Janez Lamovec

Spiradenocylindromas of the skin: Tumors with morphological features of spiradenoma and cylindroma in the same lesion: Report of 12 cases

Twelve cases of spiradenocylindromas, which revealed features of both spiradenoma and cylindroma in the same tumor mass, are presented. Nine female patients had multiple neoplasms occurring mostly on the scalp, and two female and one male patient had a solitary cutaneous lesion. Three of the female patients with multiple cutaneous tumors had a familial history of similar cutaneous neoplasms. In one of the patient’s family, the multiple cutaneous tumors were known to occur in multiple family members in four consecutive generations. One patient with multiple cutaneous lesions was known to have associated multiple kidney cysts as confirmed by computed tomography. Histologically, spiradenocylindromas are composed of intermixed areas that are either of typical spiradenoma in appearance or of typical cylindroma appearance. Apocrine and trichoepitheliomatous differentiation seen in two cases in the present series points to spiradenomas, as well as cylindromas, having complex hair follicle (folliculo‐ sebaceous apocrine) rather than eccrine differentiation. The presence of lymphoid tissue was a histological feature in the present series, which was prominent in all the spiradenomatous parts of the tumors and which was scanty or practically absent in all the cylindromatous parts. The selective presence of lymphocytes in spiradenoma and an absence in cylindroma suggest that spiradenomas have the unique property of attracting lymphocytes. The malignant tumors arising in three patients in the present series had the morphology of a poorly differentiated epithelioid neoplasm. Three patients died of the disease and the other patients were either free of disease or alive with disease 1–30 years on follow up.

Acantholytic variant of squamous-cell carcinoma of the breast

Three cases of acantholytic squamous-cell carcinoma of the breast are reported. They all had histological features resembling those of angiosarcoma or adenocarcinoma. They were not angiosarcoma, since in all three cases areas of squamous differentiation were present; in addition, the neoplastic cells were negative when stained for factor VIII, but were positive with anti-epidermal keratin. The glandular pattern exhibited, especially in Case 2, was difficult to differentiate from that of an ordinary carcinoma. However, the presence of dyskeratotic cells within the lumina, and the absence of alcian blue/periodic acid-Schiff positive material, and epithelial membrane antigen staining, were evidence against the diagnosis of adenocarcinoma. The patients died 5, 6, and 16 months after the diagnosis. Tumors with these histological features may have a very aggressive clinical course.

Acinic cell-like carcinoma of the breast

A case of infiltrating carcinoma of the breast with features similar to those seen in acinic cell carcinoma of the parotid gland is described in a 42-year-old woman. The neoplastic cells were immunoreactive with anti-lysozyme- and anti-salivary-type amylase antisera and contained electron-dense cytoplasmic globules similar to those seen in acinic cell carcinoma of salivary glands. One lymph node out of 18 was found to contain a metastatic deposit. The patient is alive and well 1 year after mastectomy. This appears to be the first case of carcinoma with acinic cell-like features reported in the breast.

Acinic cell carcinoma of the breast: an immunohistochemical and ultrastructural study.

Abstract The clinicopathological features of six cases of breast carcinomas showing features of acinic cell differentiation, which are similar to those seen in homologous tumors of salivary glands, are presented. The patients, all women, were 35–80 years of age. One case recurred after 4 years, and in two cases axillary lymph-node metastases were found at the time of surgery. Histologically the tumors showed a microglandular pattern merging with solid areas. Cytologically, immunohistochemically, and ultrastructurally the tumors were very similar to cases of acinic cell carcinoma of the parotid gland.The differential diagnostic criteria with microglandular adenosis and carcinomas showing granular cytoplasm are discussed. It seems that acinic cell carcinomas of the breast have to be added to the long list of tumors that affect the salivary glands and can also arise in the breast.

Ossifying fibromyxoid tumor of soft parts: A report of 17 cases with emphasis on unusual histological features

Ossifying fibromyxoid tumor of soft parts is an unusual benign neoplasm, with a tendency for local recurrence. Its typical microscopic appearance is that of a multinodular proliferation of round to spindle shaped cells separated by fibrous bands in which bone formation is often seen. Herein, we present the clinicopathologic features of 17 examples of this tumor with particular emphasis on some unusual histopathologic features that may place pitfalls in the diagnosis of this tumor, including satellite micronodules, mucinous microcysts, absence of myxoid areas, crush artifact, multiple microcalcifications, epidermoid cysts, atypical chondroid differentiation with binucleate lacunar cells, pericytic growth pattern, and malignant change. Awareness of these unusual morphologic features should lead to a search for areas displaying the more typical features of ossifying fibromyxoid tumor to arrive at a correct diagnosis.

