Primary squamous cell carcinoma of the endometrium: a case report.

BACKGROUND: Primary endometrial squamous cell carcinoma (PSECC) is a rare neoplasm. Squamous epithelium derived from endometrioid cancer or from cervical squamous cell carcinoma. The prevalence is about 0.1%. The genesis, histogenesis and biological behavior are unknown. CASE PRESENTATION: A 48-year old woman in postmenopause, referring pelvic pain and vaginal bleeding. Transvaginal ultrasound showed a bulky uterus with the endometrium containing an hyperecogenic area. Endometrial biopsy found an epidermoid carcinoma. MRI showed a 4 x 2 x 1.2 cm mass occupying the uterine cavity. The patient underwent radical treatment. Pathological examination showed features of PSECC. The mutation of p53 tumor suppressor protein was disclosed in 15% of neoplastic cells. PCR revealed the absence of HPV DNA. CONCLUSIONS: The findings of our case move us to underline that the pathogenesis of this tumor is still unclear. Moreover, preoperative diagnosis and staging of PESCC is extremely difficult. Most patients do not show characteristic symptoms and predisposing factors, making it almost impossible to diagnose the precise localization of tumor origin.

Ovarian metastasis following gallbladder carcinoma: a case report.

BACKGROUND: Mucinous ovarian cancer raises problems of differential diagnoses because it is often difficult to distinguish the primary from the metastatic form. Most metastatic ovarian tumors originate from the gastrointestinal tract, mainly colorectal, gastric, pancreatic; the gallbladder is a very rare source of ovarian metastases. CASE: We report a case of ovarian metastases from a gallbladder cancer, incidentally diagnosed more than 2.5 years earlier during a laparoscopic intervention for biliary lithiasis. CONCLUSION: The interest of this case lies in the long progression-free survival, the venous thromboembolism syndrome that preceded by a few months the diagnosis of the ovarian mass and the discrepancy between the radiologic and the laparoscopic stage assessment.

Extrapleural solitary fibrous tumor: a clinicopathologic study of 19 cases.

This study reports a series of 19 extrapleural solitary fibrous tumors. The patients included 6 men and 13 women with age ranging from 27 to 86 years. Three patients showed local recurrence. In 2 tumors, a diagnosis of malignancy was made. All of the tumors were strongly positive for CD34, and 3 of them expressed high levels of progesterone receptor. Solitary fibrous tumors are fairly rare, occurring in many parts of the body, and their behavior is unpredictable.

Huge primary retroperitoneal mucinous cystadenoma of borderline malignancy mimicking an ovarian mass: case report and review.

BACKGROUND: Primary retroperitoneal mucinous cystadenoma is a rare tumor only 48 cases have been reported in international literature. Patients affected by primary retroperitoneal mucinous cystadenoma/cystadenocarcinoma ranged in age from 17 to 86 years (median, 42.3 years) and the size of the cystis ranged from 5 to 35 cm (median, 16.1 cm). There is no unanimous opinion on the genesis of these tumors and, due to their extreme rarity, its histogenesis, biological behavior and the optimal management strategy remain at a speculative level. CASE REPORT: We report the case of a huge borderline primary retroperitoneal mucinous cystadenoma (24 x 25 cm) in a 35-year-old woman and the strategies adopted for the diagnosis and surgical management. CONCLUSION: Primary mucinous cystic tumor of the retroperitoneum was correctly diagnosed only at the time of surgery. As well as in the majority of cases reported in the literature, preoperative investigations were not able to give information about the tumor site. In spite of the short follow-up (two years), the patient's favorable course supports the hypothesis that primary retroperitoneal mucinous cystadenoma may be treated in the same manner as a primary ovarian tumor of the same grade and comparable stage.

Cavernous haemangioma of the temporalis muscle: case report and review of the literature.

Haemangiomas are benign vascular neoplasms characterized by an abnormal proliferation of blood vessels. They may occur in any vascularized tissue including skin, subcutaneous tissue muscle and bone. These tumours are common in infancy and childhood and commonly involve subcutaneous or mucosal tissues. Intramuscular haemangiomas, a distinctive type of haemangioma occurring within skeletal muscle, account for less than 1% of all haemangiomas. They occur more often in trunk and extremity muscles, whereas involvement of the temporal muscle is extremely rare. Herein, the case is reported of a 38-year-old male who presented with a round, painless mass in the left temporal fossa, which was interpreted as an intramuscular haemangioma after a magnetic resonance imaging scan. In this report, clinico-pathological findings are described in an additional case of haemangioma involving the temporal muscle, and a review is made of the international literature on this subject.

