Dhirendra Govender

Clear cell 'sugar' tumor of the breast: Another extrapulmonary site and review of the literature

A group of lesions show morphologic and immunophenotypic evidence of differentiation toward a putative perivascular epithelioid cell. These so-called PEComas include angiomyolipoma, lymphangiomyoma, lymphangioleiomyomatosis, renal capsuloma, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and clear cell “sugar” tumor. PEComas are characterized by strong immunoreactivity with the HMB45 antibody and variable expression of muscle markers. This family of lesions may be composed of a spectrum of cells from epithelioid to spindle cells with clear to granular eosinophilic cytoplasm. One member of this family, composed of epithelioid cells with glycogen-rich clear cytoplasm, is descriptively called a clear cell “sugar” tumor. This tumor, originally described in the lung, is being recognized increasingly in extrapulmonary sites. We report a case of a primary extrapulmonary clear cell “sugar” tumor occurring in the right breast of a 16-year-old girl. The tumor was composed of clear epithelioid cells with abundant glycogen and distinct cell borders. The tumor showed strong immunoreactivity with HMB45 antibody and Melan-A. There was focal vimentin staining. In addition, there was diffuse and strong nuclear staining for progesterone receptor. Antibodies to actins, S-100 protein, cytokeratins (AE1/AE3 and CAM5.2), desmin, and estrogen receptor were negative. The tumor was completely excised, and the patient is well without evidence of disease 9 months postexcision.

Inflammatory pseudotumor of the breast

Summary Inflammatory pseudotumors (IP) have come to the forefront in recent times with this characteristic lesion being described in several sites.1–5 The multiplicity of sites of occurrence is matched by the plethora of names given to this condition. It has been rejoiced under the rubric of plasma cell granuloma, xanthomatous pseudotumor, xanthogranuloma, inflammatory myofibroblastic tumor/lesion and inflammatory fibromyxoid tumor, to name but some. This paper presents three cases of IP occurring in the breast and highlights the histological features in this unusual site.Abbreviations: IP, inflammatory pseudotumors.

Right pulmonary artery sarcoma

Summary A 57‐year‐old man presented with cough, chest pain and dyspnea. Peripheral lung opacities detected radiologically on admission were surgically resected and histologically confirmed as pulmonary infarcts. Subsequent radiological examinations revealed a stricture of the right pulmonary artery with evidence of pulmonary hypertension. The patient was commenced on anticoagulant therapy, which resulted in some clinical improvement. A hilar mass detected later was resected together with the right lung. Pathological examination of the hilar mass revealed a tumour within the lumen of the pulmonary artery causing almost total occlusion of the artery. Histology showed angiosarcomatous and osteosarcomatous areas. The patient is well and disease‐free 9 months following resection.

EXTRASKELETAL OSTEOSARCOMA OF THE SCALP

Summary A rare case of extraskeletal osteosarcoma of the scalp in a 56-year-old woman is described. At presentation she was found to have an 8-cm diameter, tender, firm, exophytic scalp tumor. MRI scan confirmed absence of underlying skeletal origin and showed extension along the subcutaneous plane. The tumor was excised and the patient received postoperative chemotherapy. Histologically, the tumor showed classical features of an osteogenic osteosarcoma with focal fibroblastic areas. In addition, there were rhabdoid cells present, which showed paranuclear cytoplasmic immunoreactivity for epithelial membrane antigen. The patient developed metastatic disease 6 months after surgical excision.

Primary lumbosacral Wilms tumour associated with occult spinal dysraphism.

Abstract A 4-year-old child presenting with sudden- onset paraplegia and a sacral tumour in association with spina bifida occulta is reported. There were no stigmata of spinal dysraphism at birth. Imaging studies confirmed a sacral tumour with extradural extension up to T10 and spinal dysraphism. The histological features of the extradural and sacral components of the tumour were consistent with a Wilms tumour. The differential diagnosis included a primary sacral teratoma containing Wilms tumour elements or a primary extrarenal Wilms tumour arising in association with a spinal dysraphism. There was no clinical response to chemotherapy or radiotherapy.

Primary hepatic osteosarcoma: Case report and literature review

Summary We describe a primary osteosarcoma of the liver in a 72 year old man. The man was symptomatic and had suffered abdominal pain and distension for four months before he died. At autopsy a large tumor of the liver with direct extension into adjacent structures was found. There was no evidence of primary tumor or primary bone lesion at any other site. Histologically, the tumor comprised malignant cells producing abundant osteoid arranged in a lace‐like pattern. Smaller chondroblastic and fibroblastic components were also present. These findings were in keeping with a diagnosis of primary hepatic osteosarcoma.

Mature renal teratoma and a synchronous malignant neuroepithelial tumour of the ipsilateral adrenal gland.