Sclerosing Inflammatory Pseudotumor of the Urinary Bladder in a Child

A case of inflammatory pseudotumor of the urinary bladder in a 2-year-old child is presented. It was characterized by nodular intravesical growth and massive infiltration of the bladder wall. Microscopically, the lesion showed in its largest part a relatively paucicellular spindle cell growth and a sclerotic appearance with a thin superficial cellular zone resembling granulation tissue. Another morphological characteristic was a marked capillary proliferation revealed by immunohistochemical reactions to factor VIII-associated protein, laminin, and collagen IV. The last feature appears to be an integral part of the process, which most closely resembled fibromatosis of the adult type, a rare pattern of growth in inflammatory pseudotumor.

Pseudoangiomatous Hyperplasia of the Mammary Stroma: True Entity or Phenotype?

We report the clinical and pathologic features seen in 14 cases of pseudoangiomatous hyperplasia of the mammary stroma. The lesion manifested as a discrete palpable lump in twelve women and two men. The excisional biopsy specimens showed firm, circumscribed grey-tan lesions measuring from 3 to 5.5 cm in largest dimension. Histologic study revealed diffuse, anastomosing, enlongated slits with open lumina, covered by spindle cells with bland nuclei and no mitotic activity. Strong reactivity for vimentin and CD34 was noted. Smooth muscle actin was focally noted in most cases. Immunostains for other markers, including CD31 and factor VIII related antigen was negative. Spindle cells from two cases expressed both estrogen and progesterone receptors. In all the cases, there was some degree of glandular hyperplasia accompanying the stromal changes. Fibroadenoma or fibrocystic disease were also common features. Our study confirms that pseudoangiomatous hyperplasia of mammary stroma represents a proliferation of local myofibroblasts, likely related to a hormonal stimulus. The wide range of associated changes of breast parenchyma further indicates that this lesion may represent a local, non specific change rather than a true clinico-pathologic entity.

Prognostic Relevance of Silver-Stained Nucleolar Proteins in Sarcomatoid Carcinomas of the Breast

Fourteen cases of sarcomatoid carcinomas of breast were evaluated by means of a silver technique that selectively stains proteins located in the nucleolar organizer regions (Ag-NORs). The mean area of Ag-NORs (MNORA) was in each case quantitatively analyzed by means of an automated image analyzer. Patients who died early of the disease had a higher MNORA than patients who survived longer than 3 years. The difference was statistically significant. Ag-NORs might be a novel parameter of prognostic relevance in specific cases.

Extraneural metastases of anaplastic oligodendroglial tumors

Extraneural metastases of malignant gliomas are rare. According to the literature, they tend to appear in glioblastoma patients, but are exceptionally rare in anaplastic oligodendroglioma. We report on an anaplastic oligodendroglioma and an anaplastic oligoastrocytoma that metastasized to cervical lymph nodes and bones. Both patients were women aged 54 and 30 years, and the metastases appeared following craniotomy. In the first patient, metastases to cervical lymph nodes developed one year after surgery, and, despite adjuvant therapy, recurred in the same location several times. Fine needle aspiration biopsy (FNAB) of the cervical lymph node prior to neck dissection suggested a possible metastatic primitive neuroepithelial tumor. In the second case, metastases to the sacrum and femur developed after surgery for a recurrent anaplastic oligoastrocytoma. Our two cases reconfirm a rare but definite ability not only of glioblastoma but also of anaplastic oligodendroglioma, namely to metastasize to extraneural sites. It is important to bear this in mind, particularly in cases when the history of primary brain tumor is unavailable. In such instances, the correct diagnosis of the metastatic lesion may be extremely difficult if not impossible.

Pseudosarcoma in Paget's disease of bone

The appearance of a sarcoma of bone is a well-recognized complication of Pagets disease. The most common type of such a sarcoma is osteosarcoma. Much less common are soft tissue lesions adjoining the pagetic bone that clinically and radiologically simulate sarcoma but histologically represent exaggerated periosteal bone formation as a manifestation of the basic pathologic process. We present a case of a bulky juxtacortical soft tissue mass in the thigh arising from a pagetic femur in a 62-year-old patient with polyostotic Pagets disease that was clinically and radiologically suspected to be a juxtacortical osteosarcoma. Microscopically, the lesion showed features of florid Pagets disease without any evidence of sarcomatous growth. It is important to be aware of this rare manifestation of Pagets disease to avoid unnecessary overtreatment.