Primary signet-ring cell carcinoma of the uterine cervix with long term follow-up: case report.

BACKGROUND: The uterine cervix represents an exceptional localization of signet-ring cell adenocarcinoma (SRCA). Most commonly, endocervical tumors with such morphology are metastatic from the breast or gastrointestinal tract while primary pure or almost pure tumors are extremely rare. No previous case of primary pure endocervical SRCA with follow-up longer than three years has been found in the literature. CASE REPORT: The present report describes such a case of a 46-year-old woman without evidence of recurrence eight years after the diagnosis. The patient was referred to the Gynecology Department for persistent abnormal vaginal bleeding of three months duration. Specular examination and colposcopy revealed a cervical polypoid lesion occupying the posterior lip of the cervix and protruding from the external uterine orifice. A biopsy of the lesion was interpreted by the pathologist as SRCA. An extensive search for an extrapelvic primary cancer was undertaken but revealed no evidence of malignancy. The patient underwent radical hysterectomy with bilateral salpingo-oophorectomy and pelvic and paraaortic lymph node sampling for FIGO Stage 1B1 cervical cancer without any adjuvant chemo- or radiotherapy. The histological diagnosis showed neoplastic signet-ring cells confined in the head of the cervical polyps with minimal stromal infiltration. After surgery the patient underwent close followup including periodic cervicovaginal smears, bimanual vaginal examination, complete laboratory tests, ultrasound and abdominopelvic computed tomography. CONCLUSIONS: The prognostic relevance of primitive pure SRCA in the uterine cervix is unclear because of the relatively small number of cases. However the two early deaths out of six reported cases and the absence of follow-up longer than three years for the other affected patients, seem to suggest an aggressive behavior. The present case represents an exceptional long-term survival, probably related to an early diagnosis and a prominent polypoid growth of the neoplasia outside the uterus.

Chondromyxoid fibroma of the zygoma: a case report.

Chondromyxoid fibroma is a rare benign tumour of chondral origin. It usually involves the long bones of the lower extremity, whilst involvement of craniofacial skeleton is extremely unusual. The second case of chondromyxoid fibroma of the zygoma described in literature is presented and the surgical resection of the lesion with tumour-free margins as the key factor for avoiding local recurrence of this tumour is emphasised.

Submandibular gland myoepithelioma.

Benign myoepithelioma is a very rare form of salivary gland tumor, composed entirely of myoepithelial cells. It accounts for approximately 1% of all salivary gland tumors and is most frequently located in the parotid gland and in the minor salivary glands of the hard palate. We describe herein the ninth reported case of myoepithelioma of the submandibular gland. Benign myoepithelioma must be differentiated from several benign and malignant epithelial and mesenchymal tumors. Immunohistochemical staining can help differentiate between these conditions, but histopathology remains the gold standard for diagnosing this neoplastic process.

Plasma cell granuloma (inflammatory pseudotumor) of the breast.

The first case of plasma cell granuloma (PCG) of the breast is presented. The lesion was seen as a circumscribed firm mass in the right breast of a 29-year-old woman. Histologically, a prominent plasma cell infiltrate was seen in a fibrous hyalinized stroma that contained spindle- and large stellate-shaped stromal cells with bizarre nuclei. Immunohistochemical study demonstrated the polyclonal nature of the plasma cells and characterized the stromal cells as myofibroblasts. The nature of these cells was confirmed ultrastructurally. PCG of the breast should be considered analogous to inflammatory pseudotumors of the lung and extrapulmonary sites.

Intravascular B-cell lymphoma: report of two cases with different clinical presentation but rapid central nervous system involvement.

