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The clinical and pathologic features of a vulvar neuroendocrine (Merkel cell) neoplasm are presented. Cytologic studies of material obtained from needle aspiration suggested that the tumor was a small cell neoplasm possibly of neuroendocrine derivation. The light-microscopic findings of sheets of small, uniform cells were consistent with a diagnosis of neuroendocrine tumor. The electron-microscopic characteristics, including the presence of neurosecretory granules, confirmed the diagnosis of a neuroendocrine (Merkel cell) carcinoma. Regional lymph node metastases were present at the time of initial surgery, and both local and distant metastases developed 8 months later. A comprehensive pretreatment metastatic evaluation is recommended. The role of chemotherapy for primary therapy is considered.

Bartholin gland tumors are rare and management recommendations have been based on limited information. This report summarizes a 30-year clinical experience involving Bartholin gland carcinoma in 36 patients whose five-year survival rate was 84%. FIGO stages of the 36 tumors were stage I, nine; stage II, 15; stage III, ten; and stage IV, two. Cell types were: squamous, 27 (three nonkeratinizing with areas of a transitional component); adenomatous, six; adenoid cystic, two; and adenosquamous, one. Fourteen of 30 (47%) patients with lymph node dissections had nodal metastases and 11 remain disease-free. Disease recurred in nine patients (six local, two distant, one local and distant) and four were treated successfully. One of 14 (7%) patients receiving radiation and six of 22 (27%) patients not receiving radiation developed local recurrences. Wide excision (often necessitating a radical hemivulvectomy), ipsilateral inguinal lymphadenectomy, and adjunctive irradiation to the vulva and regional lymph nodes produced excellent results.

Eight cases of alveolar rhabdomyosarcoma of the female genitalia were diagnosed from 1963 to 1983 at The University of Texas M. D. Anderson Hospital. The primary sites were vulva in two, perineum in five, and broad ligament in one patient. When possible, therapy was initiated with local tumor excision (five patients). Surgery was followed by local or regional radiation (six patients) and chemotherapy (seven patients). Of the eight patients, five died within 9 months, one died 27 months after diagnosis, and only two are 5-year survivors. The aggressive behavior of this tumor is evidenced by autopsy findings of widespread metastases. Metastatic disease to the bone was present in four patients and to the breast in three patients. Local disease was controlled in two patients who died of distant metastases. Current therapy recommendations include excisional surgery, local radiation, and combination chemotherapy. A need for more effective chemotherapeutic programs is evident.

The flow cytometric measured DNA content (i.e., DNA index), S-fractions, and histopathologic malignancy grades were studied for ninety uterine cervical squamous cell carcinomas using tissue biopsies taken prior to radiotherapy. The DNA aneuploidy frequency for low S-phase tumors (%S less than 14) was only 9/29 (31%) compared to 22/30 (73%) for intermediate (%S 15-23) and 30/31 (97%) for high (%S greater than 24) tumors. An overall mean %S of 20 +/- 7 (range 2-45%) was observed for these cervical cancers, with the S-fraction significantly increasing (P = less than 0.01) from 12 +/- 5, to 18 +/- 8, and 26 +/- 7 for diploid/near diploid, low-degree DNA aneuploidy, and high-degree DNA aneuploidy tumors, respectively. Broad heterogeneity was observed for the microscopic scored malignancy grades within DNA index and the cell-cycle S-fraction subgroups. Generally, multifactorial histopathology scoring was not significantly correlated with either the tumor DNA index or %S variables. Based on statistical analysis, the malignancy grades more closely reflected the tumor proliferative activity than the DNA index, with nuclear polymorphism, mitotic frequency, and the invasion pattern showing the lowest P values (which were not significant at P = 0.05). High tumor S-fraction was associated with high malignancy grade, as evidenced by 19/25 (75%) of high S-fraction, high degree DNA aneuploidy tumors having greater than average malignancy grade compared to only 2/14 (14%) low to moderate S-fraction tumors having similar DNA index. The results indicate that more reliable identification of biologically different cervical cancers can be achieved by evaluating the tumor DNA index in relationship to the cell-cycle %S and malignancy grading.

