Dermatofibrosarcoma Protuberans of Face: A Rare Entity and Review of Literature.

Dermatofibrosarcoma protuberans is a very rare tumour that accounts for less than 0.1% of all malignant neoplasms. It is a locally aggressive tumour with high recurrence rate. Head and neck involvement is very uncommon and hence diverse treatment protocols have been recommended for its management in the currently available literature. We present a case of dermatofibrosarcoma protuberans over the lower part of right cheek in a middle aged male, which was successfully treated with wide local excision and reconstruction along with post-op radiotherapy. The patient was followed-up for 18 months with no recurrence. Prompt and accurate diagnosis along with multidisciplinary treatment is crucial for optimal management of this rare tumor. The optimal treatment option is surgical resection with wide margins along with adjuvant radiotherapy, which leads to better outcomes even in patients with positive surgical margins. Larger clinical trials with Imatinib will firmly establish its chemotherapeutic role in its management. Lifelong regular follow-up is essential for early detection of tumor.

Intra-medullary Tuberculomas: Case Series.

Intramedullary spinal tuberculoma is an extremely rare disease when compared to pulmonary, extrapulmonary and skeletal tuberculosis in developing countries. In the absence of systemic tuberculosis, clinical presentation is non distinctive from other intramedullary lesions. We report two cases of intramedullary tuberculoma both presenting with signs and symptoms of space occupying lesions. Surgical excision was done in both cases following which patients improved neurologically. Histopathological evaluation is essential to provide curative treatment.

Primary intracranial malignant melanoma: A rare casewith review of literature.

We present a rare case of the primary intracranial melanoma in the right parafalcine frontal region in a 65-year-old male. Computed tomography and magnetic resonance imaging showed a Space occupying lesion in the right parafalcine frontal region with necrosis, vasogenic edema and mass effect. A rather well-defined, dark brown-black tumor was totally removed and histologically diagnosed as malignant melanoma. The patient improved after surgery. In our case, no systemic melanomas were found in close clinical examinations, there was a single nodular tumor attached to the leptomeninges and a favorable outcome was obtained by surgical treatment alone. Primary central nervous system melanomas have rarely been reported, and this case is reported for its rarity.

Functioning oxyphil parathyroid adenoma: a case report.

Oxyphil parathyroid adenomas are rare and clinical features of patients with this entity are not well defined. We are presenting a case of primary hyperparathyroidism with marked elevation of parathyroid hormone (PTH) and near normal calcium levels, that underwent parathyroidectomy. Histopathology revealed an oxyphil adenoma which showed positivity for PTH on immunohistochemical staining. Post - operatively, there was a significant decline in both PTH and alkaline phosphatase levels. Benign oxyphil adenomas may mimic parathyroid carcinomas, both in terms of clinical features and tumour size; and they should be considered in the differential diagnosis of patients with primary hyperparathyroidism.

Severe Aplastic Anemia Manifesting After Complete Remission of Acute Promyelocytic Leukemia: Is it a Fortuitous Association?

Acute leukemia, secondary myelodysplasia and paroxysmal nocturnal hemoglobinuria evolving from severe aplastic anemia (AA) following immunosuppressive therapy are well recognized. However, severe AA occurring after complete remission of acute promyelocytic leukemia (APL) has been documented only once in 2009. We report a case of 30-year-old male diagnosed with APL who achieved complete cytogenetic remission with all-trans retinoic acid based induction regimen and developed severe AA few months later during maintenance therapy.

Primary neuroendocrine carcinoma of the thymus.

Primary neuroendocrine tumors of the thymus, previously known as carcinoid tumors of the thymus, are unusual and rare tumors, and prognosis for these patients has been difficult to predict. We hereby report a case of primary neuroendocrine tumor of the thymus that had an aggressive and fatal course in spite of surgical resection and adjuvant chemotherapy. These tumors must be regarded as a malignant neoplasm that is prone to metastasize to distant sites, even after total excision.

Surgical management of parapharyngeal space tumors: our experience.

The purpose of this study is to evaluate parapharyngeal space (PPS) tumors with regards to clinico-pathological features and pre-operative assessment and also to analyze the benefits of transcervical approach in the management of PPS tumors. We performed retrospective analysis of patients who had undergone transcervical resection of PPS tumors from May 2006 to May 2009 at KLES Dr. Prabhakar Kore Hospital and M. R. C, Belgaum. Patients were diagnosed on the basis of clinical examination, FNAC and magnetic resonance imaging. All patients were observed with an annual follow-up of at least 3 years by physical examination and ultrasonographic evaluation. The total number of patients were four; with three males and one female patient. Their age varied from 20 to 55 years with a mean age of 36 years. There were two cases of neurilemomas, one case of carotid body tumor and one case of pleomorphic adenoma. All four patients underwent surgical excision of tumor by transcervical approach. There were no major post-operative complications. All four patients are alive with no recurrence in the 3 year follow-up period. Transcervical approach is versatile, flexible and provides good access to the narrow PPS. This approach also provides good vascular control and reduced risk of post-op complications. We recommend transcervical approach even for large sized pre-styloid and post-styloid compartment benign tumors which are free from deep lobe of parotid.

Necrotizing lymphadenitis in systemic lupus erythematosus: is it Kikuchi-Fujimoto disease?

