I disease.

Endodermal sinus (yolk sac) tumor of the parotid gland: a case report.

Malignant salivary gland neoplasms in children are rare, most common being mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma and adenocarcinoma. Most germ-cell neoplasms of head and neck in children are teratomas. The authors report a case of endodermal sinus tumor (EST) of the parotid gland in a 2-year-old girl, which recurred after chemotherapy. The role of alpha-fetoprotein (AFP) serum level as a helpful marker in differential diagnosis and in evaluating tumor progression is underlined.

[Pure yolk sac tumor of the testis with brain metastasis: report of an adult case].

Herein we report an adult case of pure yolk sac tumor with brain metastasis. The patient was a 37-year-old male who presented with indulation of his left scrotum for 10 months. The plain computerized tomographic (CT) scan on entry demonstrated tumor metastasis to his lung and liver and serum alpha-fetoprotein (AFP) level was 786 ng/ml. Five days after admission, he developed hemiplegia secondary to the cerebral metastasis and hemorrhage. After chemotherapy and operation of right-posterior lobectomy, PVB (cisplatinum, vinblastine, bleomycin) chemotherapy produced a complete remission and the elevated serum AFP was normalized. However, the second course of chemotherapy had to be discontinued because of drug-induced hepatitis. He died of massive tumor metastasis to his brain 6 months after craniotomy.

Primary yolk sac tumor of the cerebellar vermis: case report.

A rare case of yolk sac tumor in the cerebellar vermis is reported. A 2-year-old boy developed headaches, vomiting, and an unsteady gait. Later a tumor was demonstrated in the medial part of the cerebellum by gadolinium-enhanced magnetic resonance imaging (MRI). The tumor was totally removed, and the surgery was followed by chemotherapy. Soon after surgery the elevated alphafetoprotein (AFP) levels in the serum and cerebrospinal fluid were observed to decrease to normal levels. Three months later enhanced MRI showed a lesion in the vermis without any elevation of AFP, and the lesion turned out to be a granuloma. Six months after the second surgery a tumor recurred that could not be totally removed. Cranial radiotherapy was given together with chemotherapy, which resulted in a decrease of AFP to the normal range. The patient is doing well without any elevation in AFP at 1 year 6 months after onset. Related problems in the diagnosis and treatment of yolk sac tumors are discussed.

Brain metastases and testicular tumors: long-term survival.

In this updated and expanded retrospective analysis, the treatment records of 24 patients with brain metastases from nonseminomatous germ cell testicular tumors (NSGCT's) treated at the Indiana University Department of Radiation Oncology from 1975 through 1988 were reviewed. All patients received standard cisplatin-based induction chemotherapy. These patients were divided into three groups. Group 1 (n = 10) consisted of patients who presented initially with brain metastases and had no prior systemic treatment. Group 2 (n = 4) consisted of those patients who, after achieving a complete response (CR) with cisplatin, vinblastine, and bleomycin (PVB) +/- doxorubicin, developed a relapse confined to the brain. Group 3 (n = 10) consisted of those patients who were initially treated with PVB +/- doxorubicin or bleomycin, etoposide, and cisplatin (BEP) and eventually developed progressive disease and brain metastases. Group 1 was treated with whole brain irradiation (WBRT) and PVB +/- doxorubicin or BEP. Group 2 was treated with WBRT, cisplatin-based chemotherapy +/- surgical excision. Group 3 was usually treated with WBRT palliatively. Six patients, three in Group 1 and three in Group 2, are alive and disease-free with follow-up of 5+ years from beginning WBRT. Two additional patients in Group 1 survived 5+ years from beginning WBRT before dying with disease. No patient in Group 3 survived. Patients with brain metastases who have potentially controllable systemic disease should be treated curatively with WBRT (5000 cGy/25 fractions) +/- surgical excision and concomitant chemotherapy.

Mediastinal yolk sac tumor. The Indiana University experience, 1976 to 1988.

Mediastinal yolk sac tumor (endodermal sinus tumor) is an extremely rare extragonadal germ cell neoplasm that has been associated with a grave prognosis. Twenty-one male patients with mediastinal yolk sac tumor received treatment at Indiana University between 1976 and 1988. Fourteen were seen after initial diagnosis, and their disease was treated with cisplatin-based chemotherapy in association with complete surgical resection if possible. Five are currently alive and disease free (36%). Seven were referred for salvage chemotherapy after relapse of their disease. Despite aggressive chemotherapy, these patients all died; they had a median survival time of 6 months. Our experience suggests that an aggressive combined modality approach with cisplatin-based chemotherapy followed by surgical resection of residual disease is the optimal management of this tumor. New regimens need to be explored for relapse of the disease after initial chemotherapy.

