Eccrine syringofibroadenoma (ESFA) is a rare, benign tumor of eccrine sweat gland origin that usually presents as a nodule on the extremities of an elderly person. It can also present as an ulcerative plaque, verrucous lesion, papular or nodular lesion or as palmoplantar keratoderma. Although the clinical features are variable, histology is characteristic in the form of anastomosing strands, cords and columns of epithelial cells embedded in a fibrovascular stroma. We report the case of a 62-year-old male with a nodular lesion on the extremity that caused secondary involvement of the nail in the form of complete nail dystrophy. The histology showed features consistent with ESFA. Nail involvement by ESFA is a rare presentation and is rarely described in the literature.
Eccrine syringofibroadenoma; fibroepithelioma of Pinkus; schopf syndrome
Port-wine stains (PWSs) are congenital vascular lesions caused by progressive ectasia of blood vessels located in the vascular plexus of the dermis. Acquired PWSs develop later in life but are identical in morphology and histology to the congenital PWSs. Less than 75 cases of acquired PWSs have been reported in the published literature, of which there has not been a single report from India so far. Various factors have been proposed for its pathogenesis like trauma, actinic exposure, drugs, tumors, and herpes zoster infection. We report an acquired port-wine stain in a 41-year-old male. The causative factors, treatment, and previous reports of this uncommon entity have also been reviewed.
Adult; acquired; port-wine stain
Frictional lichenoid dermatitis.
Frictional lichenoid dermatitis (FLE) is an entity that is probably under diagnosed and has been variably associated with either friction and/or atopy with a distinctive seasonal variation.
Aims and Objectives:
To study correlation of FLE with UV index and to assess its association with atopic dermatitis.
Materials and Methods:
A cross sectional analysis of children with FLE was done, over a period of 6 years in two tertiary hospitals. A detailed history and examination was done to assess the features of atopic dermatitis. The number of cases seen per month was compared with the mean monthly UV index. Two-tailed significance tests using Pearson's coefficient of correlation and T-test were used to interpret the data. (P < 0.05).
One hundred seventy-four patients were studied using the UKC criterion 17.2% of the patients had AD while xerosis (40.3%) was the predominant cutaneous finding. The number of patients seen in summer was more than in winter (P < 0.05) but there was no statistical difference between the cases in winter and spring. There was a significant correlation of the number of cases per month with UV index (P = 0.019). Almost 42% of patients gave a history of recurrence.
FLE is probably not associated with atopic dermatitis and is likely to be related to the ambient UV index though a larger cohort with meticulous follow up may be needed to draw a final conclusion.
Statistical Analysis Used:
The Pearson's coefficient of correlation was used for comparing the cases per month with the UV index. The tests of hypothesis used included the paired T-tests. F-test of variance, Welch test, Wilcoxon rank sum test and the Kolmogorov-Smirnov Test. P < 0.05 was considered significant.
Atopic dermatitis; frictional lichenoid dermatitis; seasonal variation; summer; Sutton's prurigo; UV Index; UV light
Clear cell sarcoma of kidney (CCSK) is an aggressive renal neoplasm. We report two boys aged three and half, and three years with CCSK, one of whom had a disease free survival of four years and eight months. These patients were managed with surgery, chemotherapy and radiotherapy. One of the patients discontinued treatment early and lost to follow up. Aggressive multimodality therapy is the keystone to improved outcome.
Clear cell sarcoma; Kidney; Wilm's tumor
One-third of schwannomas occur in head and neck region, mostly in the parapharyngeal region. Cystic change is seen in only 4% of schwannomas. The diagnosis of such tumors remains a challenge due to the rarity of these lesions and limited utility of fine needle aspiration cytology. When cystic, branchial cleft cyst is an important differential diagnosis more so due to radiological resemblance. We present a case of 42-year-old male with left sided neck mass masquerading as branchial cleft cyst clinically and radiologically. Multiple sections examined from the cystic areas of the mass revealed lymphoid aggregates beneath the cyst wall in addition to the schwannomatous areas.
cystic schwannoma; branchial cyst; lymphoid aggregates
Griscelli syndrome 2 is a rare autosomal recessive disorder of pigmentary dilution of hair, skin, splenohepatomegaly, pancytopenia, immune and neurologic dysfunction. Clinical course is characterized by recurrent infection triggered by uncontrolled T-lymphocyte and macrophage activation, called hemophagocytic syndrome. Since the primary presentation is with depigmented hair, we attempt to highlight diagnostic difficulties in such cases in developing countries like ours where pigmentary changes in hair and skin are commonly attributed to severe malnutrition. We also evaluated phenotype of all 10 cases of genotype (c.C550T; p.R184X), collected from published literature worldwide and emphasize the potential role of above mutation as hotspot in Southeast Asian region.
