Primary pulmonary nodular amyloidosis.

Primary nodular amyloidosis of the lung is an uncommon manifestation. The disease runs a benign course, but offers diagnostic problems due to non-specific radiological features entering the big field of the solitary nodule. We describe the case of a 60 year old man with multiple nodules on the left lung operated on diagnostic and therapeutic video-assisted thoracoscopy and discuss the possibilities, if any, of suspecting such a disease through radiologic characteristics along with findings from the patient's history, physical examination and laboratory tests.

Fibroepithelial tumor of the breast with digital fibroma-like inclusions in the stromal component. Case report with immunocytochemical and ultrastructural analysis.

A case of fibroepithelial tumor of the breast in a 34-year-old woman, showing digital fibroma-like inclusions in the stromal component is presented. Morphological, immunocytochemical, and ultrastructural findings indicate that the peculiar eosinophilic intracytoplasmic inclusions observed may represent tightly packed actin filaments. Although they are more frequently detected within digital fibrous proliferations of infancy, their rare occurrence at either unusual site or in adulthood has been reported. Similar inclusions have also been rarely observed in intranodal myofibroblastoma and in benign leiomyomas. These findings suggest that these inclusions may be the result of some metabolic or organisational abnormality shared by neoplastic actin-rich cells. Myofibroblastic and smooth muscle lesions are probably the commonest to show this defect.

Leiomyosarcoma of the female breast: report of two new cases and a review of the literature.

We present the clinical, light microscopic, and immunohistochemical features of two new cases of leiomyosarcoma of the female breast. Both the patients were old (83 and 86 years) and were referred with a history of a long-standing breast lump. The results of the physical examination and the preoperative radiologic investigations suggested a phyllodes tumor. The patients were treated with mastectomy. The tumors measured 6 and 6.5 cm in the largest dimension, respectively, and were composed of fascicles of atypical, actively proliferating spindle cells, often intersecting at right angles. The axillary lymph nodes were free of tumor. Immunohistochemically, the tumor cells were positive for desmin, muscle-specific actin, and vimentin and negative for other markers, including keratins and hormone receptors. Focal rhabdomyoblastic differentiation was noted in one case. Follow up at 1 year is negative for metastases or local recurrences. Our study confirms that leiomyosarcoma of the breast is a locally invasive neoplasm and that it could represent a peculiar anatomic entity among mesenchymal tumors of the breast.

Aneurysmal bone cyst of the larynx presenting with hypoglottic obstruction.

We report a new case of aneurysmal bone cyst of the larynx occurring in a 22-year-old man. The lesion manifested with progressive breathing discomfort and appeared as a polypoid pedunculated mass attached to the subglottic mucosa. Microscopically, it featured numerous mononuclear and multinucleated giant cells surrounding cavernous spaces filled with blood. Foci of proliferating spindle cells and mature osteoid tissue could be recognized. There was no apparent relationship with the cricoid perichondrium. Clinical follow-up was negative for local recurrence. Based on this report and a review of the literature, we conclude that aneurysmal bone cyst of the larynx is phenotypically comparable to its bone homologue; however, its microscopic recognition may be difficult, especially on small biopsy fragments. Since it can be confused with several lesions, including telangiectatic osteosarcoma, awareness of this rare appearance of aneurysmal bone cyst is important to avoid unnecessary radical surgery.

The Value of Fine Needle Aspiration Cytology in Breast Fibromatosis: Study of Two New Cases and Review of the Literature.

We report two new cases of breast fibromatosis studied by needle aspiration cytology observed in a 32-year-old man and a 49-year-old woman. The lesions manifested as palpable, painless, and firm masses of the para-areolar breast soft tissues. Preoperative fine needle cytology revealed scant cellularity, featuring oval and spindle cells with bland nuclei and occasional larger polygonal cells with high nuclear:cytoplasmic ratio. Histologically, interwoven fascicles of spindle cells with bland nuclei, infiltrating the adjacent breast fat, were recognized. Based on available reports in the literature, we concluded that needle aspiration cytology, although not entirely specific, may be a source of important information in patients with breast fibromatosis. In particular, it confidently allows the exclusion of breast cancer and other more common diseases and is useful in planning a surgical approach to the lesion.