The main primary childhood renal neoplasms are nephroblastoma, mesoblastic nephroma, clear cell sarcoma, and rhabdoid tumour. Other primary renal neoplasms include primitive neuroectodermal tumour (PNET), renal cell carcinoma, and angiomyolipoma. Nephroblastoma is the most common renal tumour in children. It is a complex embryonal tumour of metanephric blastemal derivation, which often contains diverse epithelial and stromal tissues. Diagnostic problems are often encountered when tumours contain a variety of heterologous elements. The term teratoid nephroblastoma has been used to describe a variant of nephroblastoma with a predominance of heterologous tissues.1 It is this variant that can be confused with a teratoma. Renal teratomas are rare and most have been dismissed as cases of teratoid nephroblastomas or retroperitoneal teratomas secondarily invading the kidney.2 The differentiation between these two neoplasms in the kidney is often problematic. Neurogenic tissues in the kidney can be found in primary tumours or as part of metastatic tumours. The primary tumours are nephroblastoma, which may contain ganglion cells, neuroblast, and neuroglial tissue,2 and PNET.3, 4 Adrenal neuroblastomas can directly invade the adjacent kidney.5 We describe the pathology of a right renal mass in a 3 year old child and discuss the differential diagnosis. A 3 year old girl presented with abdominal pain and diarrhoea. On examination she was found to have signs of pulmonary tuberculosis and was started on antituberculous treatment. Subsequently, a large, firm, tender, right flank mass clearly separate from the liver was detected and she was referred to the Regional Paediatric Surgical Unit for further investigation and management. On admission, the child was apyrexial, emaciated, and weighed 13 kg. She had bilateral coarse crackles and a wheeze. The abdomen was distended and a non-tender 3 cm hepatomegaly was palpated. Furthermore, a 10 × 12 cm non-tender, firm, non-pulsatile right …

Follicular thyroid carcinoma with rhabdoid phenotype.

Abstract The aim of this paper is to highlight the occurrence of an unusual histological variant of follicular carcinoma of the thyroid. Three cases are presented: each of the tumours contained a significant population of rhabdoid cells (accounting for 30–40% of the total tumour content). They were all found in female patients aged 65, 43 and 56 years, who presented with enlarged thyroid glands and were subjected to lobectomies. The tumours contained foci of well-differentiated follicular carcinoma, with areas of capsular and vascular invasion, and an accompanying rhabdoid cell component that merged with the neoplastic follicles. Immunohistochemically, the follicular component was positive with thyroglobulin, but the rhabdoid cells were negative in all three cases. The cytoplasmic aggregates in the rhabdoid cells were strongly positive for epithelial markers and vimentin. Two tumours pursued an aggressive biological course similar to other composite extrarenal rhabdoid tumours. A rhabdoid component accompanying thyroid follicular carcinomas is an adverse prognostic factor.

Sertoli cell nodules in the undescended testis: a histochemical, immunohistochemical, and ultrastructural study of hyaline deposits

Aims: To document the morphology, immunohistochemical staining properties, and ultrastructural features of hyaline material in Sertoli cell nodules of undescended testis and contrast them with those of sex cord tumour with annular tubules (SCTAT), which is histologically similar. To highlight the need to distinguish these nodules from other Sertoli cell hyperplasias, such as intratubular Sertoli cell proliferations, which occur in specific clinical contexts. Materials/methods: A retrospective study of 46 orchidectomy specimens from cryptorchid testes, 27 of which contained Sertoli cell nodules. Special histochemical stains, immunohistochemical stains for type IV collagen and fibronectin, and ultrastructural examination of the hyaline material were performed using tissue from paraffin wax embedded tissue blocks. Results: The hyaline deposits in SCTAT and Sertoli cell nodules had similar staining patterns—periodic acid Schiff (PAS) and PAS-diastase positivity with variable staining of Martius scarlet blue and Masson trichrome. Type IV collagen immunoreactivity was seen in hyaline areas, although fibronectin was negative. Electron microscopy of hyaline areas confirmed a compact matrix identical to components of the basement membrane in the adjacent seminiferous tubules. Conclusion: This study describes an unusual form of Sertoli cell proliferation in undescended testes, which must be distinguished from Sertoli cell tumours and other forms of proliferation. In addition, the hyaline material within Sertoli cell nodules in the cryptorchid testis is histochemically, immunohistochemically, and ultrastructurally consistent with both matrix and fibrous components of seminiferous tubule basement membranes. Increased production of basement membrane material, with subsequent invagination into tubules, is the most likely origin of this material.

Malakoplakia and tuberculosis

A 33 year old woman developed a soft mass in the left anterior neck following treatment for pulmonary tuberculosis. An incisional biopsy was performed and a cold abscess drained. Histology confirmed a diagnosis of tuberculosis. In addition, there were numerous intracellular bodies which resembled the Michaelis-Gutmann bodies of malakoplakia. This case however displayed some atypical histological, histochemical and ultrastructural features, which are highlighted in this report. The mass responded to combination antituberculous treatment.