Griscelli syndrome; Kala Azar; mutation
Rhabdoid tumors of extrarenal origin are uncommon. This is the first report of rhabdoid tumor presenting as a perineal mass. Furthermore, it is a disease of childhood, with adult presentation being rare. We report a case of extrarenal rhabdoid tumor and discuss its presentation and possible treatment options.
Rhabdoid tumor; Extrarenal rhabdoid; Perineal tumor
SNNL is a rare, benign lesion which may arise from trauma, a sclerosing haemangioma or it may represent a burned out phase of various conditions like parasitic infections, or chronic infections like tuberculosis. Histopathology provides the definite diagnosis, which thus helps clinicians in allaying unnecessary anxiety of the patient and in planning proper management.We are reporting two cases here, which had solitary necrotic nodules of liver (SNNL), with emphasis on the aetiology and morphology of the lesions. SNNL was incidentally detected during cholecystectomy done in both young female patients. The lesion was solitary in one case and it was multiple in the other. The suspected aetiology in one of the cases was parasitic, whereas it was tuberculosis in the other. The clinical features in both cases were suggestive of neoplasms.
Solitary necrotic nodule; Liver
This is a rare case of a 35 year old multiparous lady with complaints of postcoital bleeding and foul smelling discharge for 3 months. On per speculum examination, a large irregular friable growth was seen which used to bleed on touch. A provisional diagnosis of carcinoma cervix was made, but the cervical biopsy revealed granulomatous inflammation with caseation, consistent with the diagnosis of cervical tuberculosis. The patient responded well to 6 months of antitubercular treatment. Hence, tuberculosis should be kept as a differential diagnosis of carcinoma cervix.
Carcinoma; cervix; tuberculosis
Leiomyosarcomas of soft tissue are the rare tumors and the retroperitoneum is the most common site involved. We report a case of primary leiomyosarcoma of the peritoneal cavity which clinically presented with suprapubic, freely mobile, nontender mass which measured 10×10 cm in size. Contrast enhanced computed tomography revealed well defined heterogenous hypodense solid cystic mass. The mass was surgically excised out in its entirety. The histopathological examination revealed spindle cells arranged in alternating fascicles having pleomorphic nuclei, indistinct margin and eosinophilic cytoplasm with foci of haemorrhage, necrosis and 5-6 mitosis/HPF. The spindle cells were immunoreactive for smooth muscle actin, desmin and negative for S-100, CD-34 and c-kit. Histopathology and immunohistochemistry were helpful in making the final confirmatory diagnosis. Leiomyosarcomas are aggressive tumors, with poor prognosis and often difficult to treat. The survival rates are lowest among all soft tissue sarcomas.
tumor; sarcoma; soft tissue
Multifocal carcinoid is a known phenomenon. We present a rare combination of an ovarian carcinoid synchronous with gallbladder carcinoid. This rare combination has not been reported so far. An asymptomatic 45-years-old perimenopausal lady was diagnosed to have a metastatic ovarian cancer, but on laparotomy she was found to have a primary synchronous metastatic gall bladder as well. On histopathological evaluation she was found to have two separate primary carcinoids. Subsequently the patient received chemotherapy and is completely asymptomatic on follow up. Further research needs to be undertaken and guidelines need to be formulated for management of these cases.
We have discussed the case of a solitary polypoid ganglioneuroma in a 45–year–old male patient, at the base of tongue, which mimicked a malignancy. The interest of this case lay in the rarity of its incidence at the base of tongue and its gross resemblance to the more common malignant polypoidal growths at this site. The presence of neural elements and ganglion cells at this site makes it important for an inexperienced histopathologist to differentiate it from other neural lesions of the tongue, in order to avoid a misdiagnosis and this can prevent the clinician from administering an inappropriate treatment. Exhaustive search revealed only a very few case reports on the tongue.