Papillary thyroid carcinoma of the thyroglossal duct cyst: comparative cytohistologic and immunochemical study of 2 new cases and review of the literature.

We report a cytohistologic and immunohistochemical study of 2 cases of papillary thyroid carcinoma occurring in a thyroglossal duct cyst. The patients were a 21-year-old woman and a 48-year-old man. Needle aspiration cytology smears were consistent with papillary thyroid carcinoma. The Sistrunk procedure was done. Papillary carcinoma was found within a thyroglossal duct cyst. In 1 case, the tumor spread outside the cyst. Follow-up was uneventful in both patients (2 and 9 years, respectively). Our results would indicate that papillary carcinoma of thyroglossal duct cyst, though indistinguishable from its thyroid homologue, has a more indolent course and could therefore be singled out as a clinicopathologic entity. Needle aspiration cytology reliably assists in planning patient management.

Aspiration biopsy cytology of intranodal myofibroblastoma: case report with immunocytochemical analysis.

Intranodal myofibroblastoma (IM) is a recently delineated clinicopathologic entity represented by a myofibroblastic proliferation of the lymph node. It usually occurs at the groin and presents clinically as a single enlarged lymph node. Its clinical behavior is entirely benign and local excision is curative. The authors herein report a case of IM diagnosed by aspiration biopsy cytology (ABC). The main diagnostic clues to the cytologic diagnosis of this entity as well as the differential diagnosis are presented.

Aspiration biopsy cytology of tubular carcinoma of the breast.

Tubular carcinoma represents a rare, well-differentiated type of breast malignancy, characterized by relatively unaggressive clinical behavior. Cytologic diagnosis of this subgroup of tumors is considered difficult as cell population usually presents in tightly cohesive clusters and shows minimal atypia. Cytomorphologic features of 15 examples of histologically proven pure tubular carcinomas of the breast are herein reported. If a constellation of morphologic criteria is critically evaluated, tubular carcinoma may be diagnosed on cytologic specimens. Aspiration biopsy cytology represents an optimal diagnostic tool in the preoperative diagnosis of breast masses. Its application to the detection of this special type of breast carcinoma permits better planning of therapeutic procedures.

Malignant transformation of an adenomyoma of the cardia and malignant Hodgkin gastric lymphoma. An unusual coexistence.

An unusual coexistence of a malignant Hodgkin gastric lymphoma of the gastric antrum with a gastric adenocarcinoma within an adenomyoma of the cardia is reported. This is a previously undescribed occurrence.

[Leiomyosarcoma of the spermatic cord]. / Sul leiomiosarcoma del funicolo spermatico.

The case of leiomyosarcoma of the spermatic cord in a 54 year old man is reported. The patient was treated by surgical orchiepididymectomy and funnilectomy. After a review of the literature, the clinical and anatomopathological feature of this rare tumour and the surgical approach to it are discussed. The patient has had no recurrences and is still alive 16 months after surgery.

[Treatment of laryngeal adenoid cystic carcinoma]. / Sul trattamento del carcinoma adenoido-cistico laringeo.

Laryngeal localization (nearly always hypoglottic) of adenoid cystic carcinoma is quite rare (0.6% of all malignant laryngeal tumors). In most cases the local development of the neoplasm is slow, of an infiltrating and highly invasive nature (affecting the cartilage, thyroid gland, esophagus). On the other hand in some cases, particularly in the laryngeal-tracheal localization, the tumors take on an exophytic, sessile, polypoid aspect. Although the evolution is quite slow prognosis is poor due to the latent spreading of distant metastasis (average survival is 8 years). Prognosis is further worsened by delay in diagnosis due to the poor, aspecific initial symptoms. C.A.T. and N.M.R. have made a marked contribution to improved diagnostic definition, especially in terms of tumor extension, which is of great importance in therapeutic staging. Given the poor results obtained with radiotherapy and chemotherapy the only possible treatment is surgery which, in most cases, must be quite radical. Only in those forms which are still localized (with endoluminal exophytic development) is it possible to perform conservative surgery. Two paradigmatic cases are reported here indicating two different tumor extensions: in the first case total laryngectomy was performed and extended to the thyroid gland and the upper portion of the trachea; in the second, a partial glottic-subglottic laryngectomy was performed with functional restoration of laryngeal function. Conservative surgery is only possible if the hypoglottic neoformation is limited to the larynx, if it is mainly exophytic in nature and if histological examination reveals a low degree of malignancy (a high degree of cell differentiation). Since this type of carcinoma tends to give rise to latent distant metastases prognosis is, in all cases, poor no matter what surgical technique is employed for the primary tumor. Therefore it is imperative that a conservative laryngectomy be performed in all cases where it proves oncologically possible and particular attention should be paid to the quality of the patient's post-surgery life.