[Clinical observations and the planning of therapy for sacrococcygeal tumors].

Amongst sacrococcygeal tumors, teratoma is seen often while other histologic types are rare. It is apparent that the less obvious intrapelvic tumors can cause obstructive symptoms and are usually diagnosed late. Twenty cases of sacrococcygeal tumor, including 14 teratomas (9 benign, 5 malignant) and 6 others (3 benign, 3 malignant) were treated at Tokyo Medical College and Shizuoka Children's Hospital over the last 23 years. The majority of the benign tumors were readily excised, but one case of benign teratoma was difficult to excise and died of bleeding. The resectability of malignant tumors was low, but aggressive multiagent chemotherapy, radiotherapy and second look operation made it possible to increase the survival. One case is alive and has been disease free for 11 years. Recently combined therapy by chemotherapy and surgery has improved the survival rate although some problems still remain.

Endodermal sinus tumor of the ovary: a case series with flow cytometric DNA content analysis.

Endodermal sinus tumors (EST) are rare germ cell ovarian malignancies occurring primarily in young women. A retrospective review of the Magee-Womens Hospital tumor registry revealed eight cases of pure EST and two mixed tumors in which the EST component was predominant. Mean patient age was 18.2 years. Abdominal pain was the most common presenting symptom and a pelvic mass was palpable in all patients. Four patients are currently alive and well with no evidence of disease. All were treated with surgery and combination chemotherapy. Flow cytometric DNA content analysis of paraffin-embedded tumor tissue identified similar aneuploid cell populations in three of five tumors analyzed with relative peak positions of 1.72, 1.62, and 1.70. The management of women with endodermal sinus tumor remains controversial with regard to type of chemotherapy employed and the use of second-look laparotomy. The prognosis role of flow cytometric DNA content analysis is yet to be determined.

Successful treatment of a pineal endodermal sinus tumor. Case report.

A patient with a pineal endodermal sinus tumor is presented who was successfully treated by a combination of surgery, adjuvant chemotherapy, and craniospinal irradiation. Two years after diagnosis, he is free of any disease. A review of the literature shows that such an outcome is very unusual. A multidisciplinary treatment is recommended for this rare tumor, using chemotherapy as adjuvant treatment.

Long-term follow-up of children with testicular tumours: surgical issues.

Testicular tumours are rare in childhood. The clinical, pathological and follow-up details of 48 children are presented. The combined approach of surgery, chemotherapy and radiotherapy is confirmed as being extremely effective. Transcrotal orchiectomy is not an appropriate surgical procedure. Testicular biopsy is detrimental to prognosis.

Treatment of malignant ovarian germ cell tumors with preservation of fertility: a report of 28 cases.

Preservation of fertility was carried out in 28 young patients with malignant germ cell tumors of the ovary from 1962 through 1987. The pathologic diagnosis was immature teratoma in 16, endodermal sinus tumor in 7, dysgerminoma in 2, and germ cell tumor of mixed type in 3. At laparotomy, 16 patients were proved to have stage I disease, 1 had stage II disease, 9 had stage III disease, and 2 had stage IV disease. The tumor was confined to one ovary in all 22 cases treated for the first time, and the preserved ovary and uterus were normal in all 6 referred cases with recurrent diseases. Postoperative chemotherapy was given to all patients except two with stage I immature teratoma. Persistent remission was achieved in 22 patients; 5 patients died and 1 was lost to follow-up. The duration of follow-up was more than 1 year for all surviving patients, more than 3 years in 16 cases (72.7%), and more than 5 years in 13 cases (59.1%). The menstrual periods were normal in all except 3 cases, of whom 2 were below the age of 12 and one failed to menstruate at the age of 21 with a hypoplastic uterus and underdeveloped secondary sex characters. Among 12 married patients, 7 of 10 desirous of child-birth became pregnant during follow-up. Of these, 6 had a normal term delivery and 1 is currently pregnant. The preliminary conclusion is that preservation of fertility for young patients with malignant germ cell tumors of the ovary, regardless of the stage of the disease, is a safe and practicable procedure in the absence of involvement of the contralateral ovary and uterus.