Polypoidal; Tongue; Ganglion; Immunohistochemistry
Isolated soft tissue cysticercosis of the trunk in the absence of concurrent central nervous system involvement is uncommon and may be difficult to diagnose. We report 4 such cases in the pediatric age group. Preoperative diagnosis of soft tissue cysticercosis was considered only in 1 patient, the rest were diagnosed only after biopsy. Complete excision (without rupture) was done. All of them underwent a CT scan head along with ophthalmic examination to rule out the more common sites of occurrence of cysticercosis. No further treatment was undertaken as the evaluation was negative. In endemic areas like ours we must suspect this entity not only in the limb muscles, but also in the subcutaneous tissues of the trunk. If diagnosed accurately, it can be treated medically, eliminating the need for surgery.
Cysticercosis; Taenia solium; Parasitic cyst
A 22 year old male psoriatic patient presented with multiple reddish scaly plaques all over body. After hematological and biochemical investigations the patient was started on oral methotrexate 15 mg weekly. PASI score at the start of treatment was 26.2. After 3 months PASI dropped to 11.5, the dose of methotrexate was tapered to 7.5mg weekly and the patient was maintained on this dose and kept under monthly follow up. Four months later, the patient presented with reddish to hyperpigmented raised firm nodules having a central crater over the healing plaques of psoriasis. Few lesions showed self resolution over a period of 6-12 weeks. Histopathology of the lesion confirmed it to be Keratoacanthoma. We believe the most likely etiologic factors for the multiple KAs in our patient could be a genetic susceptibility stimulated by multiple causes.
Keratoacanthoma; Psoriasis; Spontaneous; Healing
Pityriasis rosea (PR) is an acute or subacute inflammatory skin disease characterized by erythematous papulosquamous eruptions localized on the trunk and arms. The eruptions are self-limiting and usually disappear gradually in 2-10 weeks, without any treatment. Typical PR is much easier to diagnose than the rare atypical forms. There is a passing mention of PR with erythema multiforme-like lesions in the literature, but no extensive case series have been published till date. We present a series of five patients for whom we believe atypical PR is the likely diagnosis.
Erythema multiforme; papulosquamous eruption; pityriasis rosea
A case of hydatid cyst within a congenital cystic adenomatoid malformation (CCAM) of the right lower lobe of lung in an 8-year-old girl is reported. Presence of CCAM was confirmed on histopathology of the lung tissue attached to the specimen.
Hydatid cyst; Congenital cystic adenomatoid malformation; Pericystectomy
Gastrointestinal stromal tumours (GIST) are rare in paediatric patients and have a discrete clinicopathological and molecular divergence from that observed in adults. In the present report we present a case of a 2-month-old female in whom colonic gastrointestinal stromal tumour acted as a lead point of colocolic intussusception. Laparoscopically assisted reduction of the intussusception and resection of tumour was done.
Gastrointestinal stromal tumour; Colonic intussusception; Child
Fetus in fetu is an uncommon anomaly in pediatric age group. There have been controversies regarding its differentiation from well differentiated teratoma. While presence of axial skeleton has been considered mandatory for diagnosis, there are many reports of fetus in fetu without any vertebral column. It has been suggested that the two entities form a spectrum of lesions depending upon the timing of embryological insult. The present case is another example of a lesion that fits in this spectrum of interesting lesion.
Fetus in fetu; Retroperitoneal teratoma
A child with isolated renal mucormycosis who was successfully treated with systemic anti-fungal therapy and nephrectomy is reported.
Amphotericin B; isolated renal mucormycosis; nephrectomy; renal zygomycosis
Epidermal inclusion cyst in the breast (EICB) is very rare. It presents as breast lump mainly in peri-areolar region and needs to be differentiated from other breast lesions with predominance of squamous cells.
To analyze the role of fine needle aspiration cytology (FNAC) in patients presenting with breast lump.
Materials and Methods:
The present study is a retrospective study over the period of past 2 years, where the patients presented with firm breast lumps. Six of these cases were diagnosed as EICB on FNAC. Smears from these cases were reviewed for cytomorphological findings and correlated with clinico-radiological details.
Patients ranged in age from 25 to 60 years, and female to male sex ratio was 2:1 (female 4, male 2). Mammography was done in women (4), which showed solid, well-defined mass lesions in peri-areolar region and were suggestive of benign breast lesions (3) and infective lesion (1). FNA was performed; Giemsa-stained smears showed predominantly mature benign squamous cell and anucleated squames in all cases, thus one of the cytological diagnoses was EICB. Five of these cases had histological confirmation.
