Geetha Narayanan

All-trans-retinoic acid-induced pseudotumor cerebri in acute promyelocytic leukemia

All-trans-retinoic acid is an integral part in the treatment strategy of acute promyelocytic leukemia (APL). Here we describe a case of pseudotumor cerebri associated with all-trans-retinoic acid (ATRA) during the induction therapy in an adult with acute promyelocytic leukemia (APL).

Bilateral synchronous plasmacytoma of the testis.

Extramedullary plasmacytoma (EMP) is usually seen in the head and neck regions and in the upper respiratory, gastrointestinal, and central nervous systems. Testis is a rare site for EMP, and bilateral synchronous testicular plasmacytoma occurring as an isolated event at initial presentation has been reported only once previously. We present herein the second such report in a 70-year-old man who underwent bilateral orchidectomy.

Rhabdomyosarcoma of the vagina in an adolescent girl

BACKGROUND Gynecologic neoplasms are rare in children and represent only less than 5% of all childhood tumors. Rhabdomyosarcoma (RMS) of the female genital tract of children accounts for only 3.5% of the cases. CASE A 16-year-old adolescent presented with a proliferating growth and foul smelling discharge from her vagina, which, on biopsy was diagnosed as RMS. She received chemotherapy and radiation to the primary site. She is alive in remission at 8 years, and with normal menstrual function. SUMMARY AND CONCLUSION RMS of the vagina is a rare, but highly curable tumor in adolescent girls. Any abnormal vaginal bleeding in girls should be promptly investigated using pelvic examination and appropriate imaging. An organ-preserving approach should be considered in these patients.

Mixed Phenotype Acute Leukemia Presenting as Leukemia Cutis

Leukemia cutis (LC) is defined as infiltration of the skin by leukemic cells resulting in clinically recognizable cutaneous lesions. It is common in congenital leukemia and acute myeloid leukemia. However, LC has rarely been reported with mixed phenotypic acute leukemia (MPAL). We report the case of a lady who presented with erythematous papular and nodular lesions all over the body. Skin biopsy showed leukemic infiltration and bone marrow aspiration showed MPAL of the T/myeloid with monocytic differentiation lineage. This is the first report of an adult patient with MPAL of the T/myeloid with monocytic differentiation type presenting with leukemia cutis. She was started on chemotherapy with Hyper-CVAD. There is complete resolution of the skin lesions and she has achieved bone marrow remission after the first cycle of chemotherapy.

Blood group change in acute myeloid leukemia.

Blood group antigens are either sugars or proteins found attached to the red blood cell membrane. ABO blood group antigens are the most clinically important antigens because they are the most immunogenic. As red blood cell antigens are inherited traits, they are usually not altered throughout the life of an individual. There have been occasional case reports of ABO blood group antigen change in malignant conditions. We report two such cases of ABO antigen alteration associated with acute myeloid leukemia. These patients had suppression of their blood group antigens during their leukemic phase, and the antigens were reexpressed when the patients attained remission.

Isolated intracranial myeloid sarcoma occurring as relapse in acute myeloid leukemia

Myeloid sarcoma (MS) or chloroma is a rare extramedullary tumor composed of extramedullary proliferation of blasts of granulocytic, monocytic, erythroid, or megakaryocytic lineage occurring at sites outside the bone marrow. MS occurs in 2%–8% of patients with acute myeloid leukemia (AML), sometimes it occurs as the presenting manifestation of relapse in a patient in remission. We describe the case of a young male with AML in remission for 6 years presenting with central nervous system symptoms. Magnetic resonance imaging showed an extra-axial altered intensity lesion in the parasagittal parietal region, infiltrating anterosuperiorly into anterior falx, and posterosuperior aspect of the superior sagittal sinus. A biopsy from the lesion was diagnostic of MS which was positive for myeloperoxidase. He did not have any other sites of disease. He has received chemotherapy with FLAG (Fludarabine, Cytosine arabinoside) followed by cranial irradiation and is in complete remission.

Synovial Sarcoma of the Larynx: Report of a Case and Review of Literature

Sarcomas account for less than 1% of malignant neoplasms arising in the head and neck in adults. Laryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature. We report the case of a 48-year-old man with synovial sarcoma of the larynx. He underwent excision of the tumor followed by radiation. He is alive in remission at 36 months. The literature on synovial sarcoma of the larynx is reviewed.

Ewing's Sarcoma of the Trachea in an Adolescent Girl

Primitive neuroectodermal tumors (PNET) are aggressive neoplasms of neuroectodermal origin. Although they are known to arise in a host of locations, involvement of the trachea has rarely been reported. We describe an adolescent girl who presented with stridor and was diagnosed with PNET of the trachea. She is in remission following treatment with combination chemotherapy and local radiotherapy.

Lymphoblastic Lymphoma of the Palate

Lymphoblastic lymphomas are neoplasms of immature or precursor lymphoid cells with no or limited bone marrow involvement, whose clinical presentation varies according to the immunophenotype. While mediastinal involvement is predominant in T-lymphoblastic lymphomas, B-lymphoblastic lymphomas frequently involve nodal sites. Extranodal presentation of B-lymphoblastic lymphomas is extremely rare. We present the case of a 49-year-old man with B-lymphoblastic lymphoma of the hard palate. The patient was treated with the R-Hyper CVAD regimen and is on maintenance chemotherapy. This is the first reported case of B-lymphoblastic lymphoma arising in the hard palate.

B-Lymphoblastic Lymphoma of the Rectum

Lymphoblastic lymphoma is a neoplasm of immature cells committed to the B-cell or T-cell lineage. B-lymphoblastic lymphoma usually involves lymph nodes and extranodal sites, such as the skin, bone, and soft tissue. The rectum is a very rare site of involvement in B-lymphoblastic lymphoma. We describe a 26-year-old man who presented with bleeding per rectum and fecal incontinence. Endoscopy showed a large nodular friable lesion narrowing the entire rectal lumen. Biopsy was diagnostic of B-lymphoblastic lymphoma. He was started on chemotherapy with the Berlin-Frankfurt-Münster protocol and achieved complete remission after induction chemotherapy. This is the first report of B-lymphoblastic lymphoma presenting as a rectal lesion in the world literature.