Kikuchi-Fujimoto disease (KFD; also called Kikuchi disease) is a rare disorder clinically characterized by lymphadenopathy along with constitutional symptoms and several systemic features which may closely mimic infections, malignancies, and autoimmune diseases. Systemic lupus erythematosus (SLE) is an autoimmune disorder which may have several clinical manifestations similar to KFD. Association of KFD with SLE is only rarely described, and whether this is a chance incidence or Kikuchi disease is a localized manifestation of SLE per se remains a matter of debate. We report here coexistence of these two diseases in 1 patient.

Primary mucinous eccrine adenocarcinoma of the scalp: a case report.

BACKGROUND: Lesions of primary mucinous carcinoma of the skin present as painless, papular or nodular masses with sizes ranging from 5 mm to 120 mm. Metastatic deposits from undiagnosed visceral and breast adenocarcinoma are virtually indistinguishable microscopically from sweat gland carcinoma and must be considered before a diagnosis of sweat gland carcinoma is made. CASE: A 60-year-old woman presenting with a swelling over the scalp of 1 year's duration was clinically diagnosed to have a sebaceous cyst. Fine needle aspiration cytology of the lesion showed malignant cells in a mucoid background. Histologic examination of the excised mass revealed nests of tumor cells floating in pools of mucin in the dermis. Many of these nests showed tubular lumina. A diagnosis of mucinous eccrine adenocarcinoma was given. CONCLUSION: Primary mucinous adenocarcinoma of the skin is a rare tumor and mimics metastatic adenocarcinoma. The scalp is the second common site for these rumors. Literature search regarding cytologic findings shows our case could be the second case reported so far.

Cardiac sarcoidosis causing sudden death.

Sarcoidosis is a systemic disease of young adults. Cardiac involvement is rarely diagnosed clinically. In most cases it presents with arrhythmias and conduction disorders. We report a case of sudden death of a young female, wherein sarcoidosis with prominent cardiac involvement was diagnosed at autopsy. The other organs involved were lung and liver. Cardiac sarcoidosis should be considered in young patients with unexplained conduction disorders.

Serous microcystic adenoma of the pancreas.

Serous microcystic adenoma (SMA) is a rare benign neoplasm. It accounts for 1-2% of all exocrine pancreatic tumors. It is thought to arise from the ductal epithelial cells. It is usually located in the body and the tail of the pancreas. It is important to identify SMAs and distinguish them from mucinous cystic neoplasm, which can be premalignant or malignant, and pseudocyst, which is a non neoplastic condition. We present one such rare case of SMA occurring in the head of the pancreas, an infrequent location requiring a Whippel's resection.

Primary endobronchial Hodgkin's disease.

We report a case of primary pulmonary Hodgkin's disease presenting as an endobronchial mass. Tissue diagnosis was made by microscopic examination following open thoracotomy and excision biopsy of the mass. The patient responded well to the chemotherapy regimen.

Isolated polycystic liver disease--a rare entity: report of a case.

A 45-year-old woman presented with a mass in the right hypochondrium and shortness of breath. The mass was felt up to 4.5 inches below the right costal margin and its dullness on percussion was continuous with liver dullness. Ultrasonography (USG) of abdomen revealed enlargement of the left lobe of the liver with multiple cysts of varying sizes. Left liver lobectomy was done, histology of which showed multiple cysts lined by cuboidal to columnar epithelium. A small amount amount of normal liver parenchyma between the cysts was observed. A diagnosis of Adult polycystic liver disease (APLD) was given.

Orbital Rosai-Dorfman disease: report of a case with fine needle aspiration cytology and histopathology.

BACKGROUND: Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare, nonhereditary, benign histiocytic proliferative disorder, affecting mainly the lymph nodes. Orbital involvement in the absence of lymphadenopathy is relatively uncommon. CASE: A 50-year-old woman presented to our hospital with gradual proptosis of the left eye for 5 years. Physical examination revealed no abnormalities, including lymphadenopathy. Ultrasonography and magnetic resonance imaging showed a soft tissue mass in the intraconal retroorbital region of the left eye. Fine needle aspiration cytology of the mass yielded a good number of mature lymphocytes, a few neutrophils, plasma cells and many histiocytes exhibiting emperipolesis. A provisional diagnosis of SHML was suggested and later confirmed by histology of the excised mass. CONCLUSION: Though the orbit is a rare site of extranodal SHML, the disease should be entertained in the differential diagnosis of orbital swellings. To the best of our knowledge, this is the fourth case of SHML involving the orbit exclusively, with no nodal involvement.

Tubercular orchitis in a patient with AIDS: report of a case with fine needle aspiration diagnosis.

BACKGROUND: Although tuberculosis is one of the most common opportunistic infections in AIDS, the testis is rarely involved. Clinically, tubercular orchitis mimics malignancy. Fine needle aspiration (FNA) can be used to distinguish these 2 lesions. CASE: A 34-year-old, heterosexual male presented with right scrotal swelling, loss of weight and fever. Clinically, malignancy was suspected. FNA showed a few lymphocytes and neutrophils in a necrotic background. Ziehl-Neelsen staining showed high acid-fast bacillus positivity. Serologic testing for HIV showed seropositivity for HIV I and II antibodies. CONCLUSION: FNA is a useful modality in differentiating tuberculosis from malignancy. In developing countries, tuberculosis should be considered in cases of unilateral testicular enlargement. To the best of our knowledge, this is the third reported case of AIDS presenting as testicular tuberculosis.

Primary malignant melanoma of the ovary--a case report.

Primary malignant melanoma arising in the ovary is extremely rare. It is known to occur in association with benign cystic teratoma especially in post menopausal women. We report one such case of a primary malignant melanoma of the ovary, occurring in a premenopausal woman, after thorough examination of the patient to rule out a primary elsewhere.