EIC of breast is not that rare lesion as considered. The patients may not seek medical attention because of small painless swelling; unless the lump increases in size or becomes painful. FNAC is confirmatory in the presence of a typical pultaceous aspirate and cytomorphological features of EIC. Thus, FNAC plays a crucial role in its diagnosis and management. Symptomatic cases should be readily excised and need histological correlation to rule out any potential complications that can arise in these cysts.
Benign breast lesions; breast lump; cytology; epidermal inclusion cyst
Fungi have emerged as important etiological agents for chronic sinusitis. Invasive aspergillosis has been reported in immunocompromised individuals or diabetics; however, it is uncommonly seen in immunocompetent patients. Definitive diagnosis of these lesions is based on histological examination and fungal culture. We report two cases of invasive maxillary lesions in immunocompetent patients, clinically suspected of malignancy; however, fine needle aspiration cytology showed fungal hyphae, morphologically suggestive of Aspergillus, which was later confirmed on histopathology. Aspiration cytology thus plays a crucial role in the early and definitive diagnosis of fungal sinusitis in cases clinico-radiologically suspected of malignancy. An early diagnosis will help the clinician for early and appropriate management and follow-up in order to decrease the high morbidity and mortality associated with it.
Aspergillosis; cytology; fungal sinusitis; immunocompromised
Though fractional lasers are widely used for acne scars, very little clinical or histological data based on the objective clinical assessment or the depth of penetration of lasers on in vivo facial tissue are available. The depth probably is the most important aspect that predicts the improvement in acne scars but the studies on histology have little uniformity in terms of substrate (tissue) used, processing and stains used. The variability of the laser setting (dose, pulses and density) makes comparison of the studies difficult. It is easier to compare the end results, histological depth and clinical results. We analysed all the published clinical and histological studies on fractional lasers in acne scars and analysed the data, both clinical and histological, by statistical software to decipher their significance. On statistical analysis, the depth was found to be variable with the 1550-nm lasers achieving a depth of 679 μm versus 10,600 nm (895 μm) and 2940 nm (837 μm) lasers. The mean depth of penetration (in μm) in relation to the energy used, in millijoules (mj), varies depending on the laser studied. This was statistically found to be 12.9–28.5 for Er:glass, 3–54.38 for Er:YAG and 6.28–53.66 for CO2. The subjective clinical improvement was a modest 46%. The lack of objective evaluation of clinical improvement and scar-specific assessment with the lack of appropriate in vivo studies is a case for combining conventional modalities like subcision, punch excision and needling with fractional lasers to achieve optimal results.
Acne scars; efficacy; fractional lasers; histological depth
Extra-abdominal desmoid tumors are known as aggressive fibromatosis (AFM). Synchronous and metachronous multicentric aggressive fibromatosis are rare lesions and pose dilemma in diagnosis and management. A rare and interesting case of recurrent multicentric, synchronous AFM is presented which to the best of our knowledge has not been reported earlier. A young male presented with well defined, hard, fixed swelling on the thigh. Resected tumor mass on histopathology was diagnosed as an extra abdominal fibromatosis. He presented again after two months with swelling at the same site; and two more swellings on the foot. Fine needle aspiration cytology (FNAC) from all three sites was performed; and was suggestive of benign spindle cell lesion of fibrogenic origin with the possibility of multicentric synchronous recurrent aggressive fibromatosis.
Aggressive fibromatosis; cytology; desmoid tumor; synchronous multicentric tumors
Isolated sternal involvement in tuberculosis is rare. Very few case reports are available in literature even from the countries where tuberculosis is endemic. We are reporting a case of 32-year-old female who presented to us with a pus discharging sinus over manubrium sterni with no other systemic features of tuberculosis. Sinus tract was excised and biopsy confirmed tuberculosis. Patient received antitubercular therapy for 9 months.
tuberculosis; sternum; sinus.
Antenatal detection of Wilms’ tumor is rare and only nine cases have been reported till now in the English literature. We report a case of a 7-month-old child with prenatally detected renal mass managed by nephroureterectomy associated with intraoperative spill. The child developed recurrence while on DD4A chemotherapy. Salvage chemotherapy was initiated but there was no response after two cycles and the child expired due to tumor rupture.
Neoplasm; prenatal diagnosis; recurrence; Wilms’ tumor