Spinal atypical teratoid rhabdoid tumor-narrative review and report of a rare case managed with multimodality approach.

BACKGROUND: Spinal atypical teratoid rhabdoid tumor (AT/RT) is an extremely rare tumor and represents less than 2% of all AT/RTs. METHODS: Available medical literature on spinal AT/RT in English was retrieved from PubMed and comprehensively reviewed. Clinical presentation, diagnosis, management, prognosis, and outcome in patients with spinal AT/RT have been elucidated by citing a case of extradural AT/RT of the cervicodorsal spine. RESULTS: The age at presentation is usually less than 3 years. The most common site is the cervicodorsal spine. The most frequent tumor location is intradural extramedullary. A contrast-enhanced magnetic resonance imaging (MRI) of the entire neuraxis is the imaging modality of choice. The incidence of leptomeningeal dissemination is high (15-30%). Histopathological examination shows an admixture of primitive neuroectodermal, mesenchymal, and epithelial elements along with rhabdoid cells. Loss of SMARCB1/INI1 is considered pathognomonic of AT/RT. Maximal safe resection of tumor is the initial management of choice. Thereafter focal radiotherapy for localized tumor or craniospinal irradiation for leptomeningeal dissemination should be considered. Post-operative intensive polychemotherapy including intrathecal and high-dose chemotherapy (with autologous stem cell rescue) is usually considered to optimize survival. Typically, the time to recurrence and overall survival are less than 6 and 12 months, respectively. However, with judicious multimodality management long-term survivors are increasingly being recognized. The illustrative patient was a 18-month-old girl diagnosed with extradural AT/RT of the cervicodorsal spine (C3-D1), who was managed with maximal safe resection of tumor, multiagent chemotherapy (ICE-ifosfamide, carboplatin, etoposide) and focal RT to the tumor bed-50.4 Gy/28 fractions/5.5 weeks. At the last follow-up visit, 30 months after surgery, she had complete clinicoradiological response. CONCLUSION: Multimodal treatment comprising maximal safe resection of tumor, multiagent chemotherapy (ICE), and focal RT can lead to successful outcome in patients with localized spinal AT/RT, under the age of 3 years.

Extramedullary Relapse of B Cell Acute Lymphoblastic Leukemia in Bilateral Breasts Treated with Whole Breast Radiotherapy.

Isolated extramedullary relapse of acute lymphoblastic leukemia (ALL) in the breast is extremely rare. We herein report a case of a 38-year-old female with B cell ALL, who had isolated extramedullary relapse initially in the left breast and subsequently in the right breast, 3 and 4 years, respectively, after hematopoietic allogenic stem cell transplantation. She was successfully salvaged with bilateral whole breast radiotherapy, 24 Gy/12 fractions/2.5 weeks. This brief report highlights the importance of awareness of extramedullary leukemic relapse in the breast as one of the differential diagnoses of breast masses in the context of ALL. Since these tumors are extremely radioresponsive, radiation therapy is a safe and effective treatment option for isolated extramedullary relapse of ALL in the breast.

Combined Modality Management of Sinonasal Anaplastic Lymphoma Kinase Negative Anaplastic Large Cell Lymphoma in a Geriatric Patient-Report of a Rare Case.

Sinonasal anaplastic lymphoma kinase(ALK)-negative anaplastic large cell lymphoma(ALCL) without nodal involvement is exceedingly rare and the rarity of this tumor often engenders diagnostic dilemma. It has been mostly reported in pediatric, adolescent and young adult patients, mostly of Asian origin. A 70-year-old female patient presented with a mass in the left nasal cavity causing nasal obstruction for 5 months. On clinical and radiological examination, there was a 5.7 cm mass in the left nasal cavity, completely obliterating the left ethmoid sinus. Biopsy from the nasal mass showed a poorly differentiated malignant tumour with large cells arranged in sheets. On immunohistochemistry, the tumour cells were positive for leukocyte common antigen(LCA), CD30, CD43, BCL6 and focally for CD5, TIA1, granzyme B and epithelial membrane antigen(EMA), and were negative for CD56, EBV-LMP1, CD79a, PAX5, myeloperoxidase, CD34, CD7, CD4, CD8, CD138, ALK and p63, suggestive of ALK-negative ALCL. Rest of the lymphoma work-up was essentially normal and she had stage IE disease. She was treated with prephase chemotherapy (Vincristine and Prednisolone) followed by 4 cycles of CEOP[Cyclophosphamide, Etoposide (from 2nd cycle onwards), Vincristine and Prednisolone] regimen and local radiotherapy (36 Gy/20 fractions/4 weeks) by intensity modulated radiotherapy(IMRT) technique resulting in complete clinical and radiological response. At last follow-up visit, 15 months from the initial diagnosis, she was alive and disease free. Sinonasal ALK-negative ALCL is a rare tumor which can be effectively treated with a combination of multi-agent CHOP/CHOP-like regimen and local conformal radiotherapy in geriatric patients.