Disseminated Congenital Neuroblastoma - An Autopsy Case Report.

Neuroblastoma is the most common malignant tumour in the neonatal age group. The tumour is derived from neural crest cells of adrenal gland medulla or sympathetic ganglia. Disseminated congenital neuroblastoma is very rare. We hereby report a case of congenital neuroblastoma with multiple metastases diagnosed on autopsy in a fetus of 23 weeks gestational age. Autopsy revealed enlarged bilateral adrenals, large fusiform mass in the right humerus, mass in the lower lobe of left lung and posterior chest wall. Diagnosis of disseminated congenital neuroblastoma was given after histopathological and immunohistochemical examination. We are reporting this case for its rare presentation of disseminated form of congenital neuroblastoma sparing liver.

Neoplastic (solid) Calcifying Ghost Cell Tumor, Intraosseous Variant: Report of A Rare Case and Review of Literature.

A neoplastic (solid), calcifying ghost cell tumour is a rare subtype of Calcifying Odontogenic Cyst. The most important features of this pathologic entity are histopathological features, which include a proliferating cystic lining which demonstrates characteristic "Ghost" epithelial cells which have a propensity to calcify. We are reporting a case of a 61-year-old male with a painful swelling in the lower right jaw. Radiology showed a bilocular, well circumscribed, radiolucent lesion in the mandible, which measured 2x1 cm, with radiopaque structures within it. Tooth extraction was done, with enucleation of the lesion, which histopathologically revealed features of a solid, calcifying ghost cell tumour, with an intraosseous variant. The case has been presented, with a brief review of literature.

Histiocytic endometritis.

PATIENT: Female, 72 FINAL DIAGNOSIS: Histiocytic endometritis Symptoms: Vaginal discharge • fever • weakness MEDICATION: - Clinical Procedure: Endometrial and cervical biopsy Specialty: Pathology • Gynaecology. OBJECTIVE: Rare disease. BACKGROUND: Histiocytic or xanthogranulomatous endometritis, characterized by disappearance of endometrial mucosa and its replacement by sheets of lipid containing histiocytic cells, is very rare. Extensive internet and PubMed searches revealed only 19 cases reported to date. The pathogenesis of histiocytic endometritis seems to be inflammation due to post-menopausal cervical stenosis or as the result of cervical carcinoma. Histiocytic endometritis can infiltrate the myometrium and can mimic a malignancy. CASE REPORT: We report the case of a 78-year-old post-menopausal female with symptoms of vaginal discharge, fever, and weakness. Radiological investigation showed a mass lesion in the cervix, extending into the myometrium, suggestive of cervical carcinoma. The lesion was biopsied and histopathological examination led to the diagnosis of histiocytic endometritis with no evidence of malignancy. CONCLUSIONS: Histiocytic endometritis, an inflammatory pathology, can mimic malignancy clinically as well as radiologically. Histopathological examination with extensive sampling of tissue is essential because presence of endometritis does not rule out malignancy.

Apocrine carcinoma of breast: a case report with review of the literature.

Apocrine carcinoma is a very rare form of breast malignancy with an incidence of <1% of female invasive breast carcinoma. We report a case of apocrine carcinoma in a 42-year female with marked adenosis showing apocrine metaplasia and discuss the criteria to diagnose apocrine carcinoma with the emerging concept of androgen receptor positivity with its implication on treatment and management of the patient.

Adenoid cystic carcinoma of female breast: A case report.

INTRODUCTION: Adenoid cystic carcinoma (ACC) of the breast is a rare neoplasm accounting for 0.1% of breast carcinomas. PRESENTATION OF CASE: A 30 year old female patient presented with multiple lumps in the right breast. Excision biopsy of a lump in the lower outer quadrant revealed adenoid cystic carcinoma. A right sided modified radical mastectomy (MRM) was performed, with axillary clearance. Histopathology revealed a fibroadenoma accounting for one mass and adenoid cystic carcinoma in another, confirmed on Periodic acid Schiff (PAS) staining. Three out of the eight excised axillary lymph nodes contained malignancy. There was no evidence of recurrence or distant spread at follow-up 6 months postoperatively. DISCUSSION: ACC of the breast is a rare neoplasm accounting for 0.1% of breast carcinomas and is of special interest because of its favourable prognosis and distinctive histological appearance. This tumor occurs predominantly in women in their sixth decade and usually presents as a tender breast mass, often in the subareolar area though in our case it was not tender and presented as multiple lumps in the right breast. The diagnosis can be made on fine needle aspiration cytology (FNAC), but in our case FNAC was inconclusive. We performed MRM with axillary clearance, and three out eight lymph nodes proved positive for malignancy. CONCLUSION: ACC is a rare malignant neoplasm. Surgical treatment is the mainstay with little role for radiotherapy and chemotherapy according to the published literature.

