Edwin Gould

The macrofollicular variant of papillary thyroid carcinoma : a study of 17 cases

We report 17 cases of a distinctive variant of encapsulated papillary carcinoma that is likely to be confused with macrofollicular adenoma or nodular goiter. The tumors showed the unusual combination of macrofollicles and foci of the conventional follicular variant of papillary carcinoma. Macrofollicles occupied over 50% of the cross-sectional areas of the tumors. The macrofollicles were lined either by cells with large ground-glass nuclei, cells with large but less pale nuclei with stippled chromatin, or cuboidal cells with hyperchromatic nuclei. Solid foci were present in four tumors, and a focal insular growth pattern was noted in a fifth tumor. All patients were females ranging in age from 15 to 69 years (mean, 35.4 years). Despite the large size of the tumors (mean diameter, 4.8 cm), only two metastasized to cervical lymph nodes. One of these neoplasms showed extrathyroidal extension, while the other widely invaded the thyroid tissue through the tumor capsule. Multicentric foci of papillary carcinoma with a follicular nonsclerosing growth pattern were demonstrated in the contralateral lobe in two thyroid glands. All patients with follow-up were alive and symptom-free 2 months to 6 years following surgery.

Pseudosarcomatous myofibroblastic proliferations in the urinary bladder of children

Ten examples of a pseudosarcomatous myofibroblastic proliferation occurring in the urinary bladder children (aged 2 to 16 years) are reported. The lesions appeared as polypoid nodular masses of variable size with myxoid and hemorrhagic areas. They consisted of compact fascicles of elongated spindle cells with minimal atypia. Myxoid areas of variable extension and scattered inflammatory cells were constant features, whereas diffuse collagen deposition was not common. Despite the striking cellularity of some of the lesions, most showed minimal mitotic activity. Ultrastructurally the predominant cells had features of myofibroblasts. Six cases studied by immunocytochemical methods expressed vimentin and muscle‐specific actin. In addition, two of these cases expressed desmin and two others cytokeratin. Infiltration into the muscularis propria of the urinary bladder was demonstrated in six cases and into the perivesical soft tissues in two. However, none of the eight patients for whom follow‐up information is available has had local recurrence or metastasis develop 18 months to 6 years after surgical excision.

The spectrum of liver disease in the acquired immunodeficiency syndrome.

Abnormal liver chemistries, unexplained fevers, or hepatomegaly prompted 36 liver biopsies on 34 patients with the acquired immunodeficiency syndrome. The most common finding was the presence of hepatic granulomas, seen in 13 of the biopsy specimens. Eight of these granulomas were ill-defined, and 5 were more clearly associated with mycobacterial disease. Portal fibrosis and fatty infiltration were common, but a paucity of significant inflammatory activity was seen despite elevated aspartate aminotransferase levels, perhaps related to the underlying immunoincompetent status. Other noteworthy histopathologic findings included 1 patient each with peliosis hepatis and cryptococcal hepatitis. Electron-microscopic evidence of cytoplasmic tubular structures or viral particles were seen within the hepatocytes of 2 patients. It is concluded that a broad spectrum of hepatic histopathology may be seen in the acquired immunodeficiency syndrome, and that liver biopsy may be diagnostically valuable in the clinical investigation of such patients.

Pilomatrix carcinoma with pulmonary metastasis. Report of a case

A 67‐year‐old white man developed a locally recurrent pilomatrix carcinoma of the back. Within a 4‐year period bilateral pulmonary metastases developed. This to the knowledge of the authors is the first reported case of a metastasizing pilomatrix carcinoma in the medical literature.

Merkel Cell Carcinoma of the Eyelid and Periocular Tissues

Five patients had eyelid and periocular Merkel cell carcinoma. The tumor was located on the left lower eyelid in two patients, the left upper eyelid in one patient, the right upper eyelid in one patient, and was metastatic to the right outer canthus in one patient. The mean duration of symptoms was approximately four months. The diagnosis of Merkel cell carcinoma was not suspected clinically in any of the four primary eyelid cases, but was only established on histopathologic examination of biopsy specimens. Light microscopy disclosed carcinoma with small primitive cells in all five tumor biopsy specimens. Immunohistochemical studies showed neuron-specific enolase and keratin and transmission electron microscopy demonstrated neurosecretory granules typical for Merkel cell carcinoma. All five patients in this study were treated with wide surgical excision of the eyelid tumors with intraoperative frozen-section monitoring of the margins of resection. The left lower eyelid Merkel cell carcinoma spread to the preauricular lymph node in one patient. This patient subsequently died of metastatic Merkel cell carcinoma. One patient with metastatic right outer canthus Merkel cell carcinoma received radiotherapy (6,550 cGy). Eyelid Merkel cell carcinoma has the potential for recurrence and metastatic spread. We recommend lifetime follow-up for patients treated for eyelid Merkel cell carcinoma.

