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Multiple acetyl CoA dehydrogenase deficiency is a rare autosomal recessive disorder of amino acid, fatty acid, and choline metabolism. It is a mitochondrial disorder with defective electron transfer flavoproteins or electron transfer flavoprotein dehydrogenases. They are vital for β-oxidation of fatty acids, an essential fuel for skeletal and cardiac muscles. It is also an important source of energy during starvation for the brain. Acute deterioration of these patients can occur during stressful periods like starvation, surgery, infection, and exercise. The anaesthetic management is a challenge with special emphasis on minimizing starvation, ensuring hydration and glucose supplementation, and considering the various effects of anaesthetic agents on the mitochondrial function. The anaesthetic management of a patient with multiple acetyl CoA dehy- drogenase scheduled for modified radical mastectomy is described. General anaesthesia can be administered safely in these patients with special emphasis on hydration, glucose supplementation, avoidance of stressors, and monitoring of metabolic status.

Follicular dendritic cells are antigen-presenting immune accessory cells of mesenchymal origin. Follicular dendritic cell sarcomas (FDCS) typically occur in nodal and extranodal sites. However, presentation in the extremity has rarely been reported. A woman in her 60s had a painless, slow-growing right posterior thigh swelling, which had been present for 9 months. Imaging revealed a subcutaneous lesion in the posterior aspect of the right mid-thigh. Fine-needle aspiration cytology from the lesion was suggestive of a malignant spindle cell neoplasm, and she underwent its wide local excision. Immunohistochemistry showed expression of epithelial membrane antigen and CD35, but an absence of cytokeratin, desmin, CD23 and S100. The Ki-67 index was low and a diagnosis of FDCS was made. The lack of clinical guidelines was a challenge in the treatment of this rare case. A multidisciplinary board discussion played a critical role in the planning of the patient's adjuvant treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Context: There is heterogeneity in the clinical behavior of breast carcinoma patients with node negativity. Studies have analyzed different factors influencing the outcome in such patients. It is suggested that the presence of nodal micrometastasis can act as a tool in predicting the aggressiveness of these tumors., Aims: The aim of this study is to assess the yield of micrometastasis/isolated tumor cell (ITC) by ultrastaging the morphologically negative axillary nodes and staining them with immunohistochemistry for epithelial membrane antigen. The association of such metastasis with the clinical outcome is determined., Settings and Design: This was a retrospective analytical study. One hundred cases of node-negative breast carcinoma patients who underwent surgery along with axillary lymph node dissection were selected., Materials and Methods: The largest node from the axillary dissection was selected and subjected to ultrastaging and immunohistochemical staining (as sentinel node dissection was not a routine practice at that time), to look for occult metastasis in the form of micrometastasis or ITCs., Statistical Analysis: Occurrence of events in the form of recurrence or death was noted. Association of the parameters was analyzed using Fisher's exact test., Results: Among the 100 cases, 79 patients were followed up for a minimum period of 5 years. Two cases had micromets in one node each. These two patients were among the eight, who developed events subsequently (25%). Hence, a statistically significant association was found between the presence of micromets with events., Conclusions: There is a statistically significant association between the presence of micromets and disease recurrence. Hence, we suggest that ultrastaging of the negative axillary node (now sentinel node, as it is being routinely done) might prove effective in predicting the events/prognosis in clinically and morphologically node-negative breast carcinoma patients., Competing Interests: There are no conflicts of interest

Granulosa cell tumors constitute less than 5 % of all ovarian tumors. Unlike epithelial ovarian tumors, they occur in a younger age group, are usually detected in an early stage and often have features of hyperestrogenism. The presenting symptoms are usually nonspecific with abdominal pain or distension. They follow an indolent course and are characterized by a long natural history. Mutation of FOXL2 (402C->G) seen in 97 % of adult GCT may be pathognomonic for adult GCT. Only stage of the disease has been consistently shown in various studies to affect survival of patients with GCT. The initial management of patients, for whom fertility is not an issue, is total abdominal hysterectomy, bilateral salpingo-oophorectomy and removal of all gross disease. Nodal dissection is not a significant factor for survival and is not recommended in surgical staging of GCT. Fertility preserving surgery with unilateral salpingo-oophorectomy is feasible in young patients with stage Ia GCT. Patients with early stage disease (stage I and II) have a very good prognosis with 5 year DFS and OS of 89 % and 99 % respectively and these groups of patients usually don't require any postoperative treatment. Patients with stage Ic disease associated with poor prognostic factors like large tumor size or high mitotic index and stage II, have a higher chance of relapse, and may benefit with postoperative treatment but role of chemotherapy is still debatable. In advanced stage disease (stage III and IV) the 5 year DFS and OS disease was 72 % and 80 % respectively hence the option of postoperative treatment with 6 cycles of BEP should be considered in this group. Recently paclitaxel is being investigated as an effective tool in GCT. The efficacy of radiation in GCT is not well defined but in optimally debulked cases postoperative radiation is a viable option. Due to high chance of recurrence even years after apparent clinical cure of the primary tumor, lifelong follow up with clinical examination and tumor markers like inhibin B is recommended. About 25 % GCT develop recurrence and the median time to recur is usually 4-5 years. Most recurrences are intraperitoneal and usually a complete debulking of the disease is feasible even in the recurrent setting. Postoperative chemotherapy (platinum based) is usually given after surgery more so in cases with widespread disease or after suboptimal cytoreduction. Recurrent chemoresistant, progressive non-responding GCT or patients with high surgical risk are ideal candidates for targeted therapy.