Intravascular lymphomatosis (IVL) is a rare large-cell lymphoproliferative disorder characterized by a widespread lymphoma proliferation within the lumen of medium and small vessels, frequently presenting with skin and/or central nervous system (CNS) manifestations. The tumor is of B-cell origin in most cases. Prognosis is poor with a reported median survival of 5-7 months. We describe here two cases of IVL. The first was that of a 55-year-old woman with a large B-cell lymphoma of the leg, successfully treated with conventional chemotherapy (CHT) followed by autologous peripheral stem cell transplantation. At 3 months from the autograft she relapsed with a picture of hemophagocytic syndrome (HPS) and CNS symptoms. She died before any specific treatment, and post-mortem examination revealed the intravascular proliferation of lymphoma B-cells in the brain and bone marrow. The second case was that of a 60-year-old male with CNS involvement at diagnosis. He responded poorly to CHOP-like CHT, and died 2 months after diagnosis and 6 months after onset of symptoms. Failure of CHT at least in some IVL patients may be related to a delay in the initiation of therapy due to non-specific neurological symptoms. Therefore, early diagnosis based upon aggressive attempts immediately followed by adequate therapy may prove beneficial to these patients. In the present report, we performed an extended medline-based review of the published series of patients with IVL.

Epithelioid haemangioendothelioma of the breast.

This article describes histopathological, immunohistologic, and morphometric features of an epithelioid haemangioendothelioma that presented in the breast of a 30-year-old woman. Histologically the tumour was composed of large round, polygonal, slightly spindle shaped endothelial cells lying in a myxoid matrix. The lesion exhibited primitive vasoformation characterized by the presence of cytoplasmic vacuoles. The tumour cells reacted positively to F-VIII related antigen, whereas mammary ducts entrapped by the tumour cells reacted positively with keratin. Although the breast is an unusual location for this type of lesion, epithelioid haemangioendothelioma should be considered a rare differential diagnosis of a breast mass.

Aggressive cemento-ossifying fibroma of the jaws.

The cemento-ossifying fibroma is probably the most frequent fibro-osseous lesion seen by oral pathologists. Occasionally, reports of lesions behaving in an aggressive fashion appear in the literature. At the present time, the relationship if these "aggressive" lesions to the "usual" fibromas is unclear. Two cases of "aggressive" cemento-ossifying fibroma are reported. Certainly, clinical, radiological, and histological characteristics of aggressive and usual fibromas do coincide. There are, however, diverging features warranting separation of different forms. These mild differences may be detected during preoperative assessment of the lesion, allowing a diagnosis of aggressive form to be made.

Endometriosis of the bowel with lymph node involvement. A report of three cases and review of the literature.

Three cases of endometriosis of the bowel with lymph node involvement were studied. Histologically, endometriotic glands with typical stroma were recognized throughout the intestinal wall, from the subserosal tissue to the mucosa. Foci of endometriosis were found in a number of pericolic lymph nodes occupying the subcapsular area or the cortex. The cytological smears were cellular, composed of large sheets of mucous columnar cells of intestinal origin, mixed with groups of recognizable endometrial cells and smaller spindly cells of endometrial stroma. Immunohistochemically, endometriotic cells showed positivity for cytokeratin and EMA, and they retained immunoreactivity for estrogen and progesteron in the intestinal lesions, as well as in the lymph nodes. Lymph nodal endometriosis was disclosed in two out of three cases only by multistep section examination of the pericolic lymph nodes, previously reported as negative for this feature.

Alveolar variant of infiltrating lobular carcinoma of the breast with stromal osteoclast-like giant cells.

An alveolar variant of infiltrating lobular carcinoma of the breast in which a prominent feature was the presence of numerous osteoclast-like giant cells in a richly vascularized stroma is described. A similar stromal reaction has previously been reported in 46 breast carcinomas of various histologic types, but only once in a pure infiltrating lobular carcinoma. The fine needle aspiration cytological and histological appearances and the immunohistochemical and ultrastructural features of this tumor are reported. Our study confirms the histiocytic nature of the multinucleated giant cells and their similarity to normal osteoclasts.

Adenomyoepithelioma of the breast. A case report.

Adenomyoepithelioma is a proliferative disorder of both epithelial and myoepithelial cells. This lesion may be found in salivary glands, skin appendages and, very rarely, in the mammary gland. Adenomyoepithelioma was first described in 1970 and very few cases have so far been reviewed in the literature. This paper reports the clinical, histological and immunohistochemical characteristics of an adenomyoepithelioma in a 24 year old woman; to our knowledge this is the first published case in such a young patient. The clinical feature suggested a fibroadenoma. A more complete study of the excised tumor tissue by immunohistochemical and ultrastructural analysis proved that the correct diagnosis was adenomyoepithelioma. Whether adenomyoepithelioma is a benign or a low-grade malignant lesion is still controversial and, therefore, the therapeutic approach is not well defined.