The cytologic findings in peritoneal washings of two women, one of whom had an ovarian ependyoma and the other a primary omental ependymoma, are reported. The ependyomas were characterized by the presence of numerous, isolated, spindle and stellate cells as well as groups of cells forming true rosettes. The tumor cells displayed slightly pleomorphic, round-to-oval eccentric nuclei and abundant fibrillary cytoplasm with tapering cytoplasmic processes. In addition, one of the patients had numerous papillae and cell clusters with associated psammoma bodies indistinguishable from those found in low-grade serous carcinoma. The demonstration of glial fibrillary acidic protein (GFAP) in both cases by immunocytochemical procedures indicates the usefulness of this method in cytologic preparations to confirm the diagnosis of these uncommon neoplasms. Diagn Cytopathol 1986;2:62-68.

Periodic structures with equally spaced radial striations identified as Liesegang-like rings were seen in two male patients' hemorrhagic perirenal cysts. The patients, one 48 and the other 60 years old, had acute right-flank pain and anemia; both had nephrectomy. The rings, initially believed to represent parasites (Dioctophyma renale), were from 8 to 500 micron in diameter and had uniform, pink-tan, radially striated double walls. Multiple small rings within a larger ring predominated in one case. Morphologically, the rings differed from D. renale when compared with specimens from animals infected naturally or experimentally with the giant kidney worm. Histochemical and immunoperoxidase tests for iron, calcium, mucopolysaccharides, amyloid, keratin, and hemoglobin had negative results. Energy-dispersive x-ray elemental analysis demonstrated no detectable elements; ultrastructurally, however, the rings displayed a fine fibrillary composition with a concentric and radial pattern. These rings are believed to be an end product of a phenomenon resembling or are, in fact, the Liesegang phenomenon. Because these Liesegang-like structures may be mistaken for parasites on fine-needle aspiration or surgical specimens of hemorrhagic areas, pathologists should be aware of them.

An 8-year-old child with nevoid basal-cell carcinoma syndrome who developed abdominal pain underwent exploratory laparotomy. Both ovaries were enlarged and replaced by fibroblastic proliferations having cellular foci with high mitotic indices (greater than 4 mitoses/10 high-power fields) diagnostic of fibrosarcoma. Two years following salpingo-oophorectomy, a metastasis was excised from one adnexa. Further recurrence or distant metastasis was not evident after 6 more years of follow-up. The association of fibrosarcoma of the ovary in a patient with nevoid basal-cell carcinoma further expands the multifarious nature of this syndrome.

This report describes six patients with endodermal sinus tumor of the vagina and cervix, a polypoid friable tumor whose clinical presentation in girls younger than age 3 years simulates the presentation of sarcoma botryoides. In four of the six patients, the referring diagnosis was sarcoma botryoides. Five patients were treated with excisional surgery, and all six with chemotherapy. One patient with pulmonary metastases maintained a complete clinical response to vincristine, actinomycin-D, and cyclophosphamide (VAC) for 11 months. This is the first report of such a response. One patient with a vaginal lesion remains clinically free of disease 2 years after local excision and 18 months of VAC chemotherapy. This is the first report of apparently successful therapy that allowed retention of childbearing potential. Four of the six patients have been disease-free from 2 to 23 years. After examining the world literature, it is concluded that a combination of chemotherapy and surgery offers a reasonable prospect of cure with a minimum of serious side effects.

From January 1971 through December 1981, 246 patients with advanced (Stages III and IV) epithelial ovarian cancer underwent second-look laparotomy at The University of Texas M. D. Anderson Hospital and Tumor Institute at Houston. Eighty-five of these patients had a complete response (negative second-look laparotomy) following treatment with a variety of chemotherapeutic regimens. Three patients had also received irradiation. Patients were analyzed according to pretreatment characteristics (age, FIGO stage, ascites, pleural effusion, histologic grade, tumor type, type of surgery, residual tumor diameter, initial clinical status) and by the number of biopsy specimens taken at second-look laparotomy. The probability of recurrence and the length of survival following a negative second-look laparotomy are statistically related to these characteristics. Twenty of the 85 patients (24%) developed recurrent disease 5 to 32 months after laparotomy. The estimated 2- and 5-year survival rates are 99% and 85%, respectively. Patients who achieve a surgically determined complete response have an excellent chance for long-term survival.