Benign hypergammaglobulinemic purpura of waldenström associated with Sjögren's syndrome. Case report and review of immunologic aspects

Since its first description, fewer than 150 cases of benign hypergammaglobulinemic purpura of Waldeström have been reported. There is a preponderance of females with this disorder, which is characterized by long-standing purpuric vasculitic lesions usually in the lower extremities, increased sedimentation rate, anemia, leukopenia, and hyperglobulinemia with a normal clotting process. Numerous associations have been shown between this and other systemic disorders such as Sjögrens syndrome, systemic lupus erythematosus, a rheumatoid arthritis. A 40-year-old woman is described who had an 18-year history of recurrent purpura and increased IgG levels. Skin biopsy showed acute vasculitis, and immunofluorescent study revealed fibrinogen precipitation and C3 deposition. Serologic evaluation demonstrated the presence of rheumatoid factor and antinuclear antibodies (1:80). Raji assay showed increased circulating immune complexes, and the T cell subsets were normal. The purpura was associated with symptoms and physical findings of Sjögrens syndrome. On the basis of serologic and skin biopsy findings, an autoimmune origin of the disease is postulated.

Malignant giant cell tumor of soft parts presenting as a skin tumor

Malignant giant cell tumor of soft parts is a rare neoplasm that histologically resembles a giant cell tumor of bone. It has a distinctive multinodular growth pattern and is composed of numerous osteoclast-like giant cells, histiocytes, and fibroblasts. Although this tumor is usually found in deep soft tissues, a superficial form has been described in the subcutaneous tissue and fascia. The authors report two patients, aged 75 and 78, with malignant giant cell tumors presenting as ulcerating skin nodules of the arm and foot. The tumors were relatively small, measuring <3.5 cm in greatest dimension, and involved the entire dermis and subcutaneous tissue. The clinical differential diagnoses included Kaposis sarcoma, melanoma, and hematoma. Dermatopathologists and dermatologists should be aware of this entity to avoid confusion with other benign and malignant neoplasms that may contain multinucleated giant cells. The distinguishing histologic and immunohistochemical features of this tumor are discussed.

Scleromyxedema and severe myositis

ABSTRACT A case of scleromyxedema with associated rhabdomyolysis is reported. This appears to be a well‐documented, although rare complication of scleromyxedema. This case is unique in that mucin was demonstrated in the muscle as well as the skin, and spontaneous resolution of the rhabdomyolysis occurred with the implementation of IV fluids and bed rest.

The Accuracy of the Histological Classification of Lung Carcinoma and Its Reproducibility: A Study of 75 Archival Cases of Adenosquamous Carcinoma

The classification of lung carcinoma into a small cell lung carcinoma (SCLC) and non-small cell lung carcinoma (NSCLC), is highly reproducible. There are few studies on the reproducibility of subtyping NSCLC, with anecdotal reports on the subtype of adenosquamous carcinoma. We undertook a study of 75 cases archived as adenosquamous carcinoma. All cases were accepted as NSCLC on independent review by three pathologists utilizing the 1982 World Health Organization (WHO) classification criteria. The acceptance rate of adenosquamous carcinoma by the three pathologists was 65%, 28%, and 65%. Cases not accepted as adenosquamous fell into the different subtypes of NSCLC, with a concordance rate between each pair of pathologists of 49%, 61% and 43%, indicating poor agreement between pathologists. The study confirms the high reproducibility of the classification into SCLC and NSCLC; it shows poor concordance for all subtypes of NSCLC with poor reproducibility of diagnosis of adenosquamous carcinoma as specified by the 1982 WHO classification. Physicians dealing with lung carcinoma should be aware of this limitation of classification.

Extraocular Muscle Metastasis 16 Years Following Primary Prostate Carcinoma

An 83-year-old man with a history of localized prostate carcinoma treated 16 years prior with transurethral partial prostatectomy and radioactive seed implants presented with a 2-month history of right globe proptosis. Computed tomography demonstrated a right medial rectus mass. Serum prostate specific antigen (PSA) level was normal. Tumor biopsy revealed poorly differentiated carcinoma with diffuse staining of tumor cells with PSA and prostatic acid phosphatase. This case demonstrates that prostate carcinoma may metastasize to an extraocular muscle many years after treatment of the primary tumor and may not be accompanied by elevated serum PSA. Immunohistochemical stains are helpful in establishing the diagnosis.