A calcitonin-producing neuroendocrine tumor of the larynx: a case report.

A moderately differentiated neuroendocrine carcinoma of the larynx is described. Although the patient had normal serum calcitonin level 36 months before, its level was elevated at the time the neck node was removed. This study supports the hypothesis that a diagnosis of calcitonin-producing neuro-endocrine tumor of the larynx with increased plasma calcitonin is possible and should be considered to avoid unnecessary thyroidectomy.

Sarcomatoid carcinoma of the colon: a case report with literature review.

Sarcomatoid carcinoma (SC) is a well defined tumor type that may occur in all organs and anatomic sites but most commonly in the head, neck, respiratory tract, breast, and genitourinary tract. It is a biphasic tumor showing both epithelial- and mesenchymal-like differentiation; however, its carcinomatous nature is widely recognized. SC is rare in the gastrointestinal tract. In the esophagus it accounts for less than 5% of all malignancies and approximately only 35 cases have been described in the stomach. Very few cases have been observed in the small intestine, anorectal junction, liver, and pancreas. To our knowledge only eight cases of SC have been reported in the colon. We report a case of primary colonic SC. Both morphological and immunohistochemical analyses are provided along with an evaluation of the unusual clinical history, therapeutic implications, and controversial differential diagnosis.

Fine-needle aspiration cytology of angiolymphoid hyperplasia with eosinophilia: a case report with electron microscopy and immunohistochemistry.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon vascular inflammatory lesion usually involving the dermis or subcutaneous tissue of the head-neck region of middle-aged women. Histologically, this lesion shows a florid proliferation of vessels lined by particular endothelial cells and an inflammatory infiltrate composed of lymphocytes and eosinophils. The fine-needle aspiration (FNA) cytology of a nodular lesion in the retroauricolar region of a 18-yr-old woman showed spindle-shaped and polygonal cells with vesicular nuclei and deeply eosinophilic cytoplasm containing well-defined vacuoles in a background of eosinophils and lymphocytes. These features were consistent with a proliferation of epithelioid endothelial cells, and a diagnosis of ALHE was suggested. The histology confirmed the preoperatory diagnosis, and ultrastructural and immunohistochemical studies further demonstrated the endothelial nature of epithelial-appearing cells. Because the clinical appearance of the lesion may mimic a large number of benign and malignant diseases, a preoperatory diagnosis of ALHE is rarely made. The FNA cytology may represent a simple and reliable method with which to study and diagnose proliferations of epithelioid endothelial cells.

Undifferentiated small round-cell tumors of childhood: the immunocytochemical demonstration of myogenic differentiation in fine-needle aspirates.

The recognition of myogenic differentiation is not always possible using routine light or electron microscopic techniques. In this article, we describe our experience with two small round-cell neoplasms occurring in young children, each of which exhibited an undifferentiated light microscopic appearance. In both cases, myogenic features were revealed by immunocytochemical methods applied to fine-needle aspiration (FNA) biopsies. Each was immunoreactive for desmin and vimentin and failed to react with antibodies to leukocyte-common antigen. Moreover, formalin-fixed tissue sections of one case were negative for cytokeratin and epithelial membrane antigen. Sporadic reactivity for neuron-specific enolase and Leu-7 antigen was observed in occasional cells in FNA specimens, but synaptophysin was not identified. The rapidity of the method and the reliability of the immunocytochemical findings, when compared with routine cytologic evaluation, emphasize the diagnostic utility of immunocytochemical analysis when FNAs of pediatric soft-tissue tumors are obtained.

Fine needle aspiration cytology of a large cell calcifying Sertoli cell tumor of the testis.

A case of a large cell calcifying Sertoli cell tumor (LCCSCT) of the testis in a 7-year-old boy is presented. Fine needle aspiration (FNA) cytologic smears showed large polygonal tumor cells with abundant finely granular or vacuolated eosinophilic cytoplasm and eccentric nuclei with one distinct nucleolus. A variable amount of amorphous calcification was a constant feature. The light microscopic and ultrastructural features of the tumor cells supported a Sertoli cell origin of the tumor. This tumor is found frequently associated with complex endocrine disorders and usually has a benign course. This case, which appears to be the first such case diagnosed by FNA cytology, shows the efficacy of FNA cytology in the presurgical evaluation of testicular masses.