Twenty cases of metastatic neoplasms in the breast were identified in a series of 1,034 fine-needle aspirations (FNAs) of the breast, of which 389 were malignant. Patients with breast carcinomas in whom metastasis to the contralateral breast developed were excluded from this study. This series consisted of 17 women and 3 men, ranging in age from 28 to 63 years (mean, 49 years). The tumors included oat cell carcinoma (three), melanoma (three), ovarian serous carcinoma (one), bronchogenic adenocarcinoma and squamous carcinoma (four and two, respectively), lymphoma (two), carcinoid (two), transitional cell carcinoma (one), plasma cell myeloma (one), and rhabdomyosarcoma (one). In two patients, the breast mass was the first manifestation of an extramammary cancer (two adenocarcinoma of the lung). Eleven patients died of disseminated cancer shortly after the breast metastasis was diagnosed. In most cases, the aspirates displayed the cytologic features characteristic of the primary tumors, thereby establishing the metastatic nature of the neoplasm. In four cases (two carcinoids, one myeloma, and one rhabdomyosarcoma), the cytologic features were difficult to differentiate from a primary breast carcinoma; however, the final diagnosis was established by electron microscopic examination and immunocytochemical studies on the aspirates. One case (adenocarcinoma of the lung) was misdiagnosed as primary breast carcinoma on both FNA and mastectomy specimen. Because metastatic neoplasms in the breast may mimic primary breast tumors, the authors recommend the following: (1) Evaluation of FNA of breast should be done with complete knowledge of the patient's clinical history. (2) The possibility of metastasis should be suspected in lesions with unusual cytologic patterns. (3) Ancillary studies on FNA can be helpful in interpreting selected cases.

Four oat cell carcinomas of the urinary tract (three of the bladder and one of the ureteropelvic junction) were studied using immunohistochemical and electron microscopic methods to evaluate the possible histogenesis of these tumors. Ultrastructurally, the oat cell component of the tumors had neurosecretory-type granules and positive immunoreaction for neuron-specific enolase, indicating the endocrine nature of the tumors. Three tumors were mixed with a non-oat cell component: adenocarcinoma and/or transitional carcinoma or carcinoid tumor. There are no previous reports of mixed oat cell and adenocarcinoma or carcinoid tumor of the urinary tract. This report is also the first of an oat cell carcinoma originating in the pelvic ureteric junction.

Ewing's sarcoma is a distinctive clinical and pathologic entity that may be intraosseous or of soft-tissue origin. In the facial bones, the incidence of the sarcoma is estimated at 2.5% of all Ewing's sarcoma of bone. The soft tissues of the head and neck account for 11% of extraskeletal sites of the neoplasm. Combined modality treatment has modified its high-grade malignancy.

The end product of the Liesegang precipitation phenomenon can produce microscopic bodies that can be and have been mistaken for parasites in human tissues. The Liesegang bodies have been recently described and defined in and about hemorrhagic and cystic lesions of the kidney. The present authors report the first instance of these bodies in the paranasal sinuses.

The spread of cervical carcinoma associated with parturition through a tumorous cervix is rarely demonstrated. This report details two patients with adenocarcinoma of the cervix who delivered term fetuses vaginally and subsequently presented with tumor nodules in the episiotomy scars. Although both patients appear to have had their recurrences treated successfully, follow-up on one patient has been only 10 months since the completion of therapy. Tumor implantation of the episiotomy wound is a potential risk associated with vaginal delivery in a pregnant patient with cervical carcinoma. However, this iatrogenic mechanism of tumor spread may permit an opportunity for successful treatment of the local recurrence.

Sinus histiocytosis with massive lymphadenopathy is a lymphoreticular disorder of obscure pathogenesis with extranodal lesions and a variable clinical course. Clinical manifestations are most often in the head and neck, with 97% of patients showing cervical lymphadenopathy and a significant but lesser number having lesional infiltrates in the upper aerodigestive tracts, particularly the nasal cavity and salivary glands.

Cytogenetic analysis by QFQ-banding of direct preparation of a testicular mass from a patient with secondary lymphoma revealed a modal chromosome number of 45,XY, including structural and numerical anomalies. The most consistent anomalies were the monosomy 21, duplication of the long arm of #11, and structural anomaly associated with chromosome #1 in band q21.