Gastric MALT lymphomas: the value of an endoscopic follow-up evaluation.

BACKGROUND: Findings have demonstrated the close association between primary gastric B-cell lymphomas originating from mucosa-associated lymphoid tissue (MALT) and Helicobacter pylori (HP) infection, with their regression after an HP eradication therapy in up to 70% of the cases. Endoscopic-biopsy diagnosis and endoscopic ultrasound are of major importance as decisive prognostic factors and therapeutic determinants. OBJECTIVES: We report 3 years of experience and follow-up evaluation in the management of MALT lymphomas. We also describe the guidelines strategy therapy used in our institution. METHODS: Since July 1996, nine patients with a histologic diagnosis of low-grade, HP-positive MALT gastric lymphomas, have been followed up. All patients had stage IE lymphomas (according to Musshoff classification). Eradication of HP was performed with triple therapy amoxycillin, clarithromycin, and omeprazole using over a 14-day period. The patients were seen for endoscopic follow-up assessment after 3, 6, 9, and 15 months, then twice a year. The actual median follow-up time was 30.4 months (range, 16-38 months). RESULTS: All the patients are now free of disease and asymptomatic. We have registered two cases of HP relapse (both after 1-year follow-up evaluation), positively treated with the same triple therapy, and three cases of disease relapse treated with single-dose chemotherapy (plus radiotherapy in one patient). CONCLUSIONS: In our experience the eradication of HP appears to be effective, and we consider it the first therapeutic option in patients with stage IE gastric low-grade MALT lymphoma, although long-term results are still needed. Prolonged follow-up evaluation (particularly by endoscopy) is necessary (and feasible in our experience) to determine whether these remissions are long-lasting. We recommend that HP be eradicated in these lymphomas before referral to other standard treatment.

Polypoid osteosarcoma of the left atrium: report of a new case with autopsy confirmation and review of the literature.

We describe a new case of heart osteosarcoma manifesting as a large polypoid mass of the left atrium. Clinically, the lesion presented with dyspnea, syncopal attacks, and fever. Ultrasound scans of the heart were suggestive of a cardiac myxoma, although some imaging features could retrospectively indicate a malignant neoplasm. In particular, the lesion was relatively immobile, did not show the characteristic myxoma motion during the heart cycle, and extended into the left pulmonary veins. Exploratory thoracotomy showed a nonresectable polypoid mass of the left atrium widely infiltrating the myocardium and the epicardium, histological features of a polymorphic sarcoma. The patient died 5 months after presentation with diffuse metastases. At autopsy, a 5-cm polypoid tumor was seen protruding into the left atrium. Neoplastic infiltration of the atrial myocardium, pericardium, and pulmonary veins was evident. Extensive metastatic deposits were observed in numerous sites, including the skin, lung, liver, and brain. Microscopically, a spindle cell sarcoma forming malignant osteoid was seen, admixed with areas indistinguishable from myxoid and pleomorphic malignant fibrous histiocytoma. This case provides further evidence that although rare, osteosarcoma of the heart usually presents as a polypoid mass of the left atrium and is histologically characterized by conspicuous polymorphism. It is associated with a poor prognosis and rapid appearance of distant metastases. Although at echocardiography it may mimic a myxoma, subtle features such as tumor extension into pulmonary veins and main origin from nonseptal atrial walls suggest the presence of a nonmyxomatous tumor and a preoperatory diagnosis of aggressive malignant disease.