Primary angiosarcoma of breast: A case report.

INTRODUCTION: Primary breast angiosarcoma is a rare form of breast cancer, accounting for only 0.04% of all malignant breast tumours. PRESENTATION OF CASE: A 28-year-old woman presented with a progressive lump in the right breast for one month. Fine needle aspiration cytology (FNAC) was highly suspicious of angiosarcoma, and this diagnosis was confirmed on core needle biopsy. Modified radical mastectomy was performed as sole treatment. DISCUSSION: Primary breast angiosarcoma is a rare type of breast malignancy, usually occurring in the third to fourth decade and only reported in women. The histological features of angiosarcoma of the breast are conventionally graded I, II or III. Total mastectomy appears to be the only treatment conferring benefit, chemotherapy and radiation therapy being of little proven value to date. The 5-year disease-free survival for grade I tumours can be as high as 76%, and up to 70% for grade II tumours whereas for grade III tumours it is reportedly about 15%. CONCLUSION: Primary angiosarcoma of the breast is a rare malignancy with a poor prognosis, even after complete resection. Surgery is the mainstay of treatment with a limited role for chemotherapy and radiotherapy.

Ischaemic fasciitis: a very rare entity with unusual presentation.

We are reporting a case of ischaemic fasciitis which occurred in a 55-year-old female with no debilitating or long bed ridden history. She presented with a large swelling over left gluteal region. On evaluation, swelling was found to be of size, 5x5 cm, slightly tender and with induration. The operative findings led to a probable diagnosis of a calcified lesion, due to its hard consistency. However, the microscopic picture was typical of ischaemic fasciitis, because of its characteristic central necrosis, vascular and atypical fibroblastic proliferations. Also seen was presence of foreign body giant cell reactions, inflammatory cells and extravasated RBCs. Ischaemic fasciitis is a very rare pseudo sarcomatous proliferation of atypical fibroblasts, which has been described to be located over bony protuberances and said to develop most often in immobile elderly or debilitated patients. Recognition of this distinct entity as a reactive process, mostly associated with debilitation is rare in occurrence.

Gastric carcinoid presented as gastric outlet obstruction.

Carcinoids are solid tumors of entero-chromaffin origin classified as neuroendocrine amine precursor uptake and decarboxylation (APUD) cell tumors and are found throughout the gastrointestinal tract from the stomach to the rectum, with a predilection for the ileum. These tumors rarely occur in the stomach, constituting only 2% of gastric tumors and 3.3% of all carcinoid tumors of the gastrointestinal tract. Localization of gastric tumors can be done by ultrasonography, computed tomography, and magnetic resonance imaging, but endoscopic biopsy is required for confirmation; demonstration of neurosecretory granules on microscopy is the most specific feature. Early resection is the treatment of choice. Prognosis of noninvasive tumors <2 cm in size in terms of 5-year survival rate is 100%. The 5-year survival rate declines and prognosis becomes poor with increase in size and with involvement of serosa, perineural infiltration, and lymph node metastasis.

Fine needle aspiration cytology diagnosis of aneurysmal bone cyst: a case report.

Aneurysmal bone cyst is a relatively rare tumour accounting 1 to 6% of all primary bone tumours. The involvement of clavicle is even rare with incidence of only 3% of all cases of aneurysmal bone cyst. Aneurysmal bone cyst is an expanding osteolytic lesion occurring mostly at metaphyseal ends of the long bones. Although excisional biopsy is diagnostic for aneurysmal bone cyst and FNAC (fine needle aspiration cytology) is mostly inconclusive. Still FNAC can be helpful in pre-operative diagnosis of aneurysmal bone cyst.

FNAC diagnosis of MPNST--a case report.

Malignant peripheral nerve sheath tumour (MPNST) is a relatively rare tumour arisingfrequently in patients with neurofibromatosis, type I (NF1). It is usually a high grade sarcoma. A 65 year old female patient presented with a swelling in left popliteal fossa of two months duration. She had multiple nodular lesions all over the body since childhood. She was subjected to fine needle aspiration for the popliteal mass and was reported as MPNST which was confirmed on histopathology. She developed recurrence within four months time. The prognosis of MPNST is poor. Fine needle aspiration cytology is a very useful, rapid sensitive and inexpensive investigative procedure for the diagnosis of soft tissue tumours and can replace an open biopsy for the diagnosis of MPNST in patients with neurofibromatosis-1.