• Paraganglionic tumors of the larynx are uncommon neoplasms and their secretory products have rarely been elucidated. We saw two patients whose laryngeal paragangliomas were examined with an electron microscope and by the use of immunocytochemical techniques. Both tumors manifested positive immunoreactivity against calcitonin and vasoactive intestinal polypeptide. One of the paragangliomas was immunoreactive against carcinoembryonic antigen and pursued a biologically malignant course. Our observations suggest the coexistence of amines and hormonal polypeptides in cells of paraganglionic tumors and demonstrate the multihormonal nature of these tumors. ( Arch Otolaryngol 1983;109:113-117)

Pancreatic endocrine tumors (PETs) are relatively uncommon neoplasms. Although their histologic patterns have been widely studied, their cytologic features as they appear in fine-needle aspiration (FNA) specimens have rarely been reported. In this study, aspirates of seven PETs, four primary and three metastatic lesions (two to liver and one to bone), are described. The tumors occurred in seven men ranging in age from 37 to 72 yr. Six tumors presented as nonfunctioning masses and one produced Zollinger-Ellison syndrome. Three were located in the head of the pancreas and four in the body and tail. The pancreatic and liver aspirations were performed under computed tomographic guidance and the bone lesion, under fluoroscopy. The aspiration specimens were hypercellular. The tumor cells occurred singly and in small clusters. In three cases, there was a tendency toward acinar formations. In two cases, there were prominent, thin-walled, branching blood vessels with tumor cells attached to the vascular walls. The cells were round or polygonal with a moderate amount of finely granular, well-defined cytoplasm. The nuclei were eccentrically located and round-to-oval—with one or two small nucleoli and finely granular, evenly-dispersed chromatin. The diagnosis was confirmed by immunocytochemistry (two cases) and electron microscopy (four cases) of the aspirated material and histology sections of the resected tumors (two cases). The results of this study demonstrate that FNA is a useful method to establish the diagnosis of PETs. Diagn Cytopathol 1987;3:35–40.

The cytologic features of fine-needle aspirates (FNA) of two hepatoblastomas (HBs) and a liver-cell carcinoma (LCC) occurring in three children, ages 1, 3, and 14 yr, are presented. Electron microscopic features of one of the HBs and the LCC are also included. The HBs displayed single or small aggregates or larger clusters of cells with a high nuclear-cytoplasmic ratio and usually round, hyperchromatic nuclei with single, and occasionally double, prominent nucleoli. In addition, one patient had extramedullary hematopoiesis; both had osteoid-like material. These findings were very helpful in differentiating the HB cases from the LCC case, which had features similar to those of adult hepatocytes, including the presence of abundant, finely granular cytoplasm. Ultrastructural studies showed an absence of differentiation into adult hepatocytes in the HB, with scant organelles, rare bile canaliculi, lakes of glycogen, and rare lysosomal bodies. In contrast, the LCC showed features similar to adult hepatocytes, such as abundant cytoplasmic organelles, lysosomes, and bile canaliculi. In addition, intranuclear pseudoinclusions caused by cytoplasmic invagination were present in the LCC but not in the HB. Fine-needle aspiration of hepatic masses in children is a useful technique for rendering a diagnosis, especially in unresectable tumors. Ultrastructural studies on FNA material may be important in differentiating hepatoblastoma from hepatocellular carcinoma.

We report a patient in whom fine-needle aspiration biopsy of a breast mass, clinically suspected to be a mammary adenocarcinoma, established the diagnosis of a carcinoid metastatic to breast. The aspirate smears resembled a primary breast carcinoma; however, subtle cytomorphologic differences were present. Ultrastructural and immunohistochemical studies of the aspirated material confirmed the diagnosis of metastatic carcinoid.

The involvement by a disease process of the parapharyngeal and retropharyngeal spaces poses significant diagnostic and therapeutic problems. Neoplasms, the majority histologically benign, comprise the greatest number of parapharyngeal space lesions. Inflammatory lesions and metastases to lymph nodes are the preponderant disorders affecting the retropharyngeal space.

A primary pure testicular carcinoid from a 48-year-old man was examined with antisera raised against various neurohormonal polypeptides. Histochemically, both argyrophil and argentaffin reactions were positive. The immunohistochemical finding of immunoreactive cells for 5-hydroxytryptamine (5-HT) (serotonin), substance P, and vasoactive intestinal polypeptide (VIP) demonstrated the multihormonal nature of this tumor.